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"identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Germans Trias i Pujol, Badalona, Barcelona, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Servicio de Endocrinología, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Madrid, Spain" "etiqueta" => "d" "identificador" => "aff0020" ] 4 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitario de Cruces, Baracaldo, Vizcaya, Spain" "etiqueta" => "e" "identificador" => "aff0025" ] 5 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Comarcal de La Línea de la Concepción, La Línea de la Concepción, Cádiz, Spain" "etiqueta" => "f" "identificador" => "aff0030" ] 6 => array:3 [ "entidad" => "Sección de Neurología Infantil, Servicio de Neurología, Hospital Universitario 12 de Octubre, Madrid, Spain" "etiqueta" => "g" "identificador" => "aff0035" ] 7 => array:3 [ "entidad" => "Servicio de Cardiología, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Madrid, Spain" "etiqueta" => "h" "identificador" => "aff0040" ] 8 => array:3 [ "entidad" => "Centre de Compétences Maladies Neuromusculaires, Hôpital Marin d’Hendaye APHP, Hendaya, Francia" "etiqueta" => "i" "identificador" => "aff0045" ] 9 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Donostia, San Sebastián, Guipúzcoa, Spain" "etiqueta" => "j" "identificador" => "aff0050" ] 10 => array:3 [ "entidad" => "Servicio de Genética, Hospital Universitario de la Santa Creu i Sant Pau, Barcelona, Spain" "etiqueta" => "k" "identificador" => "aff0055" ] 11 => array:3 [ "entidad" => "Servicio de Cardiología, Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, Madrid, Spain" "etiqueta" => "l" "identificador" => "aff0060" ] 12 => array:3 [ "entidad" => "Campus de Ciencias de la Salud, Universidad Católica San Antonio de Murcia, El Palmar, Murcia, Spain" "etiqueta" => "m" "identificador" => "aff0065" ] 13 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitario Insular de Gran Canaria, Las Palmas, Spain" "etiqueta" => "n" "identificador" => "aff0070" ] 14 => array:3 [ "entidad" => "Servicio de Neurología, Complejo Hospitalario de Navarra, Pamplona, Navarra, Spain" "etiqueta" => "o" "identificador" => "aff0075" ] 15 => array:3 [ "entidad" => "Servicio de Neurología, Hospital de Basurto, Bilbao, Vizcaya, Spain" "etiqueta" => "p" "identificador" => "aff0080" ] 16 => array:3 [ "entidad" => "Instituto Biodonostia-Departamento de Neurociencias, Universidad del País Vasco CIBERNED, San Sebastián, Guipúzcoa, Spain" "etiqueta" => "q" "identificador" => "aff0085" ] 17 => array:3 [ "entidad" => "Servicio de Medicina Genética y Molecular, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain" "etiqueta" => "r" "identificador" => "aff0090" ] 18 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain" "etiqueta" => "s" "identificador" => "aff0095" ] 19 => array:3 [ "entidad" => "Servicio de Neumología, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Madrid, Spain" "etiqueta" => "t" "identificador" => "aff0100" ] 20 => array:3 [ "entidad" => "Servicio de Neurología, Hospital General Universitario Gregorio Marañón, Madrid, Spain" "etiqueta" => "u" "identificador" => "aff0105" ] 21 => array:3 [ "entidad" => "UAM, Servicio de Neurología Pediátrica, Hospital Universitario La Paz, Madrid, Spain" "etiqueta" => "v" "identificador" => "aff0110" ] 22 => array:3 [ "entidad" => "Servicio de Cardiología, Hospital Universitario La Paz, Madrid, Spain" "etiqueta" => "w" "identificador" => "aff0115" ] 23 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitario de Canarias, Santa Cruz de Tenerife, Spain" "etiqueta" => "x" "identificador" => "aff0120" ] 24 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitario de Fuenlabrada, Fuenlabrada, Madrid, Spain" "etiqueta" => "y" "identificador" => "aff0125" ] 25 => array:3 [ "entidad" => "Medicina de Familia y Comunitaria, Centro de Salud Mar Báltico, Madrid, Spain" "etiqueta" => "z" "identificador" => "aff0130" ] 26 => array:3 [ "entidad" => "Servicio de Neurología, Hospital del Mar, Barcelona, Spain" "etiqueta" => "aa" "identificador" => "aff0135" ] 27 => array:3 [ "entidad" => "Enfermedades Neuromusculares, Servicio de Neurología, Hospital Universitario de La Princesa, Madrid, Spain" "etiqueta" => "ab" "identificador" => "aff0140" ] 28 => array:3 [ "entidad" => "Servicio de Neurología, Hospital Universitario 12 de Octubre, Madrid, Spain" "etiqueta" => "ac" "identificador" => "aff0145" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Guía clínica para el diagnóstico y seguimiento de la distrofia miotónica tipo 1, DM1 o enfermedad de Steinert" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Myotonic dystrophy type 1, Steinert's disease, Curschmann-Steinert disease, Batten-Gibb disease, atrophic myotonia or DM1 (OMIM <a href="omim:160900">160900</a>, ORPHA 273, ICD-9-CM 359.21, ICD-10 G71.1, ICD-11 8C71.0) is the most prevalent myopathy in adults.<a class="elsevierStyleCrossRefs" href="#bib0745"><span class="elsevierStyleSup">1,2</span></a> It is an autosomal dominant disease caused by expansion of a CTG triplet in the non-coding region of the <span class="elsevierStyleItalic">DMPK</span> gene, (protein kinase of myotonic dystrophy), located on the long arm of chromosome 19 (19q13.3). It is a disease traditionally diagnosed by neurologists due to its characteristic neuromuscular disorders, but which also produces systemic disorders. It has come to be considered as one of the diseases with the largest variety of phenotypes that exists. This variability in the clinical manifestations, both in quality and quantity, means that personalized treatment is required for each patient as well as extensive and practical knowledge of the disorders of each organ and system, in order to offer patients the most suitable follow-up and treatment.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Methodology</span><p id="par0010" class="elsevierStylePara elsevierViewall">The objective of this guide is to serve as a reference to the professionals involved in the diagnosis and follow-up of patients with DM1. It makes specific recommendations regarding diagnosis, monitoring and treatment of the disorders of this disease.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The group that has developed this guide includes specialists from all the professional groups involved in the care of patients with DM1: neurologists, pulmonologists, cardiologists, endocrinologists, neuropaediatricians and geneticists. The vision and preferences of the patients, their family members, and the users of this guide have been taken into account and are clearly defined.</p><p id="par0020" class="elsevierStylePara elsevierViewall">To produce this guide, a bibliographic search was carried out in the databases of the Cochrane Library, Cochrane Plus, EMBASE, PubMed-MEDLINE and ECA LOST following the PICO method (patients-intervention-comparison-results) and recommended by the National Health Service’ working group of clinical practice guidelines.<a class="elsevierStyleCrossRef" href="#bib0755"><span class="elsevierStyleSup">3</span></a> The search terms “myotonic dystrophy”, “myotonic dystrophy type 1” and “Steinert's disease” were used, without specifying a date. Published bibliographic references were also used. The most recent articles of the highest scientific quality in Spanish, English and French were selected.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Using the reviewed articles as a basis, the guidelines were elabourated by working subgroups who were each in charge of one of the corresponding subjects: diagnosis, motor and cognitive disorders, cardiac disorders, respiratory disorders, other disorders and DM1 in children. Each subgroup developed an initial document that was discussed and agreed upon at the <span class="elsevierStyleItalic">ad hoc</span> meeting held in the Spring Meeting of the Neuromuscular Diseases Study Group, in Santiago de Compostela, on April 29, 2017. Subsequently, several conversations were held to provide a definitive format to the guide.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The authors represent the national expert committee on DM1, that is, a panel of doctors from different specialities with extensive clinical experience in the disease. The authors are responsible for the interpretation presented in this review. All the authors have approved the final version of this manuscript and are fully responsible for its content.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The AGREE II tool<a class="elsevierStyleCrossRef" href="#bib0760"><span class="elsevierStyleSup">4</span></a> has been used to evaluate and guarantee the quality, clarity, stringency, applicability and editorial independence of this guide.</p><p id="par0040" class="elsevierStylePara elsevierViewall">The recommendations are summarized in <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and a proposal regarding the periodicity of the evaluations in <a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="tbl0010"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Diagnosis</span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Creatine kinase levels</span><p id="par0045" class="elsevierStylePara elsevierViewall">Patients with typical forms of the disease may present a slight increase of CK levels. In asymptomatic individuals, the CK levels are usually normal.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Neurophysiological study</span><p id="par0050" class="elsevierStylePara elsevierViewall">The electromyogram examination detects typical myotonic discharges. These consist of positive sharp waves or fibrillations, with decreasing frequency and amplitude, which provides a characteristic appearance and sound, like that of a diving aeroplane. The electromyographic pattern shows myopathic traits, with polyphasic, low amplitude potentials and an early interferential pattern. Although these changes can be observed in any muscle, they are more evident in the distal muscles.</p><p id="par0055" class="elsevierStylePara elsevierViewall">In the electroneurogram examination, a reduction in the amplitude of the composite motor evoked potential is observed.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The short exercise test (SET) shows a significant decline of compound motor action potential immediately after effort, similar to that which happens in the channelopathies when there are changes in the chloride channel gene (Thomsen and Becker myotonias). This is not surprising, given the assumption that the DM1 myotonia occurs through transcriptional interference of the chloride channel gene (CLCN-1), mutated in the congenital myotonias. This finding helps to differentiate DM1 from DM2 (proximal myotonic myopathy or PROMM), in which no change in the amplitude of the motor evoked potential is observed after effort.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Muscle biopsy</span><p id="par0065" class="elsevierStylePara elsevierViewall">Muscle biopsy has never been a key procedure for the diagnosis of DM1. There is no pathognomonic histopathologic data. However, the association of a large number of central nuclei, pyknotic nuclear clumps, sarcoplasmic masses, the existence of ring fibres and moth-eaten fibres, and selective type 1 fibre atrophy is very suggestive of Steinert myotonic dystrophy. These findings are similar to those found in DM2, except type 2 fibre atrophy predominate in DM2, contrary to the occurrence in DM1.</p><p id="par0070" class="elsevierStylePara elsevierViewall">In any case, given the accessibility and specificity of the genetic diagnosis, it is not necessary to perform a muscle biopsy in a patient with clinical suspicion of DM1.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Muscular magnetic resonance imaging</span><p id="par0075" class="elsevierStylePara elsevierViewall">Despite the fact that muscle magnetic resonance imaging (MRI) has been implanted as a fundamental diagnostic procedure in the study of neuromuscular diseases, there is little experience with this technique in DM1, due to the expressivity of the clinical features and the accessibility of genetic diagnosis. Literature has published the involvement of the flexor digitorum profundus muscles, as well as the flexor digitorum superficialis, flexor pollicis longus, extensor pollicis, abductor pollicis brevis, the lateral head of the triceps brachii and the infraspinatus in the upper limbs. In the lower limbs initial involvement is seen in the tibialis anterior and, later, in the semimembranosus, vastus intermedius and medial gastrocnemius muscles. Early involvement of the paravertebral musculature may also be detected. There is a good correlation between the clinical expression and the changes found in the muscle MRI.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Genetic diagnosis</span><p id="par0080" class="elsevierStylePara elsevierViewall">The diagnostic confirmation of DM1 is achieved through the detection of the genetic alteration produced by the disease. This consists of an expansion of the CTG triplet repeat in the 3’ untranslated region of the <span class="elsevierStyleItalic">DMPK</span> gene on the long arm of chromosome 19 (19q13.3).</p><p id="par0085" class="elsevierStylePara elsevierViewall">Normal individuals have between 5 and 34 repeats of the CTG triplet. People who have between 35 and 49 repeats (premutations) have no symptoms of the disease, but they can transmit a greater number of repeats to their offspring, which may already reach the mutation range. Alleles with 50 or more repeats are considered mutated and the person carrying it is affected with the disease, with a severity that correlates with the number of triplets. In general, the greater the number of CTG repeats, the earlier the disease begins and it manifests with a greater number of more intense symptoms. The genotype-phenotype correlation is greater when there are less than 400 repeats. Above this number, the instability in mitotic transmission produces errors in the transmission of the cell fragment to daughters, meaning that each individual is a mosaic, with different expansions in each tissue. For this reason, the number of triplets measured in lymphocytes may not be proportional to that found in other tissues, such as muscle. In fact, the number of CTG in skeletal muscle is usually between 2 and 13 times greater than that found in leukocytes.</p><p id="par0090" class="elsevierStylePara elsevierViewall">Usually, patients with a number of repeats between 50 and 100 CTG have mild forms of the disease, with cataracts at 50–60 years of age and/or mild myotonia. Patients with the classic form of the disease usually have between 100 and 1000 CTG, with the phenotype being more severe the greater the expansion. Finally, most congenital myotonic dystrophies have expansions above 1000 CTG. However, the correlation is not perfect and more severe or milder phenotypes may be seen than would seem to correspond to the range of CTG repeats indicated. In addition, the size of the expansion may change with age, so the closest genotype-phenotype correlation is observed when the clinical evaluation is close in time to the determination of the number of CTG repeats.</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Detection of the CTG expansion</span><p id="par0095" class="elsevierStylePara elsevierViewall">The study of the CTG fragment expansion is made from a sample of 10<span class="elsevierStyleHsp" style=""></span>ml of blood in EDTA. The determination of the size of this fragment in other tissues such as muscle does not contribute anything to the diagnosis.</p><p id="par0100" class="elsevierStylePara elsevierViewall">Expansions of up to 180 CTG repeats are detected using <span class="elsevierStyleItalic">polymerase chain reaction</span> (PCR). The <span class="elsevierStyleItalic">Triple-repeat Primed-PCR</span> (TP-PCR) is a cheap technique that detects if the number of CTG repeats is in the normal or pathological range, but it does not quantify them. To determine the number of repeats above 180 CTG it is necessary to use the Southern Blot analysis.</p><p id="par0105" class="elsevierStylePara elsevierViewall">The molecular study detects practically 100% of the pathogenic variants, so that the sensitivity and specificity of the genetic study are close to 100%. It is not sufficient to indicate whether the fragment expansion is greater or less than 50 CTG. <span class="elsevierStyleItalic">It is essential</span><span class="elsevierStyleItalic">to quantify the number of CTG repeats because of the prognostic value of this determination</span>.</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0110">Genetic counselling. Anticipation</span><p id="par0110" class="elsevierStylePara elsevierViewall">DM1 is transmitted in an inherited autosomal dominant pattern. Therefore, the risk that each child of a patient has of inheriting the mutation is 50%. Penetrance is very high, nearly 100% at 50 years of age, when all the clinical manifestations of the disease are sought.</p><p id="par0115" class="elsevierStylePara elsevierViewall">The severity of the child's phenotype will depend on the size of the CTGn fragment he inherits. Since the transmission of this fragment is unstable, with a tendency to increase the number of CTG triplets that pass to the offspring, the children that inherit the mutation usually present more severe forms than their parents, a phenomenon known as clinical anticipation. Expansions between 50 and 80 CTG can be transmitted over several generations without major changes. However, alleles of this size have greater instability when the parent is a male. Outside this range, maternal transmission is usually associated with a greater intergenerational jump, and the increase in the number of CTG repeats can be so massive that it reaches the range of congenital DM1. The risk of having a child with congenital DM increases the greater the maternal expansion is, especially when it is above 300 CTG, but this possibility exists whenever a woman passes the mutation to her offspring, even when its expansion is not very large. Even a woman who is asymptomatic or with a minimal form of the disease can have a child with the congenital variant.</p><p id="par0120" class="elsevierStylePara elsevierViewall">It is rarer for contractions to occur in the length of the CTG fragment that is transmitted. This phenomenon occurs more frequently when the transmitter is a male with large fragments, higher than 500 CTG.