Original Article
Long-term follow-up of ninety eight Iranian patients with primary immune deficiency in a single tertiary centre
M. Nabavia, S. Arshia, M.H. Bemaniana, A. Aghamohammadib, D. Mansouric, M. Hedayatd, A. Nateghiane, S. Noorbakhshf, F. Ehsanipourf, M. Faranoushg, R. Shakerih, M. Mesdaghii, B. Taghvaeia, B. Ghalebaghia,j, D. Babaiek, A. Bahramia, M. Fallahpoura, H. Esmaeilzadehl, A. Ali Hamidiehm, M. Rekabia..., J. Ahmadiana, N. Eslamia, S. Shokria, M. Afsharn, F. Jalalia, N. Akbarpoura, R. Molatefia,
, N. Rezaeib,o,pVer más
Corresponding author
a Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran
b Research Center for Immunodeficiency, Children's Medical Center, Tehran University of Medical Sciences (TUMS), Tehran, Iran
c Department of Internal Medicine, Division of Infectious Diseases and Clinical Immunology, National Research Institute of Tuberculosis and Lung Diseases, Masih-e-Daneshvari Hospital, Shaheed Beheshti University of Medical Sciences (SBUMS), Tehran, Iran
d Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA
e Department of Infectious Diseases, Ali-Asghar Hospital, IUMS, Tehran, Iran
f Research Center of Pediatric Infectious Diseases, Rasool-e-Akram Hospital, IUMS, Tehran, Iran
g Department of Pediatric Oncology, Rasool-e-Akram Hospital, IUMS, Tehran, Iran
h Digestive Oncology Research Center, Digestive Disease Research Institute, TUMS, Tehran, Iran
i Department of Immunology, Mofid Children's Hospital, SBUMS, Tehran, Iran
j Research Center of Otolaryngology and Head & Neck Surgery, IUMS, Tehran, Iran
k Department of Allergy and Clinical Immunology, Mofid Hospital, SBUMS, Tehran, Iran
l Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
m Hematology Oncology and Stem Cell Transplantation Research Center, TUMS, Iran
n Antimicrobial Resistance Research Center, Rasool-e-Akram Hospital, IUMS, Tehran, Iran
o Molecular Immunology Research Center, and Department of Immunology, School of Medicine, TUMS, Tehran, Iran
p Universal Scientific Education and Research Network (USERN), Tehran, Iran
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While patients with CVID have higher relative counts of naive cells than the control group, they were found to have lower relative counts of memory cells, relative counts of switched cells, and relative and absolute counts of non-switched cells. C: Control; L: Lymphocyte; N: Naive; M: Memory; S: Switched; NS: Non-switched.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M.H. Celiksoy, A. Yildiran" "autores" => array:2 [ 0 => array:2 [ "nombre" => "M.H." "apellidos" => "Celiksoy" ] 1 => array:2 [ "nombre" => "A." 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Masih-e-Daneshvari Hospital, Shaheed Beheshti University of Medical Sciences (SBUMS), Tehran, Iran" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA" "etiqueta" => "d" "identificador" => "aff0020" ] 4 => array:3 [ "entidad" => "Department of Infectious Diseases, Ali-Asghar Hospital, IUMS, Tehran, Iran" "etiqueta" => "e" "identificador" => "aff0025" ] 5 => array:3 [ "entidad" => "Research Center of Pediatric Infectious Diseases, Rasool-e-Akram Hospital, IUMS, Tehran, Iran" "etiqueta" => "f" "identificador" => "aff0030" ] 6 => array:3 [ "entidad" => "Department of Pediatric Oncology, Rasool-e-Akram Hospital, IUMS, Tehran, Iran" "etiqueta" => "g" "identificador" => "aff0035" ] 7 => array:3 [ "entidad" => "Digestive Oncology Research Center, Digestive Disease Research Institute, TUMS, Tehran, Iran" "etiqueta" => "h" "identificador" => "aff0040" ] 8 => array:3 [ "entidad" => "Department of Immunology, Mofid Children's Hospital, SBUMS, Tehran, Iran" "etiqueta" => "i" "identificador" => "aff0045" ] 9 => array:3 [ "entidad" => "Research Center of Otolaryngology and Head & Neck Surgery, IUMS, Tehran, Iran" "etiqueta" => "j" "identificador" => "aff0050" ] 10 => array:3 [ "entidad" => "Department of Allergy and Clinical Immunology, Mofid Hospital, SBUMS, Tehran, Iran" "etiqueta" => "k" "identificador" => "aff0055" ] 11 => array:3 [ "entidad" => "Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran" "etiqueta" => "l" "identificador" => "aff0060" ] 12 => array:3 [ "entidad" => "Hematology Oncology and Stem Cell Transplantation Research Center, TUMS, Iran" "etiqueta" => "m" "identificador" => "aff0065" ] 13 => array:3 [ "entidad" => "Antimicrobial Resistance Research Center, Rasool-e-Akram Hospital, IUMS, Tehran, Iran" "etiqueta" => "n" "identificador" => "aff0070" ] 14 => array:3 [ "entidad" => "Molecular Immunology Research Center, and Department of Immunology, School of Medicine, TUMS, Tehran, Iran" "etiqueta" => "o" "identificador" => "aff0075" ] 15 => array:3 [ "entidad" => "Universal Scientific Education and Research Network (USERN), Tehran, Iran" "etiqueta" => "p" "identificador" => "aff0080" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "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" => 1361 "Ancho" => 1597 "Tamanyo" => 62400 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Number of newly diagnosed patients with primary immune deficiency at Rasool-E-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran between 1990 and 2014.