Case report
Anesthesia in a pediatric patient with ROHADD syndrome
Anestesia en paciente pediátrico con síndrome de Rohhad
E. Esparza Isasa, M.A. Palomero Rodríguez
, I. Acebedo Bambaren, C. Medrano Viñas, D. Gil Mayo, F. Domínguez Pérez, D. Pestaña Lagunas
Corresponding author
Servicio de Anestesiología y Reanimación, Hospital Universitario Ramón y Cajal, Madrid, Spain
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Esparza Isasa, M.A. Palomero Rodríguez, I. Acebedo Bambaren, C. Medrano Viñas, D. Gil Mayo, F. Domínguez Pérez, D. Pestaña Lagunas" "autores" => array:7 [ 0 => array:2 [ "nombre" => "E." "apellidos" => "Esparza Isasa" ] 1 => array:4 [ "nombre" => "M.A." "apellidos" => "Palomero Rodríguez" "email" => array:1 [ 0 => "mapalomero@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:2 [ "nombre" => "I." "apellidos" => "Acebedo Bambaren" ] 3 => array:2 [ "nombre" => "C." "apellidos" => "Medrano Viñas" ] 4 => array:2 [ "nombre" => "D." "apellidos" => "Gil Mayo" ] 5 => array:2 [ "nombre" => "F." "apellidos" => "Domínguez Pérez" ] 6 => array:2 [ "nombre" => "D." "apellidos" => "Pestaña Lagunas" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Anestesiología y Reanimación, Hospital Universitario Ramón y Cajal, Madrid, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Anestesia en paciente pediátrico con síndrome de Rohhad" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2023 "Ancho" => 3333 "Tamanyo" => 603415 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Polysomnography performed some months earlier that showed persistent hypoventilation with desaturation and hypercapnia, with worsening compared to previous studies.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Rapid onset obesity, hipoventilation, hypothalamic dysfunction and autonomic dysregulation (ROHHAD), was first described by Ize-Ludlow et al.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> There are now 100 confirmed cases worldwide. Although it has been linked for many years with late-onset congenital central hypoventilation syndrome (LO-CCHS), clinically and genetically it is a totally different entity. ROHHAD syndrome is characterised by the late onset of respiratory symptoms (starting at age 3 years in ROHHAD vs 3 months in LO-CCHS), excessive weight gain, and absence of the typical mutation in the PHOX2B gene found in LO-CCHS syndrome.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">The clinical characteristics of ROHHAD syndrome can vary, but a common, and indeed defining, symptom is the onset of hyperphagia with rapid and excessive weight gain from the age of 2–4 years in previously normal children, central alveolar hypoventilation, and other alterations in the following years, such as hypothalamic dysfunction, alteration of the hydrosaline metabolism and signs of dysautonomia.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">3–6</span></a> ROHHAD patients often present early obstructive sleep apnoea, and behavioural, language and cognitive development disorders; approximately 40% of cases described today have been associated with neural crest tumours, such as ganglioneuromas and ganglioneuroblastomas. All these characteristics make the anaesthetic management of these patients particularly challenging, and a careful anesthesia strategy is essential. We present the case of a patient with ROHHAD syndrome who received several anaesthetic drugs in our hospital.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0015" class="elsevierStylePara elsevierViewall">A 10-year-old boy, weight 66.5<span class="elsevierStyleHsp" style=""></span>kg (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>99; 3.26 SD), height 136<span class="elsevierStyleHsp" style=""></span>cm (<span class="elsevierStyleItalic">p</span>37, −0.34 SD), BMI 34.92<span class="elsevierStyleHsp" style=""></span>kg/m<span class="elsevierStyleSup">2</span> (<span class="elsevierStyleItalic">p</span><span class="elsevierStyleHsp" style=""></span>><span class="elsevierStyleHsp" style=""></span>99; 5.14 SD) and body surface area 1.6<span class="elsevierStyleHsp" style=""></span>m<span class="elsevierStyleSup">2</span>, diagnosed with ROHHAD syndrome, was admitted to the pediatric intensive care unit (PICU) for hyponatraemia and remained there for 9 days. He presented rapid onset obesity that started when he was 5 years, together with dyslipidaemia, hypothalamic dysfunction with electrolyte imbalance with frequent decompensations, and multiple hospital admissions (severe hyponatraemia and hypernatraemia with seizures), multifactorial hypothyroidism, central adrenal insufficiency that was being treated with corticosteroids, hyperprolactinaemia, polydipsia and polyphagia. He also presented severe central hypoventilation and daytime hypersomnia, for which he was receiving intermittent supplemental oxygen via nasal cannula due to intolerance of non-invasive ventilation, and modafinil and melatonin, with persistent desaturation and hypercapnia (baseline TcCO<span class="elsevierStyleInf">2</span> 52<span class="elsevierStyleHsp" style=""></span>mmHg) on serial polysomnography (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>); aggressive conduct disorder, compulsive intake of water and food, and autonomic dysregulation with fever, diaphoresis, irregular pulse, blood pressure and temperature. Presence of neural crest tumours had been ruled out 1 year previously, and a clonidine stimulation test elicited no changes in growth hormone levels, with IFG-1 levels in the lower limit of normal.