</p><p id="par0125" class="elsevierStylePara elsevierViewall">Whenever a DM1 patient is diagnosed, the risk the family members have of suffering from the disease should be explained, even if the patient does not present any symptoms at that moment, and a genetic study should be put forward for all those over 18 years of age. Pre-symptomatic diagnosis in children under 18 is considered inappropriate, given that there is no treatment for the disease, it denies the autonomy of the individual to decide what they want or do not want to know, it poses a risk of stigmatization and discrimination, and it can generate anxiety in the child and overprotection by parents. The need for adequate genetic counselling is especially important in cases of women at risk who are of childbearing age, because of the possibility of having children with congenital forms of the disease. In any case, the early detection of all carriers of the mutation is of great importance, given the risk of suffering serious complications due to the disease, especially in the area of cardiology. Before conducting the test, it is advisable to interview the individual at risk and explain the characteristics of the disease and its transmission.</p></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0115">Family planning. Prenatal and preimplantation diagnosis</span><p id="par0130" class="elsevierStylePara elsevierViewall">There exists the possibility of carrying out a diagnosis on the embryo or even selecting healthy <span class="elsevierStyleItalic">in vitro</span> embryos for subsequent implantation. These options must be analyzed and offered prior to pregnancy.</p><p id="par0135" class="elsevierStylePara elsevierViewall">The prenatal diagnosis consists in conducting a genetic study on a chorionic villi sample extracted between 9 and 12 weeks of gestation. It can also be carried out by means of amniocentesis in week 16. These procedures have a 3–4% risk of causing an abortion.</p><p id="par0140" class="elsevierStylePara elsevierViewall">Preimplantation diagnosis is an option that could avoid this risk of abortion, which may have great relevance in a disease in which fertility is reduced and is already associated with a high risk of spontaneous abortion. In addition, it some ways it avoids the ethical problems that a therapeutic abortion may imply for some people. However, ovarian stimulation and egg extraction are procedures that demand great effort from the woman. It is advisable to start this procedure before the ovarian reserve falls due to natural causes (when the patient is approximately 38 years old) or due to the disease itself. On the other hand, the pregnancy rate achieved is relatively low and, often, it is necessary to repeat the process several times in order to reach the birth of a child. In addition, confirmation by means of chorionic villus sampling is frequently required, to ensure that the embryo is not a carrier of the mutation, making the risk of abortion associated with the procedure to be the same as that of prenatal diagnosis.</p></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0120">Diagnostic protocol</span><p id="par0145" class="elsevierStylePara elsevierViewall">When there is a clinical suspicion that a patient has DM1, the diagnostic procedure of choice is the genetic study. The rest of the mentioned techniques may be of interest from the investigative point of view, but they are not necessary for the realization of the diagnosis. It should be noted however that normal results for the other tests including the electromyogram, do not exclude the existence of the disease.</p><p id="par0150" class="elsevierStylePara elsevierViewall">Even when the existence of DM1 in a family is not known, in the presence of a hypotonic newborn, or if the ultrasound in the second or third trimester of gestation detects polyhydramnios or a lack of foetal movements, the possibility of DM1 should be considered and the realization of a genetic test in the child and his parents should be put forward.</p><p id="par0155" class="elsevierStylePara elsevierViewall">Due to the complexity of the disease, it is recommended that patients with DM1 be monitored in a unit of neuromuscular diseases with experience in the management of DM1 and with access to multiple specialities. Each specialist who participates in the healthcare of these patients must have adequate knowledge about DM1.</p></span></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0125">Neurological involvement</span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0130">Muscle problems</span><span id="sec0075" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0135">Weakness</span><p id="par0160" class="elsevierStylePara elsevierViewall">The weakness resulting from DM1 is predominant in the distal limbs; however it can also affect the muscles of the neck and face, as well as the movements of chewing, swallowing and phonation. It has a progressive course that is estimated at 1–3% per year.<a class="elsevierStyleCrossRef" href="#bib0765"><span class="elsevierStyleSup">5</span></a> The risk of falls and fractures increases.</p><p id="par0165" class="elsevierStylePara elsevierViewall">Various scales are used to quantify the weakness.</p><p id="par0170" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">i)</span><p id="par0175" class="elsevierStylePara elsevierViewall">The <span class="elsevierStyleItalic">Muscular Impairment Rating Scale</span> (MIRS)<a class="elsevierStyleCrossRef" href="#bib0770"><span class="elsevierStyleSup">6</span></a> is a validated scale to know the degree of muscle weakness. It evaluates 11 muscles manually with a scale of 1 (no weakness) to 5 (significant weakness). The muscle groups are:<ul class="elsevierStyleList" id="lis0010"><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">a.</span><p id="par0180" class="elsevierStylePara elsevierViewall">Neck flexors</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">b.</span><p id="par0185" class="elsevierStylePara elsevierViewall">Bilateral shoulder abductors</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">c.</span><p id="par0190" class="elsevierStylePara elsevierViewall">Bilateral elbow flexors</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">d.</span><p id="par0195" class="elsevierStylePara elsevierViewall">Bilateral wrist extensors</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">e.</span><p id="par0200" class="elsevierStylePara elsevierViewall">Bilateral finger flexors</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">f.</span><p id="par0205" class="elsevierStylePara elsevierViewall">Bilateral hip flexors</p></li><li class="elsevierStyleListItem" id="lsti0040"><span class="elsevierStyleLabel">g.</span><p id="par0210" class="elsevierStylePara elsevierViewall">Bilateral knee extension</p></li><li class="elsevierStyleListItem" id="lsti0045"><span class="elsevierStyleLabel">h.</span><p id="par0215" class="elsevierStylePara elsevierViewall">Bilateral knee flexors</p></li><li class="elsevierStyleListItem" id="lsti0050"><span class="elsevierStyleLabel">i.</span><p id="par0220" class="elsevierStylePara elsevierViewall">Bilateral ankle dorsiflexors</p></li><li class="elsevierStyleListItem" id="lsti0055"><span class="elsevierStyleLabel">j.</span><p id="par0225" class="elsevierStylePara elsevierViewall">Bilateral ankle plantar flexion</p></li></ul></p></li><li class="elsevierStyleListItem" id="lsti0060"><span class="elsevierStyleLabel">ii)</span><p id="par0230" class="elsevierStylePara elsevierViewall">The <span class="elsevierStyleItalic">Medical Research Council</span> (MRC) scale</p></li><li class="elsevierStyleListItem" id="lsti0065"><span class="elsevierStyleLabel">iii)</span><p id="par0235" class="elsevierStylePara elsevierViewall">Use of a grip-strength hand dynamometer</p></li><li class="elsevierStyleListItem" id="lsti0070"><span class="elsevierStyleLabel">iv)</span><p id="par0240" class="elsevierStylePara elsevierViewall">Functional tests<a class="elsevierStyleCrossRef" href="#bib0775"><span class="elsevierStyleSup">7</span></a>:<ul class="elsevierStyleList" id="lis0015"><li class="elsevierStyleListItem" id="lsti0075"><span class="elsevierStyleLabel">•</span><p id="par0245" class="elsevierStylePara elsevierViewall">6<span class="elsevierStyleHsp" style=""></span>minute walk test (distance)</p></li><li class="elsevierStyleListItem" id="lsti0080"><span class="elsevierStyleLabel">•</span><p id="par0250" class="elsevierStylePara elsevierViewall">10<span class="elsevierStyleHsp" style=""></span>m walk/run test (timed test)</p></li><li class="elsevierStyleListItem" id="lsti0085"><span class="elsevierStyleLabel">•</span><p id="par0255" class="elsevierStylePara elsevierViewall">Go up 4 steps (timed test)</p></li><li class="elsevierStyleListItem" id="lsti0090"><span class="elsevierStyleLabel">•</span><p id="par0260" class="elsevierStylePara elsevierViewall">Go down 4 steps (timed test)</p></li><li class="elsevierStyleListItem" id="lsti0095"><span class="elsevierStyleLabel">•</span><p id="par0265" class="elsevierStylePara elsevierViewall">Get up off the ground (timed test)</p></li></ul></p></li><li class="elsevierStyleListItem" id="lsti0100"><span class="elsevierStyleLabel">v)</span><p id="par0270" class="elsevierStylePara elsevierViewall">Questions regarding the following should always be asked:<ul class="elsevierStyleList" id="lis0020"><li class="elsevierStyleListItem" id="lsti0105"><span class="elsevierStyleLabel">•</span><p id="par0275" class="elsevierStylePara elsevierViewall">Limitations for walking</p></li><li class="elsevierStyleListItem" id="lsti0110"><span class="elsevierStyleLabel">•</span><p id="par0280" class="elsevierStylePara elsevierViewall">Falls</p></li><li class="elsevierStyleListItem" id="lsti0115"><span class="elsevierStyleLabel">•</span><p id="par0285" class="elsevierStylePara elsevierViewall">Limitations with transferring from one place to another</p></li><li class="elsevierStyleListItem" id="lsti0120"><span class="elsevierStyleLabel">•</span><p id="par0290" class="elsevierStylePara elsevierViewall">Use and convenience of a wheelchair</p></li></ul></p></li></ul></p><span id="sec0080" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0140">Treatment</span><p id="par0295" class="elsevierStylePara elsevierViewall">Aerobic exercise and moderate-intensity strength training do not cause harm, although they have not proven useful.<a class="elsevierStyleCrossRef" href="#bib0780"><span class="elsevierStyleSup">8</span></a> Moderate exercise is recommended to all patients.<a class="elsevierStyleCrossRef" href="#bib0765"><span class="elsevierStyleSup">5</span></a> Excessive exercise may accelerate the progression of the disease and is discouraged. It has been proven that those affected by DM1 fall and stumble up to 10 times more than the general population<a class="elsevierStyleCrossRef" href="#bib0785"><span class="elsevierStyleSup">9</span></a> with the consequent risk of fractures, so the possibility of using an antiechial orthosis to prevent these accidents must be assessed.<a class="elsevierStyleCrossRef" href="#bib0790"><span class="elsevierStyleSup">10</span></a></p></span></span><span id="sec0085" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0145">Myotonia</span><p id="par0300" class="elsevierStylePara elsevierViewall">Myotonia is a disorder of muscle relaxation after voluntary contraction; patients usually explain it as stiffness; myotonia improves with heat and with repeated exercise and worsens with rest and cold. It is a very frequent symptom but it produces few symptoms and it is not always necessary to treat it. It can favour the appearance of muscular rigidity, dysarthria, dysphagia, pain and gastrointestinal symptoms.</p><span id="sec0090" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0150">Treatment</span><p id="par0305" class="elsevierStylePara elsevierViewall">Mexiletine at a dose of 100<span class="elsevierStyleHsp" style=""></span>mg or 200<span class="elsevierStyleHsp" style=""></span>mg 3 times a day has shown effectiveness in the myotonia of DM1, with Class 1 evidence.<a class="elsevierStyleCrossRef" href="#bib0795"><span class="elsevierStyleSup">11</span></a> Mexiletine and other antiarrhythmic drugs should be used for myotonia with caution, due to the blocking effect on cardiac sodium channels (with functional alterations in DM1) since they act on the excitability of these channels, with the consequential impact on both cardiac arrhythmias as well as atrioventricular conduction.<a class="elsevierStyleCrossRefs" href="#bib0800"><span class="elsevierStyleSup">12–15</span></a></p><p id="par0310" class="elsevierStylePara elsevierViewall">Other treatments that have been used are phenytoin, carbamazepine, clomipramine, imipramine, amitriptyline, nifedipine, flecainide, acetazolamide and taurine. The precautions recommended with mexiletine are transferable to other drugs.<a class="elsevierStyleCrossRef" href="#bib0820"><span class="elsevierStyleSup">16</span></a></p></span></span><span id="sec0095" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0155">Myalgia</span><p id="par0315" class="elsevierStylePara elsevierViewall">Pain is a very common symptom that can affect 90% of patients, and therefore the patient should always be asked about it, and treated with the appropriate analgesic drugs.<a class="elsevierStyleCrossRef" href="#bib0825"><span class="elsevierStyleSup">17</span></a></p><span id="sec0100" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0160">Treatment</span><p id="par0320" class="elsevierStylePara elsevierViewall">Pregabalin can be tried at low doses 50–75<span class="elsevierStyleHsp" style=""></span>mg/day.</p></span></span><span id="sec0105" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0165">Fatigue</span><p id="par0325" class="elsevierStylePara elsevierViewall">It is a frequent symptom in myotonic dystrophy and is without a clear relationship with muscle weakness.<a class="elsevierStyleCrossRef" href="#bib0830"><span class="elsevierStyleSup">18</span></a> There are no specific scales for DM1 validated in Spanish, but a fatigue intensity scale can be used.<a class="elsevierStyleCrossRef" href="#bib0835"><span class="elsevierStyleSup">19</span></a></p><span id="sec0110" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0170">Treatment</span><p id="par0330" class="elsevierStylePara elsevierViewall">Modafinil 200<span class="elsevierStyleHsp" style=""></span>mg/day is recommended during the first week, and the dose can be doubled if the effect is insufficient.<a class="elsevierStyleCrossRef" href="#bib0840"><span class="elsevierStyleSup">20</span></a> However, this indication is not included in the Summary of Product Characteristics.</p></span></span></span><span id="sec0115" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0175">Central nervous system problems</span><p id="par0335" class="elsevierStylePara elsevierViewall">The participation of the central nervous system in DM1 can become one of the biggest problems for a patient's day-to-day life. The highly variable manifestations include cognitive deficit, apathy, fatigue, sleep disturbances and, in DM1 of neonatal onset, it includes mental retardation, ADHD and difficulty for executive functions.<a class="elsevierStyleCrossRefs" href="#bib0845"><span class="elsevierStyleSup">21,22</span></a></p><span id="sec0120" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0180">Intellectual retardation</span><p id="par0340" class="elsevierStylePara elsevierViewall">It is very evident in the congenital forms, but it can also be observed in the other forms, to a variable degree, although some cases show a normal IQ.<a class="elsevierStyleCrossRef" href="#bib0855"><span class="elsevierStyleSup">23</span></a> Patients with DM1 have a statistically lower level of education than the control groups, with a lower socio-economic status, which is more noticeable in males.<a class="elsevierStyleCrossRef" href="#bib0860"><span class="elsevierStyleSup">24</span></a></p><p id="par0345" class="elsevierStylePara elsevierViewall">A progressive deficit of memory has been demonstrated, even in patients with a relatively small expansion.<a class="elsevierStyleCrossRefs" href="#bib0865"><span class="elsevierStyleSup">25,26</span></a></p><p id="par0350" class="elsevierStylePara elsevierViewall">There is currently no general consensus on the tests that can be performed to study the cognitive deficit in DM1. An accumulation of screening test has been proposed,<a class="elsevierStyleCrossRef" href="#bib0860"><span class="elsevierStyleSup">24</span></a> which includes the following: (1) <span class="elsevierStyleItalic">An abbreviated form of the Wechsler Adult Intelligence Scale</span> (WAIS test)<a class="elsevierStyleCrossRef" href="#bib0875"><span class="elsevierStyleSup">27</span></a> to estimate the intellectual quotient, or the corresponding test for those of a young age: <span class="elsevierStyleItalic">Wechsler Intelligence Scale for Children</span> (WISC) and <span class="elsevierStyleItalic">Wechsler Prescholar and Primary Scale Intelligence</span> (WPPSI); (2) <span class="elsevierStyleItalic">Rey's Auditory Verbal Learning Test</span> (RAVLT test), to assess verbal memory; (3) Rey's Complex Figure, which explores the visual memory; (4) WAIS IV test – the digits and cubes part, to evaluate the attention and speed of processing. When applying these tests, some factors that could possibly lead to confusion should be taken into consideration, such as weakness, myotonia, fatigue, depression, etc. For this reason, these tests are applicable in selected cases or in academic studies.</p><p id="par0355" class="elsevierStylePara elsevierViewall">Language disorders, intellectual retardation, neurocognitive dysfunction, ADHD and autism spectrum disorders are frequent in the congenital form.<a class="elsevierStyleCrossRefs" href="#bib0880"><span class="elsevierStyleSup">28,29</span></a></p><p id="par0360" class="elsevierStylePara elsevierViewall">It has recently been suggested that cognitive behavioural therapy could improve fatigue and participation in social activities of patients with DM1.<a class="elsevierStyleCrossRef" href="#bib0890"><span class="elsevierStyleSup">30</span></a></p></span><span id="sec0125" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0185">Sleep disorders</span><p id="par0365" class="elsevierStylePara elsevierViewall">Sometimes this is the first reason for consultation. Diurnal hypersomnia manifests in one third of cases, especially after meals,<a class="elsevierStyleCrossRefs" href="#bib0845"><span class="elsevierStyleSup">21,31</span></a> with difficulty staying awake, and periodic movements during sleep have been described.<a class="elsevierStyleCrossRef" href="#bib0900"><span class="elsevierStyleSup">32</span></a> Sleep apnoea/hypopnoea syndrome (SAHS) is very common. However, diurnal hypersomnia does not relate well to SAHS nor does it always improve with treatment.</p><p id="par0370" class="elsevierStylePara elsevierViewall">The Epworth test is a good tool for diagnosing these disorders.