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Primary immunodeficiency disorders (PID) are rare inherited diseases of the immune system that present with heterogeneous infectious and autoimmune manifestations and higher incidence of malignancies.<a class="elsevierStyleCrossRefs" href="#bib0205"><span class="elsevierStyleSup">1–6</span></a> The International Union of Immunological Societies (IUIS) Expert Committee classified PID in nine main groups.<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">7</span></a> Overall, the prevalence of PID is estimated to be one in 1200 American people and appears to be underdiagnosed.<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">2</span></a> The aim of this study was to summarise the clinical manifestations and long-term outcome of 98 Iranian PID patients in a single tertiary centre.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Method</span><p id="par0010" class="elsevierStylePara elsevierViewall">This was a single-centre retrospective study carried out at Allergy and Clinical Immunology Department of Rasool-E-Akram, an 830-bed tertiary referral hospital, in Tehran, the capital city of Iran, from January 1989 to July 2014 (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The diagnosis of PID was made after secondary forms of immunodeficiency were ruled out and according to clinical and laboratory findings and diagnostic criteria of IUIS, PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies).<a class="elsevierStyleCrossRefs" href="#bib0205"><span class="elsevierStyleSup">1,7,8</span></a> Genetic testing (to confirm the diagnosis of X linked agammaglobulinaemia, X linked hyper IgM, LRBA deficiency, DOCK8 deficiency, STAT1 and HAX1) was done as part of genetic consultation with Research Center for Immunodeficiency in Children's Medical Center in Tehran University of Medical Sciences. Only patients with a definite diagnosis of PID were enrolled for the purpose of the study. Demographic, clinical and immunological data were collected using a standardised questionnaire.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Serological and immunological laboratory testing included quantitative serum immunoglobulin levels, IgG subclass levels, pre- and post-immunisation antibody titres to Pneumococcus, Diphtheria and Tetanus, isohaemagglutinin titres, flow cytometric assessment of lymphocyte subsets, lymphocyte transformation test, nitroblue tetrazolium test, complement component (C3, C4, CH50) tests and delayed type hypersensitivity skin testing to Candida antigen.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The majority of the patients were diagnosed at Rasool-E-Akram Hospital, whereas some of the older patients were initially diagnosed at other institutes (Mofid Hospital, Ali-Asghar Hospital and Children's Medical Center) and were referred for continued treatment and follow-up. The patients were routinely visited at 1–3 months interval.</p><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Statistical analyses</span><p id="par0030" class="elsevierStylePara elsevierViewall">Statistical analyses were performed using STATA 10 software. Quantitative data were reported as mean<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>SD and descriptive variables as proportion. <span class="elsevierStyleItalic">T</span>-test was used to compare means and chi-square test was used to compare proportions. <span class="elsevierStyleItalic">P</span>-values less than 0.05 were considered statistically significant.</p></span></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Results</span><p id="par0035" class="elsevierStylePara elsevierViewall">Ninety-eight patients were diagnosed with 15 different disorders in five main groups. <a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> describes demographic characteristics and mortality rates of these patients. Nineteen patients (19%) aged 17.