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Given the clinical complexity of the patient, the difficulty in placing a peripheral line, and frequent hospitalisation due to decompensations, once hyponatraemia had been resolved, we decided to place a central port-a-cath. During a previous MRI performed under sedation due to non-collaboration, the patient had presented an episode of apnoea in the course of face-mask administration of sevoflurane (inspired fraction 3), and required manual ventilation.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Before anesthesia, the patient was premedicated with 5<span class="elsevierStyleHsp" style=""></span>mg oral midazolam in the pre-op room. Once in the operating room, non-invasive blood pressure, SpO<span class="elsevierStyleInf">2</span> and ECG monitoring was started and inhalational anesthesia induction commenced with sevoflurane (5% inspired concentration increasing to 8%). During induction, with an expired fraction of 3, the patient developed apnoea, and SpO<span class="elsevierStyleInf">2</span> fell to 85%. Due to the difficulty in securing a peripheral line and in delivering mask ventilation, a no. 3 laryngeal Ambu<span class="elsevierStyleSup">®</span> airway was inserted without incident, allowing us to ventilate the patient while a 24 G catheter was placed in the upper left arm. Once the intravenous line was secure, 1<span class="elsevierStyleHsp" style=""></span>μg/kg fentanyl was administered, which again resulted in apnoea (Etsevo 2.5), and the patient was intubated with a no. 6 Portex tracheal tube without incident (laryngoscopy IIB).</p><p id="par0030" class="elsevierStylePara elsevierViewall">Anesthesia was maintained with ETsevo 2 and 0.05–0.1<span class="elsevierStyleHsp" style=""></span>μg/kg/min remifentanil. The patient presented high peak pressure (42<span class="elsevierStyleHsp" style=""></span>cmH<span class="elsevierStyleInf">2</span>O) accompanied by expiratory wheezing which subsided partially after administration of 60<span class="elsevierStyleHsp" style=""></span>mg IV methylprednisolone. After completion of the procedure, coinciding with the administration of 2<span class="elsevierStyleHsp" style=""></span>g IV metamizole (Nolotil<span class="elsevierStyleSup">®</span>), the patient presented wheezing and high peak ventilator pressure, with less than 70% desaturation and hypotension (65/35<span class="elsevierStyleHsp" style=""></span>mmHg), which was treated with 1<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>1<span class="elsevierStyleHsp" style=""></span>mg epinephrine. Following this, the patient was transferred to the pediatric intensive care unit for postoperative monitoring and care, with perfusion of noradrenaline to maintain pulse and blood pressure. The patient was extubated at 48<span class="elsevierStyleHsp" style=""></span>h without incident, and was subsequently diagnosed with an anaphylactoid reaction to magnesium metamizol (Nolotil<span class="elsevierStyleSup">®</span>).</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">The ROHHAD syndrome is a very rare, complex disorder, characterised by the onset of hyperphagia, obesity and alveolar hypoventilation from the age of 2–4 years in previously normal children,<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1–5</span></a> together with hypothalamic dysfunction, electrolyte imbalance, and signs of autonomic dysregulation.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">3,5,7</span></a> Our patient presented most of the symptoms associated with this syndrome, such as obesity, autonomic alterations and central hypoventilation, together with obstructive sleep apnoea syndrome, behavioural disorder and electrolyte imbalance, the latter being the reason for admission (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>).</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Anaesthetic management in these patients is complex, mainly because of their obstructive sleep apnoea and associated central alveolar hypoventilation, but also because of electrolyte imbalance and symptoms associated with hypothalamic dysfunction, particularly decreased gastric emptying secondary to gastroparesis, and irregular pulse rate and blood pressure.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5,8,9</span></a> During anesthesia induction, our patient presented severe central hypoventilation with signs of desaturation and hypercapnia on polysomnography. We decided to intubate him during the procedure in order to protect the airway against possible intraoperative aspiration. Clinical cases in the literature recommend inhalation induction with sevoflurane with 100% oxygen, maintaining spontaneous ventilation until there are clear signs that the patient can be intubated.