<a class="elsevierStyleCrossRef" href="#bib0905"><span class="elsevierStyleSup">33</span></a></p></span><span id="sec0130" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0190">Depression and personality disorders</span><p id="par0375" class="elsevierStylePara elsevierViewall">Although psychiatric problems are not usually severe, major depression and personality alterations give patients with DM1 special psychological characteristics.<a class="elsevierStyleCrossRef" href="#bib0910"><span class="elsevierStyleSup">34</span></a> They often show features of schizoid personality disorder, anxiety, hysteria, compulsion, depressive neurosis, self-destructive or narcissistic character, lack of initiative and apathy.<a class="elsevierStyleCrossRefs" href="#bib0860"><span class="elsevierStyleSup">24,35,36</span></a></p><p id="par0380" class="elsevierStylePara elsevierViewall">Depression is usually assessed using the Hamilton scale.<a class="elsevierStyleCrossRef" href="#bib0925"><span class="elsevierStyleSup">37</span></a></p></span><span id="sec0135" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0195">Cerebrovascular disease</span><p id="par0385" class="elsevierStylePara elsevierViewall">The incidence of stroke in DM1 has not been well studied and it is related to the presence of cardioembolic arrhythmias.<a class="elsevierStyleCrossRefs" href="#bib0930"><span class="elsevierStyleSup">38–40</span></a></p></span><span id="sec0140" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0200">Neuroimaging</span><p id="par0390" class="elsevierStylePara elsevierViewall">The disorders of the central nervous system seem to have a certain correlation with histopathological studies<a class="elsevierStyleCrossRef" href="#bib0945"><span class="elsevierStyleSup">41</span></a> and with neuroimaging findings. Thus, decreased grey matter volume and diffuse disruption of white matter have been demonstrated, in parallel with the size of the CTG triplet expansion.<a class="elsevierStyleCrossRefs" href="#bib0845"><span class="elsevierStyleSup">21,42–44</span></a></p><p id="par0395" class="elsevierStylePara elsevierViewall">FDG-PET studies can demonstrate bilateral frontotemporal hypometabolism, although no correlation with neuropsychological deficit has been demonstrated.<a class="elsevierStyleCrossRef" href="#bib0950"><span class="elsevierStyleSup">42</span></a></p></span></span></span><span id="sec0145" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0205">Cardiac involvement</span><p id="par0400" class="elsevierStylePara elsevierViewall">Cardiac involvement is a frequent and important event in DM1. It is estimated that 75–80% of patients present some degree of cardiac involvement with a variable clinical spectrum, from mild electrocardiogram (ECG) alterations to severe arrhythmias that cause sudden death. In fact, up to a third of the deaths in these patients are explained by causes of cardiac origin.</p><p id="par0405" class="elsevierStylePara elsevierViewall">In a Danish national registry, which included 1186 patients with DM1, a special incidence of arrhythmias, cardiomyopathy and heart failure was found.<a class="elsevierStyleCrossRef" href="#bib0965"><span class="elsevierStyleSup">45</span></a> The pathophysiological basis of these disorders seems to reside in fibrofatty tissue replacing the specialized cardiac and muscular conduction tissue in both ventricles.<a class="elsevierStyleCrossRefs" href="#bib0970"><span class="elsevierStyleSup">46–48</span></a></p><p id="par0410" class="elsevierStylePara elsevierViewall">Although there are contradictory data, it has not been possible to demonstrate a clear relationship between the number of CTG repeats or the neuromuscular involvement, and the cardiac events.<a class="elsevierStyleCrossRef" href="#bib0975"><span class="elsevierStyleSup">47</span></a> Given that the risk of cardiac involvement is high from childhood onwards<a class="elsevierStyleCrossRef" href="#bib0985"><span class="elsevierStyleSup">49</span></a> it is essential to perform early detection tests of these complications throughout a patients life, even in patients without involvement or with minimal muscle involvement.</p><span id="sec0150" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0210">Sudden death, arrhythmias and conduction disorders</span><p id="par0415" class="elsevierStylePara elsevierViewall">The most frequent cardiac manifestations of DM1 are arrhythmias and electrocardiogram changes. The conduction system is affected primarily at the level of the His-Purkinje system, but it can also be affected at other levels (sinoatrial and atrioventricular node).<a class="elsevierStyleCrossRefs" href="#bib0765"><span class="elsevierStyleSup">5,6</span></a> In a recent meta-analysis<a class="elsevierStyleCrossRef" href="#bib0975"><span class="elsevierStyleSup">47</span></a> it is pointed out that the most common disorders are, in order of frequency, atrioventricular block grade 1 (ABV1) (28.2%), QTc (22%) and QRS (19.9%), ventricular premature contractions (14.6%), atrial fibrillation/flutter (5%), left bundle branch block (5.7%), right bundle branch block (4.4%) and non-sustained ventricular tachycardia (4.1%).</p><p id="par0420" class="elsevierStylePara elsevierViewall">Arrhythmias can cause palpitations, syncope and sudden death, although occasionally the patient may remain clinically asymptomatic. The risk of sudden death in patients with DM1 is estimated at 0.56% per year.<a class="elsevierStyleCrossRef" href="#bib0975"><span class="elsevierStyleSup">47</span></a> There are contradictory data regarding the mechanisms underlying this situation. Traditionally, bradyarrhythmias that trigger ventricular asystole or fibrillation have been reported as the main cause of death. It is known that asymptomatic patients with DM1 and with an HV interval<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>70<span class="elsevierStyleHsp" style=""></span>ms, measured in an electrophysiological study (EPS) have a high risk of developing a complete AVB and would benefit from the prophylactic implantation of a pacemaker.<a class="elsevierStyleCrossRef" href="#bib0990"><span class="elsevierStyleSup">50</span></a> However, there seem to be other causes of sudden death that cannot be avoided with pacemakers.<a class="elsevierStyleCrossRef" href="#bib0990"><span class="elsevierStyleSup">50</span></a> Tachyarrhythmias, especially ventricular tachyarrhythmias, have been referred to as causes of sudden death.<a class="elsevierStyleCrossRef" href="#bib0995"><span class="elsevierStyleSup">51</span></a></p><p id="par0425" class="elsevierStylePara elsevierViewall">The study by Groh et al. identified predictors of sudden death to include a history of an atrial tachyarrhythmia and the existence of a severe abnormality on the ECG defined as: second-degree or third-degree AVB, a PR<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>240<span class="elsevierStyleHsp" style=""></span>ms or a QRS<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>120<span class="elsevierStyleHsp" style=""></span>ms.<a class="elsevierStyleCrossRef" href="#bib1000"><span class="elsevierStyleSup">52</span></a> More recently, a retrospective study of a cohort of patients with DM1 and basal ECG abnormalities (PR<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>200<span class="elsevierStyleHsp" style=""></span>ms and/or QRS<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>100<span class="elsevierStyleHsp" style=""></span>ms) was published which showed that an invasive strategy with electrophysiological study and prophylactic pacing in patients with HV<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>70<span class="elsevierStyleHsp" style=""></span>ms was associated with longer survival during follow-up compared to those who were managed with a non-invasive strategy.<a class="elsevierStyleCrossRef" href="#bib1005"><span class="elsevierStyleSup">53</span></a></p></span><span id="sec0155" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0215">Myocardial involvement</span><p id="par0430" class="elsevierStylePara elsevierViewall">Hypertrophy, and left ventricular dilatation and systolic dysfunction have been observed in a variable percentage of patients with DM1 (7–20%).<a class="elsevierStyleCrossRefs" href="#bib0975"><span class="elsevierStyleSup">47,53</span></a> Older age, male gender, ECG conduction abnormalities and the existence of arrhythmias are predictors of systolic dysfunction.<a class="elsevierStyleCrossRef" href="#bib1010"><span class="elsevierStyleSup">54</span></a> This entity, which initially is usually sub-clinical, increases the risk of sudden death. Likewise, a greater prevalence of left ventricle (LV) non-compaction and of myocardial fibrosis detectable by delayed myocardial enhancement on the cardiac MRI has been described.<a class="elsevierStyleCrossRef" href="#bib1015"><span class="elsevierStyleSup">55</span></a> Additionally, in patients with Steinert's disease, a reduction in myocardial mass and right ventricular dysfunction has been reported, as well as a high prevalence of mitral valve prolapse. Finally, the existence of changes in the myocardial relaxation analogous to the myotonia of skeletal muscle that may manifest in symptoms of heart failure should be considered.<a class="elsevierStyleCrossRef" href="#bib0980"><span class="elsevierStyleSup">48</span></a></p><p id="par0435" class="elsevierStylePara elsevierViewall">Recommendations<a class="elsevierStyleCrossRefs" href="#bib1020"><span class="elsevierStyleSup">56,57</span></a> (<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>):<ul class="elsevierStyleList" id="lis0025"><li class="elsevierStyleListItem" id="lsti0125"><span class="elsevierStyleLabel">1.</span><p id="par0440" class="elsevierStylePara elsevierViewall">Patients with DM1, even asymptomatic patients with no apparent cardiac involvement, must receive cardiological follow-up for life, looking out for possible complications, given the progressive nature of the disease.</p></li><li class="elsevierStyleListItem" id="lsti0130"><span class="elsevierStyleLabel">2.</span><p id="par0445" class="elsevierStylePara elsevierViewall">Patients with DM1 should receive information about the warning symptoms (syncope, palpitations) for which they should consult a professional urgently.</p></li><li class="elsevierStyleListItem" id="lsti0135"><span class="elsevierStyleLabel">3.</span><p id="par0450" class="elsevierStylePara elsevierViewall">A disease-specific anamnesis is recommended every year (paying special attention to the existence of syncope and/or palpitations), as well as an ECG and an ECG holter.</p></li><li class="elsevierStyleListItem" id="lsti0140"><span class="elsevierStyleLabel">4.</span><p id="par0455" class="elsevierStylePara elsevierViewall">A transthoracic echocardiogram is recommended every 3–5 years.</p></li><li class="elsevierStyleListItem" id="lsti0145"><span class="elsevierStyleLabel">5.</span><p id="par0460" class="elsevierStylePara elsevierViewall">The management of cardiac structural abnormalities is similar to that of the general population.</p></li><li class="elsevierStyleListItem" id="lsti0150"><span class="elsevierStyleLabel">6.</span><p id="par0465" class="elsevierStylePara elsevierViewall">Asymptomatic patients with significant ECG abnormalities (PR<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>200<span class="elsevierStyleHsp" style=""></span>ms, QRS<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>100<span class="elsevierStyleHsp" style=""></span>ms) or those with a history of cardiogenic syncope could benefit from performing an invasive EPS to measure the conduction times and the risk of ventricular arrhythmia induction. In the case of patients with a normal EPS this should be repeated during follow-up if new symptoms appear, or if there is significant progression of the basal ECG disorders (at least one increase<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>20<span class="elsevierStyleHsp" style=""></span>ms of the PR and/or QRS).</p></li><li class="elsevierStyleListItem" id="lsti0155"><span class="elsevierStyleLabel">7.</span><p id="par0470" class="elsevierStylePara elsevierViewall">Patients with HV<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>70<span class="elsevierStyleHsp" style=""></span>ms in the EPS or a second degree AVB in the ECG, due to the high rate of progression to a complete AVB, would benefit from the prophylactic implantation of a definitive pacemaker. The pacemaker does not completely prevent sudden death, because this can be produced by other mechanisms (ventricular arrhythmias, asystole).</p></li><li class="elsevierStyleListItem" id="lsti0160"><span class="elsevierStyleLabel">8.</span><p id="par0475" class="elsevierStylePara elsevierViewall">Implantable cardioverter-defibrillators should be considered in patients with DM1 and a history of ventricular arrhythmias and/or LV dysfunction. In patients with sustained monomorphic ventricular tachycardia, an EPS should be performed and, if a branch-branch re-entry mechanism is verified, catheter ablation should be performed.</p></li><li class="elsevierStyleListItem" id="lsti0165"><span class="elsevierStyleLabel">9.</span><p id="par0480" class="elsevierStylePara elsevierViewall">The management of supraventricular arrhythmias in patients with DM1 is similar to that of other groups of patients. However, special caution should be exercised in the use of antiarrhythmic drugs that can prolong the HV (especially those in group i).</p></li><li class="elsevierStyleListItem" id="lsti0170"><span class="elsevierStyleLabel">10.</span><p id="par0485" class="elsevierStylePara elsevierViewall">Sedation for electrical cardioversion in patients with DM1 should be performed with caution in an appropriate environment and by trained personnel, due to the risk of respiratory failure.</p></li></ul></p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia></span></span><span id="sec0160" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0220">Respiratory involvement</span><p id="par0490" class="elsevierStylePara elsevierViewall">Respiratory involvement in DM1 is frequent and constitutes one of the main causes of premature death in these patients: it accounts for 51–75% of deaths.<a class="elsevierStyleCrossRefs" href="#bib1030"><span class="elsevierStyleSup">58,59</span></a> It is also one of the factors that most influence the deterioration of the quality of life.<a class="elsevierStyleCrossRef" href="#bib0745"><span class="elsevierStyleSup">1</span></a> There seems to be a certain correlation between the size of the expansion and the intensity of the respiratory involvement.<a class="elsevierStyleCrossRefs" href="#bib1040"><span class="elsevierStyleSup">60,61</span></a> With respect to risk factors for respiratory insufficiency, some studies show an increased risk in men.<a class="elsevierStyleCrossRef" href="#bib1050"><span class="elsevierStyleSup">62</span></a> In addition, obesity, common in DM1, has been shown to be an independent risk factor for respiratory failure.<a class="elsevierStyleCrossRef" href="#bib1055"><span class="elsevierStyleSup">63</span></a></p><p id="par0495" class="elsevierStylePara elsevierViewall">In most cases, respiratory involvement is insidious and progressive, and as it goes unnoticed it is often diagnosed late; it generally occurs later in life (between 50 and 60 years) and in patients with a previously established diagnosis of DM1, with muscular and multi-system symptoms.<a class="elsevierStyleCrossRef" href="#bib1060"><span class="elsevierStyleSup">64</span></a> However, there are cases in which respiratory insufficiency can appear acutely, triggered by different processes such as an anaesthetic procedure or a respiratory infection.<a class="elsevierStyleCrossRef" href="#bib1065"><span class="elsevierStyleSup">65</span></a></p><p id="par0500" class="elsevierStylePara elsevierViewall">The physiopathogenic mechanisms of the respiratory disorder in these patients continue to be poorly understood, but there are many studies that support the hypothesis of a double mechanism: peripheral (due to the dystrophic and myotonic muscular involvement of the muscles involved in breathing [diaphragm and abdominal and intercostal musculature]) and central (due to anomalies of the central nervous system that result in a change to the central control of respiration).<a class="elsevierStyleCrossRefs" href="#bib1070"><span class="elsevierStyleSup">66,67</span></a></p><p id="par0505" class="elsevierStylePara elsevierViewall">Respiratory involvement of patients with DM1 may be related to restrictive ventilatory disturbances due to muscle weakness or the presence of SAHS or nocturnal hypoventilation. Therefore, the most frequent clinical manifestations that suggest respiratory affectation are recurrent respiratory infections, progressive dyspnoea, diurnal hypersomnolence and the morning headache.<a class="elsevierStyleCrossRefs" href="#bib1070"><span class="elsevierStyleSup">66,68–71</span></a></p><p id="par0510" class="elsevierStylePara elsevierViewall">The behavioural and cognitive affectation of patients with DM1 can hinder the correct identification of the respiratory symptoms and a bad adhesion to the nocturnal respiratory therapies.<a class="elsevierStyleCrossRef" href="#bib1100"><span class="elsevierStyleSup">72</span></a></p><p id="par0515" class="elsevierStylePara elsevierViewall">Weakness of the respiratory muscles that causes a restrictive ventilatory alteration can lead to hypoventilation, initially only at night.</p><p id="par0520" class="elsevierStylePara elsevierViewall">The weakness of the expiratory muscles (intercostal and abdominal) increases the risk of respiratory infections due to decreased coughing capacity and dysphagia. The risk of pneumonia is also increased due to an increased risk of bronchoaspiration as a result of oropharyngeal and oesophageal weakness.<a class="elsevierStyleCrossRefs" href="#bib1105"><span class="elsevierStyleSup">73,74</span></a></p><p id="par0525" class="elsevierStylePara elsevierViewall">The physiopathogenic and clinical heterogeneity of the respiratory affectation in patients affected by DM1 makes it necessary to protocolise the screening studies for this complication.</p><p id="par0530" class="elsevierStylePara elsevierViewall">Recommendations (<a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>):<ul class="elsevierStyleList" id="lis0030"><li class="elsevierStyleListItem" id="lsti0175"><span class="elsevierStyleLabel">1.</span><p id="par0535" class="elsevierStylePara elsevierViewall">The assessment of respiratory dysfunction in patients with DM1 should be periodic.</p></li><li class="elsevierStyleListItem" id="lsti0180"><span class="elsevierStyleLabel">2.</span><p id="par0540" class="elsevierStylePara elsevierViewall">The respiratory history should include symptoms of dysphagia, cough efficacy, dyspnoea, diurnal hypersomnia, morning headaches and fatigue. The Epworth Sleepiness Scale is recommended.