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>18.2 years died during the follow-up period due to severe infections, malignancies, graft versus host disease and hepatic failure.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Predominantly Antibody Deficiencies</span><p id="par0040" class="elsevierStylePara elsevierViewall">PAD was the most common diagnosis and included: common variable immunodeficiency (CVID), X-linked agammaglobulinaemia (XLA), hyper-IgM (HIGM) syndrome, thymoma with hypogammaglobulinaemia (Good's syndrome), and hypogammaglobulinaemia with mutations in lipopolysaccharide-responsive beige-like anchor (LRBA) gene (<a class="elsevierStyleCrossRefs" href="#tbl0005">Tables 1 and 2</a>). All patients were treated with intravenous immune globulin (IVIG) at least on a monthly basis, and those with severe bronchiectasis or recurrent bacterial infections were treated with prophylactic antibiotics.</p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0045" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">CVID</span> – Recurrent infections (85%) and autoimmune manifestations (15%) were first presentations in these patients. The age at onset in patients with parental consanguinity was significantly lower than the others (7.7<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>7.8 versus 16.3<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>11 years; <span class="elsevierStyleItalic">P</span> value<span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>0.001). There was no relationship between CVID phenotypes or complications such as autoimmunity or malignancy and parental consanguinity. A total of 12 (22%) patients had severe viral infections, including cytomegalovirus (gastroenteritis, colitis, pneumonia), varicella zoster virus (disseminated varicella, pneumonia), human papillomavirus (recurrent warts), herpes simplex virus (severe skin infections, gingivostomatitis), and John Cunningham virus (progressive multifocal leukoencephalopathy). Fungal infections occurred in three patients and included recurrent oral thrush and Pneumocystis jiroveci pneumonia. Twenty-five (45%) patients developed autoimmune manifestations, among them 12 patients had multiple autoimmune diseases. Six patients developed seven malignancies, including Hodgkin's disease (in three cases), pre B cell leukaemia, breast cancer, gastrointestinal adenocarcinoma and brain cancer. Six patients aged 35.4<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>17.2 years died during the follow-up period of 4.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>4.3 years due to sepsis (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>2), malignancy (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>2), viral CNS infection (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>1), and hepatic failure (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>1).</p><p id="par0050" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">XLA</span> – Six males aged 22.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>8.6 years were followed for mean 19.4<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>6.9 years. The diagnosis was confirmed with mutational analysis of the BTK gene in all the cases. One patient was diagnosed and treated for isolated growth hormone deficiency. No patients died during the follow-up period.</p><p id="par0055" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">HIGM syndrome</span> – This was diagnosed in one male patient with a history of recurrent infections and hypogammaglobulinaemia and the diagnosis was confirmed by mutational analysis of the CD40 ligand gene (CD40L – Exon 5 – c.187C>T). At the time of study, the patient was 20 years old with no history of severe infections or complications after proper therapeutic interventions were initiated.</p><p id="par0060" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Good's syndrome</span> – One male patient with a history of myasthenia gravis and thymoma, undergoing thymectomy, was diagnosed with Good's syndrome following the development of clinical and laboratory features of hypogammaglobulinaemia (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>). The patient died aged 49 years of severe acute graft-versus-host disease syndrome following transfusion of irradiated blood products.