<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">7–9</span></a> However, one of the main problems encountered during anesthesia was the presence of apnoea during inhalation induction with sevoflurane. We believe that severe central hypoventilation associated with premedication with benzodiazepines and subsequent administration of fentanyl were the primary factors that led to onset of apnoea during anaesthetic induction. This, coupled with the difficulty in placing a peripheral line due to the patient's obesity compelled us to use a laryngeal mask airway to maintain adequate oxygenation during peripheral cannulation. Other authors have reported inducing sedation with intravenous ketamine and dexmedetomidine as a safe option in this type of patient, since opioids depress the hypoxic response, decrease the respiratory rate and create a right-shift in CO<span class="elsevierStyleInf">2</span> response curves, thus facilitating hypercapnia. We believe that an opioid-sparing alternative, with deep sedation and regional anesthesia of the chest wall with no perception and normal physiological response in situations of hypoxaemia and hypercapnia, would have been the best anaesthetic option, but had to be ruled out due to the patient's aggressive conduct disorder.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Regarding haemodynamic instability with autonomic dysregulation, propofol or thiopental has been shown to increase the incidence of hypotension secondary to autonomic dysregulation in these patients.<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">7–9</span></a> We decided to maintain anesthesia with sevoflurane and an ultra-short-acting opioid, which allowed us to maintain good blood pressure and heart rate at all times. However, metamizole magnesium (Nolotil<span class="elsevierStyleSup">®</span>) triggered a reaction characterised by respiratory and haemodynamic symptoms that required the use of vasopressors and inotropes for 24<span class="elsevierStyleHsp" style=""></span>h to maintain blood pressure. In the absence of diagnostic confirmation of anaphylactic and anaphylactoid reaction, we believe that could have been related to the autonomic dysregulation found in these patients.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0050" class="elsevierStylePara elsevierViewall">ROHHAD syndrome is a rare disorder with onset in children aged 2–4 years. It is characterised by hyperphagia, hypothalamic dysfunction, central hypoventilation and autonomic dysregulation. The main perioperative considerations to be borne in mind include the presence of hypoventilation along with the problem derived from obesity and associated autonomic dysregulation.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflicts of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres1103955" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1044196" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1103956" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1044197" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conclusion" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflicts of interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2017-12-15" "fechaAceptado" => "2018-03-06" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1044196" "palabras" => array:8 [ 0 => "Central hypoventilation syndrome" 1 => "ROHHAD syndrome" 2 => "Obesity" 3 => "Autonomic dysregulation" 4 => "Sleep apnoea" 5 => "General anesthesia" 6 => "Hypothalamic dysfunction" 7 => "Sleep disorder breathing" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1044197" "palabras" => array:8 [ 0 => "Síndrome de hipoventilación central" 1 => "Síndrome de ROHHAD" 2 => "Obesidad" 3 => "Disfunción autonómica" 4 => "Síndrome de apnea del sueño" 5 => "Anestesia general" 6 => "Disfunción hipotalámica" 7 => "Trastorno del sueño" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare entity that is characterised by its onset in healthy children at 2–4 years of age. It is a complex syndrome that includes, among other symptoms, rapid weight gain with hyperphagia, hypothalamic dysfunction, central hypoventilation, and autonomic dysregulation. The case is presented of a 10-year-old boy with a diagnosis of ROHHAD syndrome undergoing insertion of a port-a-cath under general anesthesia, who developed complications during the anaesthetic procedure related to his illness. The peri-operative management of these patients represents a challenge for the anaesthetist, given the involvement of multiple systems and the frequent respiratory comorbidities associated with them. A summary is presented of some of the implications and anaesthetic considerations that must be taken into account in the management of these patients.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El síndrome obesidad de rápida progresión, hipoventilación alveolar, disfunción hipotalámica y disregulación autonómica (ROHHAD) es una entidad infrecuente caracterizada por un comienzo en niños sanos a los 2-4 años. Se trata de un síndrome complejo caracterizado por una rápida ganancia de peso con hiperfagia, disfunción hipotalámica, hipoventilación central y disregulación autonómica, entre otros síntomas. Presentamos el caso de un niño de 10 años con diagnóstico de síndrome de ROHHAD a quien se colocó un <span class="elsevierStyleItalic">porth-a-cath</span> bajo anestesia general y que desarrolló complicaciones durante el procedimiento anestésico relacionadas con su enfermedad. El manejo perioperatorio de estos pacientes supone todo un reto para el anestesista dada la afectación de múltiples sistemas y las frecuentes comorbilidades respiratorias que asocian. Se resumen algunas de las implicaciones y consideraciones anestésicas que hay que tener en cuenta en el manejo de estos pacientes.