<a class="elsevierStyleCrossRef" href="#bib0905"><span class="elsevierStyleSup">33</span></a></p></li><li class="elsevierStyleListItem" id="lsti0185"><span class="elsevierStyleLabel">3.</span><p id="par0545" class="elsevierStylePara elsevierViewall">The initial study should include spirometry, plethysmography, maximal inspiratory and expiratory pressure measurements (MIP and MEP) or SNIP, peak expiratory cough flow (PECF), basal gasometry and sleep cardiorespiratory polygraph (CRP).</p></li><li class="elsevierStyleListItem" id="lsti0190"><span class="elsevierStyleLabel">4.</span><p id="par0550" class="elsevierStylePara elsevierViewall">A night polysomnograph (PSG) is recommended in case of diurnal hypersomnia with normal or pathological CRP and with no response to nocturnal ventilatory therapy.</p></li><li class="elsevierStyleListItem" id="lsti0195"><span class="elsevierStyleLabel">5.</span><p id="par0555" class="elsevierStylePara elsevierViewall">CPAP treatment is recommended if the SAHS is moderate or severe and there is an absence of nocturnal hypoventilation (CT90<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>30%).</p></li><li class="elsevierStyleListItem" id="lsti0200"><span class="elsevierStyleLabel">6.</span><p id="par0560" class="elsevierStylePara elsevierViewall">Treatment with non-invasive mechanical ventilation (BiPAP) is recommended if nocturnal hypoventilation predominates (CT90<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>30%).</p></li><li class="elsevierStyleListItem" id="lsti0205"><span class="elsevierStyleLabel">7.</span><p id="par0565" class="elsevierStylePara elsevierViewall">Initial intra- or extra-hospital training of nocturnal devices is recommended to improve adherence to treatment.</p></li><li class="elsevierStyleListItem" id="lsti0210"><span class="elsevierStyleLabel">8.</span><p id="par0570" class="elsevierStylePara elsevierViewall">Early antibiotic treatment is recommended in the case of respiratory infections.</p></li><li class="elsevierStyleListItem" id="lsti0215"><span class="elsevierStyleLabel">9.</span><p id="par0575" class="elsevierStylePara elsevierViewall">Teaching respiratory physiotherapy techniques and the use of mechanical assistance for coughing with insufflation/exsufflation patterns (PECF<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>270) for the management of secretions is recommended.</p></li><li class="elsevierStyleListItem" id="lsti0220"><span class="elsevierStyleLabel">10.</span><p id="par0580" class="elsevierStylePara elsevierViewall">Annual flu vaccinations, and a pneumococcal vaccine at least once in a lifetime.</p></li><li class="elsevierStyleListItem" id="lsti0225"><span class="elsevierStyleLabel">11.</span><p id="par0585" class="elsevierStylePara elsevierViewall">A respiratory evaluation should be performed before any surgery with general anaesthesia.</p></li></ul></p><elsevierMultimedia ident="tbl0020"></elsevierMultimedia></span><span id="sec0165" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0225">Affectation of other organs and systems</span><p id="par0590" class="elsevierStylePara elsevierViewall">DM1 can affect multiple organs and tissues, to varying degrees.</p><span id="sec0170" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0230">Dermatology</span><span id="sec0175" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0235">Multiple pilomatricomas (calcifying epithelioma of Malherbe)</span><p id="par0595" class="elsevierStylePara elsevierViewall">It is a benign tumour derived from the primitive cells of the hair follicle matrix that manifests as a subcutaneous, non-painful nodule of 0.5–5<span class="elsevierStyleHsp" style=""></span>cm in diameter. It can be located on the scalp, face, neck or upper limbs. It can be treated by surgical exeresis, when there is local discomfort or an aesthetic problem and it is confirmed by the histopathological diagnosis. It can be confused with simple sebaceous cysts.<a class="elsevierStyleCrossRefs" href="#bib1115"><span class="elsevierStyleSup">75,76</span></a></p></span><span id="sec0180" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0240">Alopecia</span><p id="par0600" class="elsevierStylePara elsevierViewall">It has multifactorial causes, due to accelerated skin and hair follicle ageing and hormonal changes. There are no specific therapeutic recommendations.<a class="elsevierStyleCrossRef" href="#bib1125"><span class="elsevierStyleSup">77</span></a></p></span><span id="sec0185" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0245">Seborrhoeic dermatitis</span><p id="par0605" class="elsevierStylePara elsevierViewall">It correlates with decreased serum vitamin D values. The diagnosis is made by a dermatological examination and serum vitamin D analysis. It can be improved with calcifediol.<a class="elsevierStyleCrossRef" href="#bib0770"><span class="elsevierStyleSup">6</span></a></p></span><span id="sec0190" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0250">Dysplastic nevus, not associated with melanoma</span><p id="par0610" class="elsevierStylePara elsevierViewall">It is associated with decreased serum vitamin D values. The diagnosis is made by dermatological examination and this is completed by serum vitamin D analyses. It can be improved with calcifediol.<a class="elsevierStyleCrossRef" href="#bib1125"><span class="elsevierStyleSup">77</span></a></p></span></span><span id="sec0195" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0255">Endocrinology and metabolism</span><p id="par0615" class="elsevierStylePara elsevierViewall">Patients with DM1 may present different endocrinopathies, the most prevalent being hypogonadism, thyroid disorders, hydrocarbon and phosphocalcium metabolism abnormalities. Less frequently, they may present a dysfunction of the corticotropic axis, dyslipidemia, and electrolyte abnormalities. The incidence of endocrine pathology increases as the disease progresses, and therefore periodic screening is important.<a class="elsevierStyleCrossRef" href="#bib1130"><span class="elsevierStyleSup">78</span></a></p><span id="sec0200" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0260">Hypogonadotropic hypogonadism</span><p id="par0620" class="elsevierStylePara elsevierViewall">It is the most frequent endocrinological manifestation in DM. It has been published that up to 80% of males present with testicular atrophy, with greater tubular damage than interstitial damage. It is characterized by decreased levels of testosterone and/or infertility, by impaired spermatogenesis and elevation of gonadotropins (follicle-stimulating hormone [FSH], luteinizing hormone [LH]). The deficit of testosterone produces a progressive regression of secondary sexual characteristics (decrease in body hair, libido and frequency of shaving) and less muscle mass. This decrease in lean mass can, in turn, worsen the underlying muscle symptomatology of the disease, and in the long term it can induce lower bone mass.</p><p id="par0625" class="elsevierStylePara elsevierViewall">In women there may be more incidences of infertility, spontaneous abortions and, in rare cases, premature ovarian failure. Premature ovarian failure is also a cause of secondary osteoporosis.<a class="elsevierStyleCrossRef" href="#bib1135"><span class="elsevierStyleSup">79</span></a></p><p id="par0630" class="elsevierStylePara elsevierViewall">Given the high prevalence of hypogonadism, it is recommended to request an evaluation of LH/FSH and testosterone levels to adult males at the first visit and subsequently, annually or earlier, if they present clinical signs of hypogonadism.</p><p id="par0635" class="elsevierStylePara elsevierViewall">In women with secondary amenorrhoea, evaluations of estradiol and LH/FSH should be requested.</p><p id="par0640" class="elsevierStylePara elsevierViewall">If there is male hypogonadism, it should be referred to endocrinology to complete the study and evaluate it, offering replacement therapy with testosterone.</p><p id="par0645" class="elsevierStylePara elsevierViewall">In women, depending on the age ovarian failure occurs, hormone replacement therapy will be evaluated.</p></span><span id="sec0205" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0265">Alterations of hydrocarbon metabolism</span><p id="par0650" class="elsevierStylePara elsevierViewall">In DM1, the pathophysiological defect underlying the alterations in hydrocarbon metabolism is normally insulin resistance, due to a lower sensitivity of the same in the muscles. It can manifest as:<ul class="elsevierStyleList" id="lis0035"><li class="elsevierStyleListItem" id="lsti0230"><span class="elsevierStyleLabel">-</span><p id="par0655" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Hyperinsulinism</span> with normal glucose tolerance: elevated insulin levels with normal plasma glucose.</p></li><li class="elsevierStyleListItem" id="lsti0235"><span class="elsevierStyleLabel">-</span><p id="par0660" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Prediabetes</span> which includes altered basal glycaemia (fasting plasma glucose 100–125<span class="elsevierStyleHsp" style=""></span>mg/dl), glycosylated haemoglobin (HbA1c): 5.7–6.4% (standardized method from the Diabetes Control and Complications Trial [DCCT] – National Glycohemoglobin Standardization Program [NGSP]) or carbohydrate intolerance (glycaemia at 2) h after oral overload of 75<span class="elsevierStyleHsp" style=""></span>g of glucose of 140–199<span class="elsevierStyleHsp" style=""></span>mg/dl).</p></li><li class="elsevierStyleListItem" id="lsti0240"><span class="elsevierStyleLabel">-</span><p id="par0665" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Diabetes mellitus</span> diagnosed by fasting plasma glucose<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>126<span class="elsevierStyleHsp" style=""></span>mg/dl, glycaemia after oral overload with 75<span class="elsevierStyleHsp" style=""></span>g of glucose<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>200<span class="elsevierStyleHsp" style=""></span>mg/dl or HbA1c<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>6.5%.</p></li></ul></p><p id="par0670" class="elsevierStylePara elsevierViewall">To diagnose prediabetes or diabetes, at least 2 criteria are required unless the patient has a random plasma glucose greater than 200<span class="elsevierStyleHsp" style=""></span>mg/dl with clinical manifestations (polyuria, polydipsia, polyphagia and/or weight loss).</p><p id="par0675" class="elsevierStylePara elsevierViewall">It is advisable to determine fasting plasma glucose, HbA1c, and insulin once a year or sooner if there are cardinal symptoms of hyperglycaemia: polyuria, polydipsia, polyphagia, weight loss.</p><p id="par0680" class="elsevierStylePara elsevierViewall">The antidiabetic treatment of choice in patients with diabetes mellitus is insulin-sensitising drugs. Metformin is the first choice.</p></span><span id="sec0210" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0270">Lipid alterations</span><p id="par0685" class="elsevierStylePara elsevierViewall">The most frequent lipid alterations are the elevation of triglycerides and the decrease of HDL, present in 67% and 35% of patients respectively. Dyslipidaemia, together with an altered hydrocarbonated metabolism, leads to a predisposition of a greater cardiovascular risk due to the metabolic syndrome, which is why it is important to monitor the blood pressure and the lipid profile (cholesterol and its fractions and triglycerides) one to two times a year.</p><p id="par0690" class="elsevierStylePara elsevierViewall">It is essential to insist on healthy eating and regular physical exercise, adapted to each individual, since the sedentary lifestyle of these patients is a fundamental etiopathogenic factor in the appearance of the metabolic syndrome. In case of needing pharmacological treatment for dyslipidemia, statins and fibrates could be used, but with close monitoring, because they could worsen the clinical myopathy of these patients. If prescribed, careful monitoring over the CK, glutamic-oxalacetic transaminase (GOT) and glutamic pyruvic transaminase (GPT) levels should be ensured periodically and especially during the first year.<a class="elsevierStyleCrossRef" href="#bib1140"><span class="elsevierStyleSup">80</span></a></p></span><span id="sec0215" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0275">Phospho-calcium metabolism</span><p id="par0695" class="elsevierStylePara elsevierViewall">The prevalence of vitamin D deficiency, defined as a value lower than 30<span class="elsevierStyleHsp" style=""></span>ng/ml, is detected in 90% of patients with DM1 in some case series. Severe deficit (25 OH vitamin D<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleHsp" style=""></span>ng/ml) is detected in 40%. The causes of hypovitaminosis D are the same as those described for the general population: low intake of foods rich in this vitamin, little sun exposure and changes in body composition (increase in fat mass). Vitamin D deficiency can cause osteomalacia (inadequate bone mineralization), muscle weakness and secondary hyperparathyroidism, which in turn worsens the muscle weakness already present in subjects with myotonic dystrophy.</p><p id="par0700" class="elsevierStylePara elsevierViewall">In relation to phosphocalcic metabolism, there is a higher prevalence of primary hyperparathyroidism, up to 17.5%, usually associated with parathyroid adenomas.<a class="elsevierStyleCrossRef" href="#bib1130"><span class="elsevierStyleSup">78</span></a></p><p id="par0705" class="elsevierStylePara elsevierViewall">The management of primary hyperparathyroidism in patients with DM1 is the same as in the general population.</p><p id="par0710" class="elsevierStylePara elsevierViewall">The recommendations are to monitor the levels of calcium, phosphorus, vitamin D and parathyroid hormone (PTH) annually. In case of hypercalcaemia, hypophosphataemia or elevated PTH levels, a second analysis should be requested and if the alteration is confirmed the patient should be referred to endocrinology.</p></span><span id="sec0220" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0280">Thyroid metabolism</span><p id="par0715" class="elsevierStylePara elsevierViewall">Thyroid alterations are less frequent than the rest of the endocrinopathies, which is contrary to the rest of the population. Nodular pathology is the most frequently observed alteration in patients with DM1, and in the majority of patients it is observed together with euthyroidism.</p><p id="par0720" class="elsevierStylePara elsevierViewall">In the case of primary hypothyroidism, the characteristic clinical signs of hormonal hypofunction can increase muscle weakness and worsen the symptoms of dystrophy, so early diagnosis and replacement therapy is important.</p><p id="par0725" class="elsevierStylePara elsevierViewall">The thyroid function (thyrotropin [TSH]) and a physical examination of the thyroid region should be monitored once a year. If thyroid growth is observed or a thyroid nodule is detected during the physical examination, a thyroid ultrasound should be performed and referred to the endocrinologist, and the same action should be taken if there is evidence of TSH alteration in 2 determinations.<a class="elsevierStyleCrossRefs" href="#bib1145"><span class="elsevierStyleSup">81–83</span></a></p></span><span id="sec0225" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0285">Corticotrope axis</span><p id="par0730" class="elsevierStylePara elsevierViewall">Another of the endocrinological abnormalities observed in patients with DM1 are the <span class="elsevierStyleItalic">alterations of the corticotrope axis</span> (corticotropin-releasing hormone [CRH] – corticotropin [ACTH] – cortisol) with adrenocortical dysregulation, whose frequency is not clearly established. The results obtained so far are variable, as well as their clinical significance. There could be hyperactivity, reflected by an increase in basal cortisol and ACTH, with a flattened circadian rhythm of cortisol and ACTH. The stimulation of cortisol response by exogenous ACTH is variable. In most patients the plasma cortisol response is adequate, although there are some cases of hyporesponsiveness, which would imply a low adrenal reserve, and a few cases of hyperresponsiveness, in the presence of greater CTG repeats. After CRH an exaggerated response of ACTH is observed.</p><p id="par0735" class="elsevierStylePara elsevierViewall">Other authors, on the other hand, have found low basal cortisol levels, and after CRH stimulation, a lower cortisol response, with higher mean ACTH values, indicating a lack of efficacy of ACTH on the adrenal receptor.</p><p id="par0740" class="elsevierStylePara elsevierViewall">The results of the stimulation tests with insulin and metyrapone are normal, as well as those of dexamethasone suppression.<a class="elsevierStyleCrossRefs" href="#bib1160"><span class="elsevierStyleSup">84–86</span></a></p><p id="par0745" class="elsevierStylePara elsevierViewall">It is not necessary to evaluate this axis routinely, unless there are clinical manifestations of hypocortisolism (asthenia, orthostatic hypotension, hyperkalaemia or hyponatremia), for which it would be necessary to request basal cortisol in blood at 8.00, and if it is less than 18<span class="elsevierStyleHsp" style=""></span>μg/dl, the patient should be referred to endocrinology. It would also be advisable to refer patients to endocrinology with hypercortisolism symptoms (midsection obesity, capillary fragility, facial plethora, reddish stretch marks on the abdomen, etc.). For the screening of increased cortisol secretion, either a 24<span class="elsevierStyleHsp" style=""></span>h urinary free cortisol test, a salivary cortisol test or a 1<span class="elsevierStyleHsp" style=""></span>mg overnight dexamethasone suppression test starting at 23.00<span class="elsevierStyleHsp" style=""></span>h with cortisol determination at 8.00<span class="elsevierStyleHsp" style=""></span>h, is requested. If the blood cortisol after suppression is greater than 1.8<span class="elsevierStyleHsp" style=""></span>μg/dl, salivary cortisol is elevated, and the patient should be referred to endocrinology.</p><p id="par0750" class="elsevierStylePara elsevierViewall">There have also been isolated cases of <span class="elsevierStyleItalic">hydroelectrolytic alterations</span>, whose causes are not yet clearly established. Some patients present hyperkalaemia, which seems secondary to hyperreninemic hypoaldosteronism,<a class="elsevierStyleCrossRef" href="#bib1175"><span class="elsevierStyleSup">87</span></a> and others present high levels of sodium, without clinical repercussion, where it is thought that this may be due to an alteration of the sodium osmoregulation secondary to an ADH production deficit and a decrease in thirst.