</p><p id="par0065" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">LRBA deficiency</span> – One seven-year-old male patient, referred at six years of age with a history of recurrent infections, agammaglobulinaemia and absent B-lymphocyte, who was diagnosed with genetic analysis. He developed severe pneumonia with Pneumocystis jiroveci and CMV six months after diagnosis, and was later diagnosed with bronchiolitis obliterans with organising pneumonia.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Combined T-cell and B-cell immunodeficiencies</span><p id="par0070" class="elsevierStylePara elsevierViewall">Twelve patients were diagnosed with CID and of them eight had severe combined immunodeficiency (six T−B+ and two T−B−) and four had less severe CID (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>). The most common presentations were pneumonia (100%), gastroenteritis (75%), chronic oral thrush (66%), and disseminated BCG infection after vaccination (25%). Severe viral (CMV, HSV) and fungal (Pneumocystis jiroveci) infections were diagnosed in 33 and 25% of patients respectively (<a class="elsevierStyleCrossRef" href="#tbl0015">Table 3</a>). Three patients developed autoimmune disorders, including ITP, vasculitis and eczema. Lymphopenia was observed in five (42%) patients: two (33%) of T−B+, two (100%) of T−B− and one patient (100%) with CD4 deficiency. A positive family history of diagnosed or suspected immune deficiency was reported in three patients. Patients were treated with IVIG, prophylactic antibiotics, antifungals and isoniazid (because of BCG vaccination) and were considered for haematopoietic stem-cell transplantation (HSCT). Only one patient (T−B+) had HSCT from a matched sibling donor after low intensity conditioning regimen with fludarabine, melphalan and anti thymocyte globulin therapy and was seven years old at the time of study with no long-term complication following bone marrow transplant. Eight patients died at the age of 36<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>34 months due to severe pneumonia, gastroenteritis, sepsis and disseminated BCG infection. Age at expiration was 29<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>31 months in T−B+, 8.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>3.5 months in T−B−, 72 and 84 months in CD4 deficiency and CD8 low patients respectively.</p><elsevierMultimedia ident="tbl0015"></elsevierMultimedia><p id="par0075" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Ataxia Telangiectasia Syndrome (ATS)</span> – Two patients were diagnosed with neurologic symptoms, telangiectasia and history of recurrent pneumonia and gastroenteritis. Both were treated with IVIG infusion, prophylactic antibiotics and pulmonary care. One of the patients with history of UTI, sepsis, severe bronchiectasis, pulmonary hypertension and respiratory failure, died at 10 years of age after a follow-up period of three years. The second patient, an eight-year-old boy at the time of study, was diagnosed with large B cell lymphoma and was under chemotherapy.</p><p id="par0080" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Dedicator of Cytokinesis 8 (DOCK8) deficiency</span> – One female patient was referred with history of severe eczema, recurrent urticaria and angioedema, recurrent sinopulmonary infections and BCG adenitis. Laboratory evidences included eosinophilia and normal immunoglobulin levels on multiple, separate occasions, except for an elevated IgE level of 534<span class="elsevierStyleHsp" style=""></span>IU/mL (reference range: <81) reported on one occasion. The diagnosis was confirmed by genetic analysis that showed large homozygous deletion in exon 6 through 15 of the DOCK8 gene. She was treated symptomatically and was candidate for HSCT. Her sister had a similar history and died at 11 years of age due to undiagnosed brain tumour.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Chronic Mucocutaneous Candidiasis</span><p id="par0085" class="elsevierStylePara elsevierViewall">Autoimmune manifestations of three CMCC patients included: hypothyroidism, hypoparathyroidism, Addison disease, autoimmune hepatitis, ITP, autoimmune haemolytic anaemia, alopecia and vitiligo. Increased susceptibility to bacterial infections, including sinopulmonary infections, perianal abscess, urinary tract infection, gingivitis and diarrhoea were identified in these patients during the follow up. One patient with heterozygous gain-of-function mutation in STAT1, who was 30 years old at the time of study, had developed bronchiectasis and pulmonary hypertension due to recurrent severe pulmonary infections. All patients received antifungal prophylaxis.