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Esparza Isasa E, Palomero Rodríguez MA, Acebedo Bambaren I, Medrano Viñas C, Gil Mayo D, Domínguez Pérez F, et al. Anestesia en paciente pediátrico con síndrome de Rohhad. Rev Esp Anestesiol Reanim. 2018;65:525–529.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2023 "Ancho" => 3333 "Tamanyo" => 603415 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Polysomnography performed some months earlier that showed persistent hypoventilation with desaturation and hypercapnia, with worsening compared to previous studies.</p>" ] ] 1 => 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=""><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Respiratory manifestations</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Central alveolar hypoventilation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Reduction in the ventilatory response to carbon dioxide \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Sleep apnoea \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Autonomic dysregulation</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Changes in blood pressure (labile blood pressure) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Body temperature dysregulation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Alternations in gastrointestinal motility (delay in gastric emptying and possible full stomach) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Decreased sensation of pain \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Altered sweating \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Low heart rhythm that may require a cardiac pacemaker \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Neural crest tumours \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Seizures \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleItalic">Hypothalamic dysfunction</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Rapid weight gain, hyperphagia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Impaired growth hormone secretion \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Early or late puberty \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Polydipsia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Hyponatraemia: central pontine myelinolysis, spastic quadriplegia and involvement of cranial nerves, with impaired level of consciousnes \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Hipernatraemia: diabetes insipidus requiring desmopressin and very strict control of electrolyte balance \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Central adrenal insufficiency \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Hyperprolactinaemia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Diabetes insipidus \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Hypothyroidism \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Adrenal insufficiency, with possible need for chronic hydrocortisone \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Polyuria \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top"><span class="elsevierStyleHsp" style=""></span>Type 2 diabetes mellitus \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1888832.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Summary of possible complications to be considered in the management of this type of patient.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib0050" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation presenting in childhood" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "D. 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Chung" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1097/ALN.0b013e31825add60" "Revista" => array:6 [ "tituloSerie" => "Anesthesiology" "fecha" => "2012" "volumen" => "117" "paginaInicial" => "188" "paginaFinal" => "205" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22614131" "web" => "Medline" ] ] ] ] ] ] ] ] 7 => array:3 [ "identificador" => "bib0085" "etiqueta" => "8" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Perioperative care of a child with ROHHADNET Syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "A. Goykhman" 1 => "J. Fitch" 2 => "J.D. Tobias" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "J Med Cases" "fecha" => "2013" "volumen" => "4" "paginaInicial" => "710" "paginaFinal" => "714" ] ] ] ] ] ] 8 => array:3 [ "identificador" => "bib0090" "etiqueta" => "9" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Noninvasive ventilation in a child with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "L. Luccoli" 1 => "M. Ellena" 2 => "I. Esposito" 3 => "E. Bignamini" 4 => "C. Gregoretti" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Minerva Anestesiol" "fecha" => "2012" "volumen" => "78" "paginaInicial" => "1171" "paginaFinal" => "1172" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/23059524" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23411929/0000006500000009/v1_201811070606/S2341192918301549/v1_201811070606/en/main.assets" "Apartado" => array:4 [ "identificador" => "67195" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Case Reports" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23411929/0000006500000009/v1_201811070606/S2341192918301549/v1_201811070606/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2341192918301549?idApp=UINPBA00004N" ]
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