<a class="elsevierStyleCrossRef" href="#bib1180"><span class="elsevierStyleSup">88</span></a></p><p id="par0755" class="elsevierStylePara elsevierViewall">It is recommended to request plasmatic ions annually, and if they are altered, to repeat the tests to include urinary sodium and potassium. If electrolyte alterations persist, the patient should be referred to endocrinology for study.</p><p id="par0760" class="elsevierStylePara elsevierViewall"><a class="elsevierStyleCrossRef" href="#tbl0025">Table 5</a> contains recommendations on endocrinological follow-up.</p><elsevierMultimedia ident="tbl0025"></elsevierMultimedia></span></span><span id="sec0230" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0290">Gastrointestinal involvement</span><p id="par0765" class="elsevierStylePara elsevierViewall">The involvement of the digestive system is one of the most frequent occurrences and it has not been well studied. In general the manifestations derived from its alteration are little valued both by patient and doctor. This is mainly due to the involvement of the smooth muscle in DM1. The frequency and intensity of the symptoms it provokes are very variable, although they can become severe and even be the form of presentation of the disease. This disorder has been attributed to inclusions of RNA and “muscleblind-like 1” protein (MBNL1) in smooth muscle nuclei.<a class="elsevierStyleCrossRef" href="#bib1185"><span class="elsevierStyleSup">89</span></a> Other hypotheses suggest a deficient innervation of the smooth muscle, fatty infiltration of the hollow viscera walls, fibrosis of these walls or even degeneration of the smooth muscle. For these hypotheses there are, however, no conclusive studies.<a class="elsevierStyleCrossRefs" href="#bib1190"><span class="elsevierStyleSup">90–93</span></a></p><span id="sec0235" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0295">Alterations in the chewing process</span><p id="par0770" class="elsevierStylePara elsevierViewall">They are produced by weakness and myotonia of the oral and masticatory muscles (tongue, palate, pharynx, masseter, pterygoid). They should be evaluated clinically once a year. If there is involvement, physiotherapy with a speech therapist is recommended.<a class="elsevierStyleCrossRef" href="#bib0765"><span class="elsevierStyleSup">5</span></a></p></span><span id="sec0240" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0300">Dysphagia</span><p id="par0775" class="elsevierStylePara elsevierViewall">Dysphagia is present in approximately 55% of patients with myotonic dystrophy. It carries a high risk of aspiration pneumonia and malnutrition, and it is an important cause of morbidity and mortality.<a class="elsevierStyleCrossRef" href="#bib1200"><span class="elsevierStyleSup">92</span></a></p><p id="par0780" class="elsevierStylePara elsevierViewall">It is neither related to the degree of skeletal muscle involvement nor to the duration of the muscle disease. It is produced by weakness of the masticatory muscles, pharyngeal muscles, upper oesophageal sphincter and oesophageal body, as well as myotonia of these muscles. They present less pharyngeal propulsion with an increase in residue in that area. All these mechanisms with the weakness of the respiratory muscles favour the appearance of respiratory infections.<a class="elsevierStyleCrossRef" href="#bib1205"><span class="elsevierStyleSup">93</span></a> The dysphagia of the patient with myotonic dystrophy has the peculiarity that it usually causes more difficulty to swallow solid foods, unlike other neurological diseases, in which the difficulty is to swallow food with a liquid texture or a mixture of two types of textures. In all patients it is advisable to conduct a disease-specific clinical history, insisting on the presence of symptoms suggestive of dysphagia such as: cough when swallowing, respiratory infections, and weight loss.<a class="elsevierStyleCrossRef" href="#bib1135"><span class="elsevierStyleSup">79</span></a> A simple screening test that can help to detect the presence of dysphagia is the <span class="elsevierStyleItalic">Eating Assessment Tool-10</span> (EAT-10).<a class="elsevierStyleCrossRef" href="#bib1210"><span class="elsevierStyleSup">94</span></a> It is a questionnaire containing 10 questions, validated in Spanish. If the test is positive, it is necessary to perform a diagnosis of dysphagia, using techniques directed by trained personnel: volume-viscosity swallow test (V-VST), fibroendoscopy evaluation of swallowing or videofluoroscopy. The first consists of giving the patient different volumes and textures and monitoring for signs of alarm: coughing, desaturation, changes in the voice, etc. This study shows the texture and the volume that is safe for the patient. The fibroendoscopy test is a direct method performed by the ear nose and throat specialist. It shows whether there is penetration or aspirations by giving different volumes and textures of food, and as it is a direct method, it allows silent aspirations to be detected that sometimes go unnoticed with the V-VST.<a class="elsevierStyleCrossRef" href="#bib1215"><span class="elsevierStyleSup">95</span></a> Finally, videofluoroscopy, considered as the gold standard, is a radiological technique in real time allowing a study of swallowing and its safety. However, it is a costly technique and is not available in all centres.</p><p id="par0785" class="elsevierStylePara elsevierViewall">For the treatment, the foods used will be adapted to the safest texture in each case. If the dysphagia is with liquids, thickeners will be used and two types of textures will be avoided, and if the dysphagia is to solids an attempt will be made with soft or crushed foods, and the foods which are risky for the patient will be avoided. Physiotherapy guided by a speech therapist can improve the patient's condition.</p><p id="par0790" class="elsevierStylePara elsevierViewall">Do not forget that dysphagia is associated with the risk of malnutrition, so it is important to monitor the weight of these patients, to detect involuntary weight loss or decrease in food intake, and to monitor analytical parameters of malnutrition: albumin and prealbumin. In the case of malnutrition or weight loss, it will be necessary to enrich the diet from a caloric and protein point of view; if this is not enough, nutritional supplements should be added, and in some cases enteral nutrition through nasogastric tube may be needed if a short time is envisaged (<4–6 weeks), or ostomies (gastrostomies or jejunostomies) if the disorder is expected to last longer.</p><p id="par0795" class="elsevierStylePara elsevierViewall">In addition, in the nutritional approach, physical exercise must be included and adapted to each patient to preserve or minimize the loss of muscle mass.</p><p id="par0800" class="elsevierStylePara elsevierViewall">Recommendations on dysphagia:<ul class="elsevierStyleList" id="lis0040"><li class="elsevierStyleListItem" id="lsti0245"><span class="elsevierStyleLabel">1.</span><p id="par0805" class="elsevierStylePara elsevierViewall">The presence of symptoms of dysphagia to solid, liquid and dual textures should be monitored at least once a year.</p></li><li class="elsevierStyleListItem" id="lsti0250"><span class="elsevierStyleLabel">2.</span><p id="par0810" class="elsevierStylePara elsevierViewall">The use of the EAT-10 dysphagia screening test is recommended once a year.</p></li><li class="elsevierStyleListItem" id="lsti0255"><span class="elsevierStyleLabel">3.</span><p id="par0815" class="elsevierStylePara elsevierViewall">If the EAT-10 test shows a result<span class="elsevierStyleHsp" style=""></span>≥<span class="elsevierStyleHsp" style=""></span>3, then perform the volume-viscosity test, fibroendoscopy of swallowing or videofluoroscopy, according to hospital availability.</p></li><li class="elsevierStyleListItem" id="lsti0260"><span class="elsevierStyleLabel">4.</span><p id="par0820" class="elsevierStylePara elsevierViewall">Monitor the weight once a year or more frequently if the patient has dysphagia.</p></li><li class="elsevierStyleListItem" id="lsti0265"><span class="elsevierStyleLabel">5.</span><p id="par0825" class="elsevierStylePara elsevierViewall">Specific nutritional recommendations will be provided for patients with dysphagia (<a class="elsevierStyleCrossRef" href="#sec0360">Appendix A</a>).</p></li><li class="elsevierStyleListItem" id="lsti0270"><span class="elsevierStyleLabel">6.</span><p id="par0830" class="elsevierStylePara elsevierViewall">In patients with malnutrition or unintentional weight loss nutritional guidelines for food enrichment and the use of nutritional supplements are recommended if the weight loss is<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>10% in 6 months or if there is no improvement with the nutritional recommendations.</p></li><li class="elsevierStyleListItem" id="lsti0275"><span class="elsevierStyleLabel">7.</span><p id="par0835" class="elsevierStylePara elsevierViewall">Individualized physical exercise will be encouraged in all visits.</p></li><li class="elsevierStyleListItem" id="lsti0280"><span class="elsevierStyleLabel">8.</span><p id="par0840" class="elsevierStylePara elsevierViewall">Similar to other neurological diseases or situations of dysphagia, in cases in which it is severe and it is not possible to establish specific recommendations, a nasogastric tube or gastrostomy will be placed depending on the duration of the situation.</p></li></ul></p></span><span id="sec0245" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0305">Ooesophagus and stomach</span><p id="par0845" class="elsevierStylePara elsevierViewall">Disorders attributed to oesophageal myotonia, hypotonia of the oesophagus, weakness of the cardia, and poor oesophageal and gastric peristalsis have been described.</p><p id="par0850" class="elsevierStylePara elsevierViewall">A delay of the transit through the oesophagus can be demonstrated by cinerradiography, oesophageal manometry or, in a much simpler, faster and cheaper way, by means of the study of the oesophageal transit with radioisotopes. This latter is a technique that has demonstrated an excellent correlation with the oesophageal manometry.<a class="elsevierStyleCrossRefs" href="#bib1220"><span class="elsevierStyleSup">96–99</span></a></p><p id="par0855" class="elsevierStylePara elsevierViewall">The gastric emptying slows, and manifests itself as heavy and prolonged digestion and a sensation of abdominal fullness. This alteration, together with weakness of the cardia, also favours regurgitation and the risk of bronchoaspiration. It can be treated with proton pump inhibitors and smooth muscle prokinetics.<a class="elsevierStyleCrossRef" href="#bib1135"><span class="elsevierStyleSup">79</span></a> The study of gastric emptying with radioisotopes confirms this alteration.<a class="elsevierStyleCrossRef" href="#bib1240"><span class="elsevierStyleSup">100</span></a></p></span><span id="sec0250" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0310">Intestine</span><p id="par0860" class="elsevierStylePara elsevierViewall">The weakness and scarcity of the peristaltic movements of the small and large intestine cause phases of constipation alternating with diarrhoea, as well as abdominal discomfort of varying intensities, which can result in intestinal pseudo-obstruction. Both megacolon and intestinal volvulus associated with DM1 have been described. In the radiological studies, segmental dilatation and the disappearance of the colonic haustra folds can be seen, due to a decrease in peristalsis.<a class="elsevierStyleCrossRefs" href="#bib1240"><span class="elsevierStyleSup">100–103</span></a></p><p id="par0865" class="elsevierStylePara elsevierViewall">It is recommended to evaluate constipation annually. Improvement can be achieved with a diet rich in fibre, prokinetics and laxatives.<a class="elsevierStyleCrossRef" href="#bib0765"><span class="elsevierStyleSup">5</span></a></p><p id="par0870" class="elsevierStylePara elsevierViewall">Diarrhoea is due to intestinal bacterial overgrowth and is usually accompanied by abdominal swelling, bloating and malaise. Possible nutritional deficit should be monitored, especially that of vitamin B12. The diagnosis is clinical. There is a carbohydrate metabolism test, the D-xylose test, which is no longer used. It should be treated with diet, probiotics, cholestyramine and antibiotics (rifaximin 400<span class="elsevierStyleHsp" style=""></span>mg/12<span class="elsevierStyleHsp" style=""></span>h for 7 days in monthly cycles or ciprofloxacin 500<span class="elsevierStyleHsp" style=""></span>mg/12<span class="elsevierStyleHsp" style=""></span>h for 7 days in monthly cycles).<a class="elsevierStyleCrossRef" href="#bib1260"><span class="elsevierStyleSup">104</span></a></p></span><span id="sec0255" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0315">Gallbladder</span><p id="par0875" class="elsevierStylePara elsevierViewall">The incidence of cholelithiasis is increased in patients with DM1 (25–50% of cases), due to the slow mobility of the gallbladder, which favours the increase of biliary sludge and the formation of stones.<a class="elsevierStyleCrossRef" href="#bib1265"><span class="elsevierStyleSup">105</span></a></p></span><span id="sec0260" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0320">Anus</span><p id="par0880" class="elsevierStylePara elsevierViewall">The internal anal sphincter can show intense myotonia and the external anal sphincter can show a mixture of milder myotonia and weakness, which contributes to the constipation of the patient. Myotonia can be demonstrated by electromyography.<a class="elsevierStyleCrossRef" href="#bib1270"><span class="elsevierStyleSup">106</span></a></p></span></span></span><span id="sec0265" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0325">Genitourinary system</span><p id="par0885" class="elsevierStylePara elsevierViewall">In addition to the hormonal alterations indicated previously, and that present with hypogonadism, decreased sexual potency, reduced libido and hypofertility, there are other symptoms that may appear during the course of the disease, due to smooth muscle involvement.</p><p id="par0890" class="elsevierStylePara elsevierViewall">During childbirth, women present inadequate uterine contractions, delayed uterine relaxation and increased labour. After delivery there is an increased risk of haemorrhage and retention of the placenta due to uterine muscle incompetence.<a class="elsevierStyleCrossRef" href="#bib0910"><span class="elsevierStyleSup">34</span></a></p><p id="par0895" class="elsevierStylePara elsevierViewall">The bladder of patients with DM1 is usually normal, and focal ureteral dilatation may occasionally be found.<a class="elsevierStyleCrossRef" href="#bib0910"><span class="elsevierStyleSup">34</span></a></p></span><span id="sec0270" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0330">Stomatology</span><span id="sec0275" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0335">Caries, gingivitis, bacterial plaque</span><p id="par0900" class="elsevierStylePara elsevierViewall">Dental alterations are especially frequent because of poor oral hygiene, especially in the posterior dental arch, and hyposalivation. It should be evaluated annually and proper oral hygiene must be emphasized, with instructions for brushing teeth, the use of dental floss, rinsing with chlorhexidine and avoiding sugary foods that promote tooth decay.<a class="elsevierStyleCrossRefs" href="#bib1275"><span class="elsevierStyleSup">107,108</span></a></p></span><span id="sec0280" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0340">Facial dysmorphism, ogival palate, mandibular retrognathia or prognathism</span><p id="par0905" class="elsevierStylePara elsevierViewall">These are found in congenital DM1, due to alterations in the embryonic development. If the alterations are severe, and disrupt eating ability or speech, orthognathic surgery can be considered.<a class="elsevierStyleCrossRef" href="#bib1285"><span class="elsevierStyleSup">109</span></a></p></span><span id="sec0285" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0345">Limited buccal opening, jaw pain and claudication associated with chewing</span><p id="par0910" class="elsevierStylePara elsevierViewall">These alterations are temporomandibular dysfunctions due to morphological changes of the articular disc with bone remodelling and chewing muscle weakness. The study uses imaging tests of the temporomandibular joint (functional radiography, MRI). If they present with pain, they can be treated with an intraoral stress breaker or physiotherapy.<a class="elsevierStyleCrossRef" href="#bib1290"><span class="elsevierStyleSup">110</span></a></p></span></span><span id="sec0290" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0350">Ophthalmology</span><span id="sec0295" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0355">Cataracts</span><p id="par0915" class="elsevierStylePara elsevierViewall">Throughout the evolution of DM1, the development of cataracts is very frequent; these are abnormal cataracts which are very characteristic, subcapsular and iridescent. They are found in 90% of patients and are usually diagnosed when a patient is over 50 years of age, although they may appear earlier. In cases with 50–100 repeats of the triplet, the appearance of early cataracts is usually the only evidence of the disease. All patients with DM1 should be evaluated periodically by an ophthalmologist, every 2 years at least.</p><p id="par0920" class="elsevierStylePara elsevierViewall">An ophthalmologic examination, including the slit lamp, is the usual diagnostic procedure. Cataracts are treated by removing the lens.<a class="elsevierStyleCrossRefs" href="#bib1295"><span class="elsevierStyleSup">111–114</span></a></p></span><span id="sec0300" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0360">Ocular hypotension</span><p id="par0925" class="elsevierStylePara elsevierViewall">Almost all patients with DM1 have ocular hypotension and glaucoma is exceptional. It is attributed to ciliary body detachment or smooth muscle weakness. It usually occurs asymptomatically and is diagnosed by measuring the intraocular pressure, which is usually below 5<span class="elsevierStyleHsp" style=""></span>mmHg. It does not require treatment, unless it causes functional or structural changes in the eye.<a class="elsevierStyleCrossRef" href="#bib1315"><span class="elsevierStyleSup">115</span></a></p></span><span id="sec0305" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0365">Eyelid ptosis</span><p id="par0930" class="elsevierStylePara elsevierViewall">Eyelid ptosis, due to weakness of the eyelid levator muscle, is usually constant in this disease. It has a progressive course and contributes to the characteristic appearance of these patients’ faces. If it limits the visual field, then blepharoplasty should be considered.