</p><p id="par0090" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Chronic granulomatous disorder (CGD)</span> – The clinical manifestations and complications of eleven patients with CGD are summarised in <a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>. The inheritance pattern in four patients was X linked and in the others was autosomal recessive, according to pedigrees and maternal NBT results. Six patients had a positive family history. Patients were treated with itraconazole and co-trimoxazole prophylactically; however, not all patients were compliant to this regimen due to its cost and lack of insurance. Inaccessibility and cost precluded the use of interferon-gamma (IFN-γ) in any of the patients. None of the patients received bone marrow transplant. Two patients died of severe pneumonia and respiratory failure during the follow-up period.</p><elsevierMultimedia ident="tbl0020"></elsevierMultimedia><p id="par0095" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Leucocyte adhesion molecule deficiency type 1 (LAD1)</span> – All patients were presented with omphalitis and delayed separation of umbilical cord. Other manifestations included cellulitis, peritonitis, chronic diarrhoea, failure to thrive, septic arthritis, recurrent gingivitis, conjunctivitis and sepsis. Two cases were siblings with CD18 of 2%; one died of extra-nodal large B cell lymphoma at 11 years of age and the other one was 10 years old at the time of study. The other two patients had undetectable CD18 levels and were 4 and 12 months old, respectively. The patients were under prophylactic antibiotics while waiting to receive HSCT.</p><p id="par0100" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Kostmann disease</span> – One male patient with a history of neonatal sepsis, recurrent pneumonia, otitis media, cellulitis, skin abscess and gingivitis was diagnosed with congenital neutropenia due to mutation in <span class="elsevierStyleItalic">HAX1</span> gene. The patient treated with prophylactic antibiotics and G-CSF, with only mild neutropenia and no serious complication.</p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Discussion</span><p id="par0105" class="elsevierStylePara elsevierViewall">Since the first report of Iranian PID registration in 2002, more than 1600 PID cases were reported from 14 Iranian academic medical centres.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">1</span></a> An increasing number of experts in the field of clinical immunology, as well as increasing knowledge and awareness of physicians, has enhanced early diagnosis and management of this rare but significant group of diseases.<a class="elsevierStyleCrossRefs" href="#bib0220"><span class="elsevierStyleSup">4,9</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Consanguinity</span> – In this report, the total parental consanguinity rate was 57%. All patients diagnosed with phagocytic disorders and well defined syndromes had a positive history of parental consanguinity. This rate was 83% and 41% among patients diagnosed with CID and PAD, respectively. Consanguinity increases the risk of autosomal recessive diseases, including PID; the closer the relationship, the higher the risk.<a class="elsevierStyleCrossRef" href="#bib0250"><span class="elsevierStyleSup">10</span></a> In regions where consanguineous marriage is intrinsic to the culture, the frequency and type of genetic defects causing PID differ in comparison with those in other populations.<a class="elsevierStyleCrossRefs" href="#bib0255"><span class="elsevierStyleSup">11–14</span></a></p><p id="par0115" class="elsevierStylePara elsevierViewall">Clinical phenotypes and the severity of CVID have been shown to be related to parental consanguinity in some registries,<a class="elsevierStyleCrossRefs" href="#bib0275"><span class="elsevierStyleSup">15,16</span></a> but in our CVID cases, there was no relationship between CVID phenotypes or complications such as autoimmunity or malignancy and parental consanguinity.</p><p id="par0120" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Age at onset</span> – Most types of PID begin early in life. In our cohort, patients with CID, LAD1 and Kostmann disease were symptomatic in the first months of life, whereas, patients with PAD (except for CVID), ATS, DOCK8 deficiency, CMCC, and CGD were symptomatic in the first few years of life. This was similar to previous reports from Iran and other countries.<a class="elsevierStyleCrossRefs" href="#bib0205"><span class="elsevierStyleSup">1,4,17–22</span></a> In patients with CVID, the mean age at onset was 12.5 years, which falls in line with that of western countries in the second or third decades of life.