<a class="elsevierStyleCrossRef" href="#bib1320"><span class="elsevierStyleSup">116</span></a></p></span><span id="sec0310" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0370">Pregnancy</span><p id="par0935" class="elsevierStylePara elsevierViewall">Patients with DM1 who are pregnant are at risk of procreating a child with DM1, who usually has more life-altering symptoms than the mother. A prenatal diagnosis can be made with a rough estimate of the affectation according to the number of CTG triplet repeats.</p><p id="par0940" class="elsevierStylePara elsevierViewall">Women with DM1 more frequently suffer from hydramnios, spontaneous abortions, weakness during labour, retained placenta and postpartum haemorrhage.<a class="elsevierStyleCrossRef" href="#bib1325"><span class="elsevierStyleSup">117</span></a> Studies show 19% pre-term deliveries, 13% urinary tract infections, 9% placenta previa and 4% ectopic pregnancies in affected individuals.<a class="elsevierStyleCrossRef" href="#bib1330"><span class="elsevierStyleSup">118</span></a> Therefore, in addition to the possible cardiac complications of the pregnant woman and the anaesthetic implications of the disease, these patients should be considered as high risk pregnancies, and therefore obstetrically controlled with special attention.</p></span></span><span id="sec0315" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0375">Anaesthesia</span><p id="par0945" class="elsevierStylePara elsevierViewall">Patients with DM1 have some specific risks when undergoing anaesthetic procedures, which should be taken into consideration. These patients frequently receive surgical interventions for the treatment of cataracts and abdominal surgery, especially cholecystectomy, in addition to possible interventions for other disorders.<a class="elsevierStyleCrossRefs" href="#bib0845"><span class="elsevierStyleSup">21,119</span></a></p><p id="par0950" class="elsevierStylePara elsevierViewall">Locoregional anaesthesia is safe, but opiates and sedatives with respiratory effect should be used with caution, since these patients are especially sensitive to these drugs<a class="elsevierStyleCrossRefs" href="#bib1325"><span class="elsevierStyleSup">117–120</span></a> and may even suffer paradoxical reactions with muscle relaxants. Spinal anaesthesia is the most recommended for tocological surgery.</p><p id="par0955" class="elsevierStylePara elsevierViewall">For general anaesthetic, drugs such as propofol and short-acting opioids, such as remifentanil, may be used.<a class="elsevierStyleCrossRef" href="#bib1335"><span class="elsevierStyleSup">119</span></a> In the postoperative period, it is convenient to monitor respiratory function by pulse oximetry in the first 24<span class="elsevierStyleHsp" style=""></span>h, due to the risk of apnoea<a class="elsevierStyleCrossRefs" href="#bib0845"><span class="elsevierStyleSup">21,117,120</span></a> and of atelectasis due to hypoventilation, especially if the forced vital capacity is already altered. Likewise, there is an increased risk of cardiac arrhythmias, which requires heart rate monitoring.<a class="elsevierStyleCrossRef" href="#bib1340"><span class="elsevierStyleSup">120</span></a> The risk of possible pulmonary aspiration should not be forgotten.</p><p id="par0960" class="elsevierStylePara elsevierViewall">In patients who are going to undergo general anaesthesia, a respiratory evaluation is always recommended.</p><p id="par0965" class="elsevierStylePara elsevierViewall">In cases treated with mexiletine, class I antiarrhythmic agents should not be administered due to the risk of conduction blockage. Depolarizing muscle relaxants are contraindicated because they can induce hyperkalemia. If muscle relaxants are essential, it is advisable to use those that are short action, taking into account that their action can be longer than normal. The use of anticholinesterase drugs, such as prostigmine, can produce intense muscle weakness.</p><p id="par0970" class="elsevierStylePara elsevierViewall">The <span class="elsevierStyleItalic">Deutschen Gesellschaft für Anästhesiologie und Intensivmedizin e. V.</span> (German Society of Anaesthesiology and Intensive Medicine) has prepared an anaesthetic guide for DM1 that can be consulted (it is translated into Spanish on the Orphanet portal).<a class="elsevierStyleCrossRef" href="#bib1335"><span class="elsevierStyleSup">119</span></a></p></span><span id="sec0320" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0380">Myotonic dystrophy type 1 and cancer</span><p id="par0975" class="elsevierStylePara elsevierViewall">An increase in the incidence of tumours in patients with DM1 has been described.</p><p id="par0980" class="elsevierStylePara elsevierViewall">The first neoplasms that were described in these patients were pilomatricomas, benign skin tumours which are often calcified and derived from cells of the hair matrix. In several articles the appearance of tumours of different lineages have been described, including a possible association with multiple basal cell carcinomas. A study that used the Swedish and Danish registers of patients with myotonic dystrophy between 1977 and 2008<a class="elsevierStyleCrossRef" href="#bib1345"><span class="elsevierStyleSup">121</span></a> found a higher than expected incidence of tumours. The elevated overall cancer risk with respect to the general population was primarily due to malignancies of the endometrium, brain, ovary and colon.<a class="elsevierStyleCrossRef" href="#bib1350"><span class="elsevierStyleSup">122</span></a> This study did not differentiate between patients with DM1 and DM2, although given the relative prevalence of both diseases it is reasonable to think that the majority of patients had DM1. In a cohort of 307 patients with DM1 and DM2 from the Mayo Clinic, collected between 1993 and 2010, a possible increase in the risk of suffering from thyroid cancer and choroidal melanoma was found, as well as a possibility of prostate and testicular cancer.<a class="elsevierStyleCrossRef" href="#bib1355"><span class="elsevierStyleSup">123</span></a> Another population-based study conducted in Utah found an excess of endometrial and testicular tumours, and non-Hodgkin's lymphomas.<a class="elsevierStyleCrossRefs" href="#bib1360"><span class="elsevierStyleSup">124–126</span></a> These data have been validated in a Spanish cohort with a higher prevalence in women, the most prevalent tumours being ovarian and endometrial, while the thyroid and brain tumours were found in men.<a class="elsevierStyleCrossRef" href="#bib1375"><span class="elsevierStyleSup">127</span></a></p><p id="par0985" class="elsevierStylePara elsevierViewall">The cause of this possible increase in tumours in DM1 is not known, although several hypotheses involving betacatenin or the downregulation of a family of miRNA suppressors (200c/141) have been described.<a class="elsevierStyleCrossRef" href="#bib1375"><span class="elsevierStyleSup">127</span></a> Interestingly, no increase in tumour incidence has been found in other diseases caused by triplet expansion.</p><p id="par0990" class="elsevierStylePara elsevierViewall">The increased risk of tumours should be taken into account, since it is the third cause of death in these patients. The elevated risk neoplasms are mostly low incidence tumours, in which screening programmes in the general population have not shown clear utility. These patients should be subjected to periodical examinations in their regular consultancies, with special clinical suspicion of symptoms suggestive of cancer, especially cerebral or abdomen/pelvic symptoms, or uterine bleeding. Thyroid palpation is recommended in the endocrinological examinations and imaging studies if alterations are found. It is important to remember the possibility of choroidal melanoma in the ophthalmological evaluation. It is important to keep on the look out for the most frequent tumours in the general population, such as skin tumours.</p></span><span id="sec0325" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0385">Special considerations in the paediatric age</span><p id="par0995" class="elsevierStylePara elsevierViewall">DM1 usually manifests in adulthood, but there are 2 forms of paediatric presentation: congenital myotonic dystrophy and the variant of childhood onset DM1. Both have differential characteristics with respect to the classic adult form, although the diagnostic process is similar: the clinical suspicion followed by genetic confirmation by means of a disease-specific study.</p><span id="sec0330" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0390">Congenital myotonic dystrophy</span><p id="par1000" class="elsevierStylePara elsevierViewall">It is the most serious form, but also the most infrequent. The incidence varies between 2.1 and 5.2 per 100,000 live births.<a class="elsevierStyleCrossRefs" href="#bib1380"><span class="elsevierStyleSup">128,129</span></a> In Spain it has been estimated at 0.8 per 10,000.<a class="elsevierStyleCrossRef" href="#bib1390"><span class="elsevierStyleSup">130</span></a> In the vast majority of cases, transmission is maternal, resulting in a massive intergenerational expansion of the number of triplets. It is normal that the child with congenital myotonic dystrophy is the index case that leads to diagnosing the mother, who is usually paucisymptomatic, and other family members.</p><p id="par1005" class="elsevierStylePara elsevierViewall">The manifestations of the disease are present from birth, although the obstetric history usually reflects poor mobility of the foetus in the prenatal period: reduced foetal movements, polyhydramnios and articular deformities detected by ultrasound, or a pregnancy completed by caesarean section because the childbirth does not progress. The difficulties in the delivery pose an added risk of perinatal asphyxia. At birth, the neonate shows marked hypotonia associated with weakness and poor movement. Faces are characteristic with facial paresis and inverted V-shaped upper lip. The presence of respiratory problems is very common (more than 50% of cases) due to weakness of the diaphragm and intercostal muscles, lung immaturity (the risk of prematurity is increased) and failure of the cerebral respiratory control. There are also difficulties in suction-swallowing, due to weakness of the bulbar musculature.<a class="elsevierStyleCrossRef" href="#bib1395"><span class="elsevierStyleSup">131</span></a> Joint retractions are common, especially in the form of clubfoot or equine-varus. Unlike in the adult forms, cardiological problems are not frequent at this age and there is no clinical or electrical myotonia until later ages, therefore the electromyogram is not indicated. A higher incidence of ventriculomegaly and cerebral malformations of cortical development has been described.<a class="elsevierStyleCrossRef" href="#bib1400"><span class="elsevierStyleSup">132</span></a></p><p id="par1010" class="elsevierStylePara elsevierViewall">The differential diagnosis is established preferably with muscular dystrophies and congenital myopathies and congenital myasthenic syndromes.</p><p id="par1015" class="elsevierStylePara elsevierViewall">The neonatal mortality of the congenital forms is between 16 and 41%.<a class="elsevierStyleCrossRefs" href="#bib1380"><span class="elsevierStyleSup">128,133,134</span></a> This is essentially due to respiratory problems and the withdrawal of support in patients ventilated for more than 4 weeks, in which the possibility of recovery in the short/medium term is considered as scarce.<a class="elsevierStyleCrossRef" href="#bib1415"><span class="elsevierStyleSup">135</span></a> However, this concept is changing due to the histopathological evidences that consider the congenital form as an immature entity and, therefore, with the potential for improvement in the long run. In this sense, the duration of ventilation should not be the only reason to be taken into account when considering the continuity of medical care.<a class="elsevierStyleCrossRef" href="#bib1380"><span class="elsevierStyleSup">128</span></a></p><p id="par1020" class="elsevierStylePara elsevierViewall">Children who survive the sixth month of life improve slowly, although they manifest an evident delay in motor skills. Virtually all children reach the autonomous walking stage, and improvement is seen in hypotonia and feeding difficulties at around 3–4 years of age. Facial weakness, on the other hand, continues to be prominent, causing the typical “carp mouth”. Myotonia does not appear until adolescence. Together with the motor abnormalities, during the first few years cognitive affectation of variable intensity becomes evident, although a minority of cases exist with normal intellectual capacity.<a class="elsevierStyleCrossRef" href="#bib1420"><span class="elsevierStyleSup">136</span></a> The average intelligence quotient is 70, but mental retardation can reach a moderate or severe level. This is one of the main problems of the congenital form. Children may present features of the autistic spectrum and other associated behavioural problems.<a class="elsevierStyleCrossRef" href="#bib1425"><span class="elsevierStyleSup">137</span></a> The facial aspect can condition the child to being attributed a lower cognitive capacity than he really has. Language development can be hindered both by the weakness in the musculature of the mouth, palate and jaw, preventing good pronunciation, and due to hearing loss from repeated infections of the upper respiratory tract. Over the years, weakness appears in limbs with distal distribution, as well as the other manifestations of adulthood.</p></span><span id="sec0335" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0395">Myotonic dystrophy of childhood onset</span><p id="par1025" class="elsevierStylePara elsevierViewall">In the infantile form, the clinical manifestations appear between the first and the tenth year of life, with pre-, peri- and post-natal development being normal during the first 12 months. This clinical variant has little to do with the congenital form. The transmission can be maternal or paternal. The motor manifestations may not be initially striking, but the language problems and school difficulties are highlighted. In this context there must be a high index of suspicion to be able to diagnose the disease at this age.<a class="elsevierStyleCrossRef" href="#bib1430"><span class="elsevierStyleSup">138</span></a> There are behavioural disorders, with attention deficit hyperactivity disorder being the most frequent problem, and visuospatial impairment is possible even with a normal intelligence quotient.<a class="elsevierStyleCrossRef" href="#bib0885"><span class="elsevierStyleSup">29</span></a> There is a correlation between cognitive impairment and the size of the triplet expansion.<a class="elsevierStyleCrossRef" href="#bib1435"><span class="elsevierStyleSup">139</span></a></p><p id="par1030" class="elsevierStylePara elsevierViewall">In cases with initial muscular impairment there may be awkwardness in the movements and weakness in the facial and neck muscles, but without the typical appearance of the congenital form.<a class="elsevierStyleCrossRef" href="#bib1410"><span class="elsevierStyleSup">134</span></a> It is also possible to find weakness in limbs with distal distribution and myotonic symptoms, as occurs later in life. Sometimes gastrointestinal discomfort is persistent due to the involvement of the smooth muscles. Heart disease is rare, as it normally appears after the second decade of life. Nor are cataracts detected, although they are characteristic in adulthood, but there is a greater frequency of other ophthalmological problems such as refractive defects, reduction of visual acuity and oculomotor disorders.<a class="elsevierStyleCrossRefs" href="#bib1435"><span class="elsevierStyleSup">139,140</span></a> Over time, the same complications seen in adult forms appear in this form.</p></span><span id="sec0340" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0400">Management of type 1 myotonic dystrophy in childhood</span><p id="par1035" class="elsevierStylePara elsevierViewall">The treatment must be multidisciplinary and cover all the problems of the disease.<a class="elsevierStyleCrossRef" href="#bib1395"><span class="elsevierStyleSup">131</span></a> Those born with congenital myotonic dystrophy should be treated in a neonatal intensive care unit, with special monitoring of the respiratory function. Depending on the situation, the need for assisted, non-invasive or tracheostomy ventilation will be considered. The decision to limit the therapeutic effort must be individualized, since, as already mentioned, the need for prolonged ventilation does not necessarily entail a bad prognosis in the medium/long term. In survivors, pulmonary monitoring should be maintained throughout childhood. Respiratory assessment is also necessary in the childhood onset form due to the possible appearance of obstructive sleep apnoea and daytime sleepiness. The problems of suction-swallowing should be studied to avoid aspirations, and the need for tube feeding or gastrostomy will be considered. Cognitive and behavioural alterations are especially relevant in childhood, so a full psychopedagogical evaluation must be carried out, including the evaluation of cognitive capacity and the executive and visuospatial functions. Coordination with the school is necessary to carry out an adequate psychoeducational union.</p><p id="par1040" class="elsevierStylePara elsevierViewall">Cardiological complications are rare before the second decade of life, but there is a possibility of cardiomyopathy and cardiac conduction alterations, so periodic cardiological reviews with annual ECGs are recommended.<a class="elsevierStyleCrossRefs" href="#bib1445"><span class="elsevierStyleSup">141,142</span></a></p><p id="par1045" class="elsevierStylePara elsevierViewall">From the motor point of view, the child must act according to his abilities and his physical activity should not be restricted.<a class="elsevierStyleCrossRef" href="#bib1395"><span class="elsevierStyleSup">131</span></a> The child can benefit from occupational therapy and targeted physiotherapy programmes. Orofacial problems must be addressed by speech therapists. Orthopaedic treatment, through orthosis or surgery, is indicated if there are joint deformities.<a class="elsevierStyleCrossRef" href="#bib1455"><span class="elsevierStyleSup">143</span></a> It is advisable to perform ophthalmological and auditory evaluations to prevent both amblyopia and hearing loss.</p><p id="par1050" class="elsevierStylePara elsevierViewall">In anaesthetic procedures, caution should be exercised due to the possibility of cardiac dysrhythmias and apnoeas. A close observation should be maintained in the first hours after the intervention.