<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">5</span></a> However, it should be underscored that the mean age at onset of patients with CVID was lower than three years in reports from other centres in Iran. This could be due to the high rate of parental consanguinity and complex inherited genetic basis in Iranian CVID patients, as we reported significantly lower age at onset in patients with a positive history of parental consanguinity than the others (7.7 versus 16.3 years). Moreover, our centre is located at a tertiary general hospital, unlike other centres in Iran that are mainly affiliated to children's hospitals, which could explain the higher age at onset of referral patients at our institution. We previously described that higher age of onset of CVID is significantly associated with the higher risk of malignancy and autoimmune disorders.<a class="elsevierStyleCrossRef" href="#bib0270"><span class="elsevierStyleSup">14</span></a></p><p id="par0125" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Age at diagnosis and diagnostic delay</span> – In our cohort of patients, the mean diagnostic delay was 6.1<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>7 years (1 month to 31 years), and those with less severe forms of PID, such as PAD and CMCC, had more prolonged diagnostic delay. Patients with CVID had a mean diagnostic delay of 8.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>7.9 years, while it was only 2<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>1.4 years in other PAD subtypes. The majority of patients with CID had a diagnostic delay of only a few months, except for two patients with CD4 deficiency and normal flow cytometry. Most patients with LAD1 and Kostmann disease were diagnosed in infancy. Patients with well-defined immunodeficiency syndromes were diagnosed in childhood with 4<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>2 years of diagnostic delay. The delayed diagnosis and treatment of PID is associated with an increased overall risk of disease-related complications.<a class="elsevierStyleCrossRefs" href="#bib0205"><span class="elsevierStyleSup">1,4,23</span></a></p><p id="par0130" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Infections</span> – In patients with PAD, sinopulmonary infections were the most common bacterial infections. Viral infections were diagnosed in 22% of CVID patients and the patient with LRBA deficiency. Fungal infections occurred in three of the CVID patients, as well as patients with LRBA deficiency and Good's syndrome. These infections can be due to associated abnormalities in T cell and innate immunity.<a class="elsevierStyleCrossRef" href="#bib0320"><span class="elsevierStyleSup">24</span></a> None of the XLA patients had history of severe viral or fungal infection.</p><p id="par0135" class="elsevierStylePara elsevierViewall">Patients with CID are prone to bacterial, fungal and viral infections.<a class="elsevierStyleCrossRefs" href="#bib0295"><span class="elsevierStyleSup">19,25,26</span></a> The most common infections in patients with CID were pneumonia, gastroenteritis, oral thrush. Severe viral and fungal infections were reported in 25% of patients. BCG vaccine is given once at birth in most developing countries, including Iran and disseminated BCG infection is a common feature in immune deficiency syndromes, including CID, CGD and defects in the IL-12/IFN-γ axis.<a class="elsevierStyleCrossRef" href="#bib0335"><span class="elsevierStyleSup">27</span></a> In our study, 25% of CID and 36% of CGD patients had disseminated BCG infection.</p><p id="par0140" class="elsevierStylePara elsevierViewall">Consistent with other reports, the most common infectious presentation in our cohort of CGD patients was sinopulmonary infections, followed by lymphadenitis, and skin and visceral abscess (<a class="elsevierStyleCrossRef" href="#tbl0020">Table 4</a>). Mycobacterium tuberculosis (pulmonary infection, lymphadenitis) was diagnosed in two patients. Mycobacterium tuberculosis can be the first manifestation of CGD in endemic regions.<a class="elsevierStyleCrossRef" href="#bib0340"><span class="elsevierStyleSup">28</span></a> All four patients with LAD1 initially presented with omphalitis and delayed umbilical separation in the first weeks of life. Other infections included cellulitis, peritonitis, chronic diarrhoea and septic arthritis, and were similar to previous reports.<a class="elsevierStyleCrossRef" href="#bib0290"><span class="elsevierStyleSup">18</span></a> Severe congenital neutropenia is clinically characterised by omphalitis, pneumonia and gingivitis caused by resident bacteria of the skin, mouth, and oropharynx.<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">21</span></a> Similarly, the patient reported herein presented with neonatal sepsis, respiratory tract and mucocutaneous infections.</p><p id="par0145" class="elsevierStylePara elsevierViewall">CMCC is characterised with chronic non-invasive Candida infections of the skin, nails, and mucous membranes, in association with a variety of clinical features, including increased susceptibility to bacterial and viral infections recommended.<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">26</span></a> We reported recurrent sinopulmonary infections, perianal abscess, urinary tract infection, and gingivitis in patients diagnosed with CMCC.</p><p id="par0150" class="elsevierStylePara elsevierViewall">In patients with ATS, immune deficiency is quite variable but often manifests as recurrent sinopulmonary infections. Infections outside of the respiratory tract are generally not increased in frequency, and opportunistic infections rarely occur. Pulmonary infections may be the result of underlying immunodeficiency and aspiration.<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">22,29</span></a> Similarly, both patients with ATS had history of recurrent pneumonia and gastroenteritis. Patients with DOCK8 deficiency develop recurrent upper and lower respiratory tract infections, extensive cutaneous viral and bacterial infections, mucosal or nail candidiasis and otitis externa.<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">30</span></a> The reported DOCK8 deficient patients reported herein had recurrent sinopulmonary infections and BCG adenitis, with no history of severe viral infections.</p><p id="par0155" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Autoimmunity</span> – Autoimmunity and abnormal inflammation has often been observed in association with different forms of PID.<a class="elsevierStyleCrossRef" href="#bib0215"><span class="elsevierStyleSup">3</span></a> Autoimmune disorders may be the first manifestation of the disease in CVID patients.<a class="elsevierStyleCrossRefs" href="#bib0225"><span class="elsevierStyleSup">5,6</span></a> In our study 25 (46%) CVID patients had autoimmune disorders and ITP was the most common diagnosis. Autoimmune disorders were the first signs of immunodeficiency in eight (15%) patients. Twelve (22%) patients had multiple autoimmune disorders. Patients with Good's syndrome and LRBA deficiency were diagnosed with multiple autoimmune disorders, and one out of six patients with XLA had history of juvenile rheumatoid arthritis. Three (25%) patients with CID developed ITP, vasculitis and eczema. Severe, chronic eczema was the main autoimmune feature of the two DOCK8 deficient patients. Patients with CMCC developed multiple autoimmune disorders including endocrinopathies, autoimmune hepatitis, ITP, AHA, alopecia and vitiligo. Two (18%) patients with CGD were diagnosed with psoriasis and eczema.</p><p id="par0160" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Treatment protocols</span> – Lack of or inadequate medical insurance coverage for the treatment of PID manifestations and complications in Iran has limited the extent of therapeutic interventions to symptomatic and supportive treatments.<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">1</span></a> The cohort of 63 PAD patients in this study was treated with IVIG replacement at least monthly, in addition to treatment of any active infections and/or autoimmune disorders as was recommended.<a class="elsevierStyleCrossRefs" href="#bib0225"><span class="elsevierStyleSup">5,26,31</span></a> The goal of therapy was to maintain IgG trough levels (drawn just before the next infusion) of at least 500<span class="elsevierStyleHsp" style=""></span>mg/dL up to the end of 2012, which was later increased to 600–700<span class="elsevierStyleHsp" style=""></span>mg/dL.</p><p id="par0165" class="elsevierStylePara elsevierViewall">The treatment of patients with CID includes HSCT and gene therapy and earlier detection of the disease and treatment in few months after birth significantly improves the outcomes.<a class="elsevierStyleCrossRefs" href="#bib0325"><span class="elsevierStyleSup">25,26,31</span></a> In this study, only one patient (T<span class="elsevierStyleSup">−</span>B<span class="elsevierStyleSup">+</span>) underwent a bone marrow transplant, who showed no evidence of long-term complications at the time of study. The remaining three patients were treated with antimicrobials and IVIG while waiting to receive HSCT.