</p><p id="par1055" class="elsevierStylePara elsevierViewall">Genetic counselling is especially important in paediatric cases, as these are families formed by young parents who may want to increase the size of their family.</p></span></span><span id="sec0345" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0405">Documents for care and attention in Emergency department and myotonic dystrophy type 1 emergencies</span><p id="par1060" class="elsevierStylePara elsevierViewall">When a patient with DM1 goes to an emergency department there may be some uncertainty that makes it difficult to manage any medical problem, whether it is due to the disease or a common problem. In order to assist the emergency physicians and the patients, emergency cards were created to facilitate quick access to the relevant medical data of the patients and emergency action guidelines with proven clinical information. It is recommended that access to this information is provided to patients, relatives and caregivers, and that the use of emergency cards is encouraged.</p><p id="par1065" class="elsevierStylePara elsevierViewall">The <span class="elsevierStyleItalic">Société Française de Médecine d’Urgence</span> (SFMU) conducted a survey to know the expectations of emergency physicians regarding patients affected by minority diseases.<a class="elsevierStyleCrossRef" href="#bib1460"><span class="elsevierStyleSup">144</span></a> 91% of the respondents wanted the patients to carry a card with their personal information and recommendations for the treatment of their illness while in the emergency department. Approximately 75% of the physicians wished to have an online service (via Internet) that would provide a practical guide for these situations.</p><p id="par1070" class="elsevierStylePara elsevierViewall">In response to these needs, France created new services for health professionals, in order to improve the information that should be available on rare diseases on the Internet. This was through the implementation of the programme <span class="elsevierStyleItalic">Orphanet Urgences</span> (“Good practices in emergency cases”), developed with the <span class="elsevierStyleItalic">Service d’Aide Médicale Urgente</span> and the professionals of the hospital emergency services. These services are emergency action guides and care and emergency cards for various diseases, including DM1, with free and open access through the Internet.<a class="elsevierStyleCrossRefs" href="#bib1025"><span class="elsevierStyleSup">57,145</span></a> Its degree of use, quantified through the number of downloads, is satisfactory.<a class="elsevierStyleCrossRef" href="#bib1470"><span class="elsevierStyleSup">146</span></a></p><p id="par1075" class="elsevierStylePara elsevierViewall">There are no similar investigations in our environment, but it is reasonable to extrapolate the results of this survey to the context of Spain's healthcare. An attempt has been made to try to respond to the 2 needs expressed by the emergency physicians (information provided by the patient and online information) for patients with DM1, by means of the “Tarjeta de Alerta Médica” (Spain's Medical Alert Card). It is a pocket document that the patient should always carry with him, intended for emergency professionals who are not normally familiar with the disease. It contains a brief description of the ailment, contact details of the reference professional, a QR link to the emergency guide of <a href="http://www.orpha.net/">www.orpha.net/</a> for DM1, and it can host updated information on the evolution of the patient.<a class="elsevierStyleCrossRef" href="#bib1475"><span class="elsevierStyleSup">147</span></a></p><p id="par1080" class="elsevierStylePara elsevierViewall">It should be noted that several entities have been developing and disseminating emergency documents for those affected by DM1 (the British associations <span class="elsevierStyleItalic">Muscular Dystrophy UK</span> and <span class="elsevierStyleItalic">Myotonic Dystrophy Support Group</span>, the <span class="elsevierStyleItalic">Scottish Muscle Network</span> and, even, hospitals of the <span class="elsevierStyleItalic">National Health Service</span>, also in the United Kingdom).</p><p id="par1085" class="elsevierStylePara elsevierViewall">Despite the risk of loss, deterioration or forgetting the document and the limited information it contains, and aside from the possible reassuring effect it has on the patient, the “Medical Alert Card” for DM1 is principally a means to facilitate quick access to <span class="elsevierStyleItalic">information validated by experts</span>, through new technologies. Unfortunately, it has not yet been possible to quantify their degree of use.<a class="elsevierStyleCrossRef" href="#bib1480"><span class="elsevierStyleSup">148</span></a></p></span><span id="sec0350" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0410">Funding</span><p id="par1090" class="elsevierStylePara elsevierViewall">This article has not received funding for its preparation.</p></span><span id="sec0355" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0415">Conflict of interests</span><p id="par1095" class="elsevierStylePara elsevierViewall">None.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:21 [ 0 => array:3 [ "identificador" => "xres1331053" "titulo" => "Abstract" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Background and objectives" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Material and methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Recommendations" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusion" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1226374" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1331054" "titulo" => "Resumen" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Antecedentes y objetivos" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Material y métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Recomendaciones" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusión" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1226375" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Methodology" ] 6 => array:3 [ "identificador" => "sec0015" "titulo" => "Diagnosis" "secciones" => array:9 [ 0 => array:2 [ "identificador" => "sec0020" "titulo" => "Creatine kinase levels" ] 1 => array:2 [ "identificador" => "sec0025" "titulo" => "Neurophysiological study" ] 2 => array:2 [ "identificador" => "sec0030" "titulo" => "Muscle biopsy" ] 3 => array:2 [ "identificador" => "sec0035" "titulo" => "Muscular magnetic resonance imaging" ] 4 => array:2 [ "identificador" => "sec0040" "titulo" => "Genetic diagnosis" ] 5 => array:2 [ "identificador" => "sec0045" "titulo" => "Detection of the CTG expansion" ] 6 => array:2 [ "identificador" => "sec0050" "titulo" => "Genetic counselling. Anticipation" ] 7 => array:2 [ "identificador" => "sec0055" "titulo" => "Family planning. Prenatal and preimplantation diagnosis" ] 8 => array:2 [ "identificador" => "sec0060" "titulo" => "Diagnostic protocol" ] ] ] 7 => array:3 [ "identificador" => "sec0065" "titulo" => "Neurological involvement" "secciones" => array:2 [ 0 => array:3 [ "identificador" => "sec0070" "titulo" => "Muscle problems" "secciones" => array:4 [ 0 => array:3 [ "identificador" => "sec0075" "titulo" => "Weakness" "secciones" => array:1 [ 0 => array:2 [ …2] ] ] 1 => array:3 [ "identificador" => "sec0085" "titulo" => "Myotonia" "secciones" => array:1 [ 0 => array:2 [ …2] ] ] 2 => array:3 [ "identificador" => "sec0095" "titulo" => "Myalgia" "secciones" => array:1 [ 0 => array:2 [ …2] ] ] 3 => array:3 [ "identificador" => "sec0105" "titulo" => "Fatigue" "secciones" => array:1 [ 0 => array:2 [ …2] ] ] ] ] 1 => array:3 [ "identificador" => "sec0115" "titulo" => "Central nervous system problems" "secciones" => array:5 [ 0 => array:2 [ "identificador" => "sec0120" "titulo" => "Intellectual retardation" ] 1 => array:2 [ "identificador" => "sec0125" "titulo" => "Sleep disorders" ] 2 => array:2 [ "identificador" => "sec0130" "titulo" => "Depression and personality disorders" ] 3 => array:2 [ "identificador" => "sec0135" "titulo" => "Cerebrovascular disease" ] 4 => array:2 [ "identificador" => "sec0140" "titulo" => "Neuroimaging" ] ] ] ] ] 8 => array:3 [ "identificador" => "sec0145" "titulo" => "Cardiac involvement" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0150" "titulo" => "Sudden death, arrhythmias and conduction disorders" ] 1 => array:2 [ "identificador" => "sec0155" "titulo" => "Myocardial involvement" ] ] ] 9 => array:2 [ "identificador" => "sec0160" "titulo" => "Respiratory involvement" ] 10 => array:3 [ "identificador" => "sec0165" "titulo" => "Affectation of other organs and systems" "secciones" => array:3 [ 0 => array:3 [ "identificador" => "sec0170" "titulo" => "Dermatology" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0175" "titulo" => "Multiple pilomatricomas (calcifying epithelioma of Malherbe)" ] 1 => array:2 [ "identificador" => "sec0180" "titulo" => "Alopecia" ] 2 => array:2 [ "identificador" => "sec0185" "titulo" => "Seborrhoeic dermatitis" ] 3 => array:2 [ "identificador" => "sec0190" "titulo" => "Dysplastic nevus, not associated with melanoma" ] ] ] 1 => array:3 [ "identificador" => "sec0195" "titulo" => "Endocrinology and metabolism" "secciones" => array:6 [ 0 => array:2 [ "identificador" => "sec0200" "titulo" => "Hypogonadotropic hypogonadism" ] 1 => array:2 [ "identificador" => "sec0205" "titulo" => "Alterations of hydrocarbon metabolism" ] 2 => array:2 [ "identificador" => "sec0210" "titulo" => "Lipid alterations" ] 3 => array:2 [ "identificador" => "sec0215" "titulo" => "Phospho-calcium metabolism" ] 4 => array:2 [ "identificador" => "sec0220" "titulo" => "Thyroid metabolism" ] 5 => array:2 [ "identificador" => "sec0225" "titulo" => "Corticotrope axis" ] ] ] 2 => array:3 [ "identificador" => "sec0230" "titulo" => "Gastrointestinal involvement" "secciones" => array:6 [ 0 => array:2 [ "identificador" => "sec0235" "titulo" => "Alterations in the chewing process" ] 1 => array:2 [ "identificador" => "sec0240" "titulo" => "Dysphagia" ] 2 => array:2 [ "identificador" => "sec0245" "titulo" => "Ooesophagus and stomach" ] 3 => array:2 [ "identificador" => "sec0250" "titulo" => "Intestine" ] 4 => array:2 [ "identificador" => "sec0255" "titulo" => "Gallbladder" ] 5 => array:2 [ "identificador" => "sec0260" "titulo" => "Anus" ] ] ] ] ] 11 => array:2 [ "identificador" => "sec0265" "titulo" => "Genitourinary system" ] 12 => array:3 [ "identificador" => "sec0270" "titulo" => "Stomatology" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0275" "titulo" => "Caries, gingivitis, bacterial plaque" ] 1 => array:2 [ "identificador" => "sec0280" "titulo" => "Facial dysmorphism, ogival palate, mandibular retrognathia or prognathism" ] 2 => array:2 [ "identificador" => "sec0285" "titulo" => "Limited buccal opening, jaw pain and claudication associated with chewing" ] ] ] 13 => array:3 [ "identificador" => "sec0290" "titulo" => "Ophthalmology" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "sec0295" "titulo" => "Cataracts" ] 1 => array:2 [ "identificador" => "sec0300" "titulo" => "Ocular hypotension" ] 2 => array:2 [ "identificador" => "sec0305" "titulo" => "Eyelid ptosis" ] 3 => array:2 [ "identificador" => "sec0310" "titulo" => "Pregnancy" ] ] ] 14 => array:2 [ "identificador" => "sec0315" "titulo" => "Anaesthesia" ] 15 => array:2 [ "identificador" => "sec0320" "titulo" => "Myotonic dystrophy type 1 and cancer" ] 16 => array:3 [ "identificador" => "sec0325" "titulo" => "Special considerations in the paediatric age" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0330" "titulo" => "Congenital myotonic dystrophy" ] 1 => array:2 [ "identificador" => "sec0335" "titulo" => "Myotonic dystrophy of childhood onset" ] 2 => array:2 [ "identificador" => "sec0340" "titulo" => "Management of type 1 myotonic dystrophy in childhood" ] ] ] 17 => array:2 [ "identificador" => "sec0345" "titulo" => "Documents for care and attention in Emergency department and myotonic dystrophy type 1 emergencies" ] 18 => array:2 [ "identificador" => "sec0350" "titulo" => "Funding" ] 19 => array:2 [ "identificador" => "sec0355" "titulo" => "Conflict of interests" ] 20 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2018-09-07" "fechaAceptado" => "2018-10-18" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1226374" "palabras" => array:6 [ 0 => "Clinical guideline" 1 => "Steinert's disease" 2 => "Myotonic dystrophy type 1" 3 => "Complications" 4 => "Recommendations" 5 => "Dysphagia" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1226375" "palabras" => array:6 [ 0 => "Guía clínica" 1 => "Enfermedad de Steinert" 2 => "Distrofia miotónica tipo 1" 3 => "Complicaciones" 4 => "Recomendaciones" 5 => "Disfagia" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Background and objectives</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Steinert's disease or myotonic dystrophy type 1 (MD1), (OMIM <span class="elsevierStyleInterRef" id="intr0010" href="omim:160900">160900</span>), is the most prevalent myopathy in adults. It is a multisystemic disorder with dysfunction of virtually all organs and tissues and a great phenotypical variability, which implies that it has to be addressed by different specialities with experience in the disease. The knowledge of the disease and its management has changed dramatically in recent years. This guide tries to establish recommendations for the diagnosis, prognosis, follow-up and treatment of the complications of MD1.</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Material and methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Consensus guide developed through a multidisciplinary approach with a systematic literature review. Neurologists, pulmonologists, cardiologists, endocrinologists, neuropaediatricians and geneticists have participated in the guide.</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Recommendations</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The genetic diagnosis should quantify the number of CTG repetitions. MD1 patients need cardiac and respiratory lifetime follow-up. Before any surgery under general anaesthesia, a respiratory evaluation must be done. Dysphagia must be screened periodically. Genetic counselling must be offered to patients and relatives.</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusion</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">MD1 is a multisystemic disease that requires specialized multidisciplinary follow-up.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Background and objectives" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Material and methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Recommendations" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusion" ] ] ] "es" => array:3 [ "titulo" => "Resumen" "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Antecedentes y objetivos</span><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">La enfermedad de Steinert o distrofia miotónica tipo 1 (DM1), (OMIM 160900) es la miopatía más prevalente en el adulto. Es una enfermedad multisistémica con alteración de prácticamente todos los órganos y tejidos y una variabilidad fenotípica muy amplia, lo que implica que deba ser atendida por diferentes especialistas que dominen las alteraciones más importantes. En los últimos años se ha avanzado de manera exponencial en el conocimiento de la enfermedad y en su manejo. El objetivo de la guía es establecer recomendaciones para el diagnóstico, el pronóstico, el seguimiento y el tratamiento de las diferentes alteraciones de la DM1.</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Material y métodos</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Esta guía de consenso se ha realizado de manera multidisciplinar. Se ha contado con neurólogos, neumólogos, cardiólogos, endocrinólogos, neuropediatras y genetistas que han realizado una revisión sistemática de la literatura.</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Recomendaciones</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Se recomienda realizar un diagnóstico genético con cuantificación precisa de tripletes CTG. Los pacientes con DM1 deben seguir control cardiológico y neumológico de por vida. Antes de cualquier cirugía con anestesia general debe realizarse una evaluación respiratoria. Debe monitorizarse la presencia de síntomas de disfagia periódicamente. Debe ofrecerse consejo genético a los pacientes con DM1 y a sus familiares.</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Conclusión</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">La DM1 es una enfermedad multisistémica que requiere un seguimiento en unidades especializadas multidisciplinares.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Antecedentes y objetivos" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Material y métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Recomendaciones" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Conclusión" ] ] ] ] "NotaPie" => array:2 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Gutiérrez Gutiérrez G, Díaz-Manera J, Almendrote M, Azriel S, Eulalio Bárcena J, Cabezudo García P, et al. Guía clínica para el diagnóstico y seguimiento de la distrofia miotónica tipo 1, DM1 o enfermedad de Steinert. Med Clin (Barc). 2019;153:82.</p>" ] 1 => array:2 [ "etiqueta" => "☆☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">This Consensus Guide has obtained the scientific endorsements of the Spanish Society of Neurology (SEN), the Spanish Society of Endocrinology and Nutrition (SEEN) and the Familial Cardiomyopathies Group of the Spanish Society of Cardiology (SEC) and will be published in parallel in the journal Neurology, <span class="elsevierStyleInterRef" id="intr0005" href="https://doi.org/10.1016/j.nrl.2019.01.001">https://doi.org/10.1016/j.nrl.2019.01.001</span>, with the consent of the authors and editors.</p>" ] ] "apendice" => array:1 [ 0 => array:1 [ "seccion" => array:1 [ 0 => array:4 [ "apendice" => "<p id="par1105" class="elsevierStylePara elsevierViewall">The following are the supplementary data to this article:<elsevierMultimedia ident="upi0005"></elsevierMultimedia></p>" "etiqueta" => "Appendix A" "titulo" => "Supplementary data" "identificador" => "sec0365" ] ] ] ] "multimedia" => array:6 [ 0 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Speciality \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Disorder \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Recommendation \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Neurology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Genetic diagnosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">DMPK <span class="elsevierStyleItalic">Mutation</span> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Quantification of the number of CTG triplets (<span class="elsevierStyleItalic">n</span>) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Genetic counselling \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Offer to patient and family \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Muscle balance \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Weakness \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Muscular exploration every 2 years \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Moderate aerobic exercise \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Myotonia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Mexiletine 150–200<span class="elsevierStyleHsp" style=""></span>mg/8<span class="elsevierStyleHsp" style=""></span>h \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Fatigue, hypersomnia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Epworth test \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Modafinil 50–100<span class="elsevierStyleHsp" style=""></span>mg breakfast and lunch \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cognitive deterioration \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Neuropsychological evaluation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cardiology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Rhythm disorders \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Annual evaluation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">EPS study if PR<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>200<span class="elsevierStyleHsp" style=""></span>ms, QRS<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>100<span class="elsevierStyleHsp" style=""></span>ms or symptoms \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cardiomyopathy \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Echocardiogram every 3–5 years \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pulmonology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Restrictive effect \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Annual clinical assessment \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Annual spirometry \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Annual flu vaccination \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pneumococcal vaccination \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">SAHS \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Polysomnography \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Endocrinology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Dysphagia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Annual EAT-10 test \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Dysphagia test \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Nutritional recommendations \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Ophthalmology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cataracts \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Intervene \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Eyelid ptosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Blepharoplasty \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Dermatology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Seborrhoeic dermatitis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Vitamin D study \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Gastroenterology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Delayed gastric emptying \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Prokinetics \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Constipation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">High fibre diet, prokinetics, laxatives \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Diarrhoea (bacterial overgrowth syndrome) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Rifaximin 400/12 7 days \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Colelithiasis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If there are symptoms, then conventional treatment \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Stomatology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Caries, gingivitis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Evaluation by dentist \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Obstetrics \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pregnancy \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Follow up in high risk consultation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Anaesthesia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Respiratory complications \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Check with your usual pulmonologist \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Oncology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Increase in the incidence of cancer \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Conventional screening tests \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Other considerations \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Risk of inadequate treatment and management in the emergency room \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Emergency guides, emergency card or medical alert card \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2281237.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Summary of recommendations.</p>" ] ] 1 => array:8 [ "identificador" => "tbl0010" "etiqueta" => "Table 2" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at2" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Upon diagnosis \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Annually \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Every 2 years \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">When there are symptoms \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Later \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Muscle balance \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Muscular enzymes \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Electromyogram \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">At the discretion of the specialist \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Intelligence quotient and psychological evaluation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">At the discretion of the specialist \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Cardiology study \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Pulmonological study \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Study of dreams \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">At the discretion of the specialist \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Endocrine study \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">At the discretion of the specialist \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Dermatology \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">At the discretion of the specialist \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Digestive \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">At the discretion of the specialist \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Ophthalmological evaluation \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">X \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">At the discretion of the specialist \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Obstetrics \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If pregnant \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="" valign="\n \t\t\t\t\ttop\n \t\t\t\t"> \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2281239.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Recommended periodic evaluations.</p>" ] ] 2 => array:8 [ "identificador" => "tbl0015" "etiqueta" => "Table 3" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at3" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1. Patients with DM1, even asymptomatic patients with no apparent cardiac involvement, must receive cardiological follow-up for life, looking out for possible complications, given the progressive nature of the disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2. Patients with DM1 should receive information about the warning symptoms (syncope, palpitations) for which they should consult a professional urgently \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3. A disease-specific anamnesis is recommended every year (paying special attention to the existence of syncope and/or palpitations), as well as an ECG and an ECG holter \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4. A transthoracic echocardiogram is recommended every 3–5 years \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5. Cardiac structural alterations will be managed in the same manner as for the general population \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6 Asymptomatic patients with significant ECG abnormalities (PR<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>200<span class="elsevierStyleHsp" style=""></span>ms, QRS<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>100<span class="elsevierStyleHsp" style=""></span>ms) or those with a history of cardiogenic syncope could benefit from performing an invasive EPS to measure the conduction times and the risk of ventricular arrhythmia induction. In the case of patients with a normal EPS this should be repeated during follow-up if new symptoms appear, or if there is significant progression of the baseline ECG disorders (at least one increase<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>20<span class="elsevierStyleHsp" style=""></span>ms of the PR and/or QRS) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">7 Patients with HV<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>70<span class="elsevierStyleHsp" style=""></span>ms in the EPS or a second degree AVB in the ECG, due to the high rate of progression to a complete AVB, would benefit from the prophylactic implantation of a definitive pacemaker. The pacemaker does not completely prevent sudden death, because this can be produced by other mechanisms (ventricular arrhythmias, asystole) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8 Implantable cardioverter-defibrillators should be considered in patients with DM1 and a history of ventricular arrhythmias and/or LV dysfunction. In patients with sustained monomorphic ventricular tachycardia, an EPS should be performed and, if a branch-branch re-entry mechanism is verified, catheter ablation should be performed \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">9. The management of supraventricular arrhythmias in patients with DM1 is similar to that of other groups of patients; however, special caution should be exercised in the use of antiarrhythmic drugs that may prolong HV (especially those in group i) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 Sedation for electrical cardioversion in patients with DM1 should be performed with caution in an appropriate environment and by trained personnel, due to the risk of respiratory failure \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2281236.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Cardiological recommendations.</p>" ] ] 3 => array:8 [ "identificador" => "tbl0020" "etiqueta" => "Table 4" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at4" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">1. The assessment of respiratory dysfunction in patients with DM1 should be periodic \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">2. The respiratory history should include symptoms of dysphagia, cough efficacy, dyspnoea, diurnal hypersomnia, morning headaches and fatigue. The Epworth Sleepiness Scale is recommended.<a class="elsevierStyleCrossRef" href="#bib0905"><span class="elsevierStyleSup">33</span></a> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">3. The initial study should include spirometry, plethysmography, maximal inspiratory and expiratory pressure measurements (MIP and MEP) or SNIP, peak expiratory cough flow (PECF), baseline gasometry and sleep cardiorespiratory polygraph (CRP) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">4. A night polysomnograph (PSG) is recommended in case of diurnal hypersomnia with normal or pathological CRP and with no response to nocturnal ventilatory therapy \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">5. CPAP treatment is recommended if the SAHS is moderate or severe and there is an absence of nocturnal hypoventilation (CT90<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>30%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">6 Treatment with non-invasive mechanical ventilation (BiPAP) is recommended if nocturnal hypoventilation predominates (CT90<span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>30%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">7 Initial intra- or extra-hospital training of nocturnal devices is recommended to improve adherence to treatment \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">8 Early antibiotic treatment is recommended in the case of respiratory infections \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">9. Teaching respiratory physiotherapy techniques and the use of mechanical assistance for coughing with insufflation/exsufflation patterns (PECF<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>270) for the management of secretions is recommended. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">10 Annual flu vaccinations, and a pneumococcal vaccine at least once in a lifetime \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">11. A respiratory evaluation should be performed before any surgery with general anaesthesia \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2281238.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Pulmonological recommendations.</p>" ] ] 4 => array:8 [ "identificador" => "tbl0025" "etiqueta" => "Table 5" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at5" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:1 [ "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Disorder \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Screening \t\t\t\t\t\t\n \t\t\t\t\t\t</th><th class="td" title="\n \t\t\t\t\ttable-head\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Recommendation (if alteration) \t\t\t\t\t\t\n \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Male hypogonadism \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Testosterone, LH, FSH at diagnosis and annual or if symptoms \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If low testosterone: refer to endocrinologyEvaluate testosterone supplementation in males \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Alterations of hydrocarbon metabolism \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Basal plasma glucose, insulin, annual HbA1c or if cardinal symptoms request before testing \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If criteria of diabetes, hyperinsulinism or prediabetes: assess metforminRefer to endocrinology if diabetes or prediabetes \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Dyslipidaemia \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Lipid profile every 6–12 months \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Blood pressure controlHealthy nutritionPhysical exerciseIndividualized treatment Caution with statins and fibrates \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Vitamin D deficiency \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">25-0H-Vitamin D per year \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Vitamin D supplements<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>calcium (if low intake) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Primary hyperparathyroidism \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Calcium, phosphorus and annual PTH. Confirm alteration in 2 determinations \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If high calcium and/or high PTH in 2 determinations: refer to endocrinology \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Thyroid disorders \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">TSH and thyroid region exploration once a year. Confirm TSH alteration in 2 determinations \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If TSH alteration or goitre/thyroid nodules are felt: refer to endocrinology \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Hypocortisolism \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If suggestive symptoms: Order a cortisol in blood test at 8:00<span class="elsevierStyleHsp" style=""></span>h \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If cortisol <<span class="elsevierStyleHsp" style=""></span>18<span class="elsevierStyleHsp" style=""></span>μg/dl: refer to Endocrinology \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Hypercortisolism \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If suggestive symptoms: Order 24<span class="elsevierStyleHsp" style=""></span>h urinary free cortisolA 1<span class="elsevierStyleHsp" style=""></span>mg dexamethasone suppression starting at 23:00<span class="elsevierStyleHsp" style=""></span>h the previous daySalivary cortisol \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If urinary or salival cortisol is elevated or plasma cortisol at 8.00<span class="elsevierStyleHsp" style=""></span>h after 1<span class="elsevierStyleHsp" style=""></span>mg dexamethasone<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>1.8<span class="elsevierStyleHsp" style=""></span>μg/dl: refer to endocrinology \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Electrolyte alterations \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">Sodium and potassium in plasma annually. If altered: repeat blood and urine tests \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="\n \t\t\t\t\ttop\n \t\t\t\t">If electrolyte alterations in 2 determinations: refer to endocrinology \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2281240.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">Endocrinological recommendations.</p>" ] ] 5 => array:5 [ "identificador" => "upi0005" "tipo" => "MULTIMEDIAECOMPONENTE" "mostrarFloat" => false "mostrarDisplay" => true "Ecomponente" => array:2 [ "fichero" => "mmc1.docx" "ficheroTamanyo" => 28001 ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:148 [ 0 => array:3 [ "identificador" => "bib0745" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ …2] ] "host" => array:1 [ 0 => array:2 [ …2] ] ] ] ] 1 => array:3 [ "identificador" => "bib0750" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ …2] ] "host" => array:1 [ 0 => array:1 [ …1] ] ] ] ] 2 => array:3 [ "identificador" => "bib0755" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:1 [ "referenciaCompleta" => "Grupo de trabajo sobre GPC. 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