</p><p id="par0170" class="elsevierStylePara elsevierViewall">The two patients with ATS reported herein were treated with IVIG replacement therapy and antibiotics as recommended.<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">22,26,29,31</span></a> Treatment of DOCK8 deficient patients are mainly supportive, and HSCT remains the only definitive option.<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">30</span></a> Antibiotic prophylaxis, skin care and prompt treatment of skin infections were supportive treatment in these patients and the living case is waiting to receive HSCT.</p><p id="par0175" class="elsevierStylePara elsevierViewall">Management of patients with CMCC included antifungal therapy and treatment of associated endocrinopathies and autoimmune abnormalities, as well as antibiotic therapy as recommended.<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">26</span></a></p><p id="par0180" class="elsevierStylePara elsevierViewall">The cornerstones of management of CGD are prophylaxis with antibacterial, antifungal and IFN-γ and attempts to early diagnosis and aggressive treatment of infectious complications. However, successful HSCT can be a definitive cure for CGD.<a class="elsevierStyleCrossRefs" href="#bib0330"><span class="elsevierStyleSup">26,31,32</span></a> In our study, costs of antifungal prophylaxis resulted in poor patient compliance. No patients received IFN-γ and HSCT during the follow-up period because of unavailability. Mild to moderate LAD1 usually responds to antibiotic therapy, while HSCT is recommended in severe cases (CD11a CD18<span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>1%).<a class="elsevierStyleCrossRefs" href="#bib0330"><span class="elsevierStyleSup">26,31</span></a> All four patients with LAD1 were treated for acute infections and received prophylactic antibiotics. The two patients with the severe form of the disease were potential candidates for HSCT. The patient with Kostmann disease was treated with G-CSF, as well as antibiotics for prophylaxis and treated for acute infections. In severe cases of congenital neutropenia, who are unresponsive to G-CSF or complicated with lymphoproliferative disorders, HSCT is recommended.<a class="elsevierStyleCrossRefs" href="#bib0305"><span class="elsevierStyleSup">21,26,31</span></a></p><p id="par0185" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">Complications and mortality</span> – Bronchiectasis was the most common complication among patients with PAD (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>). Other common complications in patients with CVID were splenomegaly (32%) and malignancy (11%). Malignancies are reported in 2.5–8% of CVID patients, with non-Hodgkin's lymphoma being the most common reported malignancy.<a class="elsevierStyleCrossRefs" href="#bib0225"><span class="elsevierStyleSup">5,33</span></a> During the mean follow-up period of 8.5 years, 11% of CVID patients died, compared to a mortality rate of 35% during a mean follow-up period of 6.5 years in an Iranian study performed in 2007.<a class="elsevierStyleCrossRef" href="#bib0370"><span class="elsevierStyleSup">34</span></a> The decrease in the mortality rate could be due to earlier diagnosis and improved availability of IVIG and supportive care. Similar to previous reports,<a class="elsevierStyleCrossRef" href="#bib0375"><span class="elsevierStyleSup">35</span></a> patients with XLA had lower morbidity and mortality compared to patients with CVID: six patients with XLA were followed up for a mean period of 19.4 years with no severe complications. One of the patients had XLA and growth hormone deficiency. This was first described by Fleisher et al. in 1980 and since that time other patients have been reported with this syndrome<a class="elsevierStyleCrossRefs" href="#bib0380"><span class="elsevierStyleSup">36,37</span></a> and clinicians should follow the stature growth in their XLA patients. The patient with Good's syndrome died of acute graft-versus-host disease syndrome following the transfusion of irradiated blood products for symptomatic treatment of aplastic anaemia. This emphasizes the importance of awareness of physicians about the risk of GVHD in cellular immunodeficiency conditions such as Good's syndrome.<a class="elsevierStyleCrossRef" href="#bib0390"><span class="elsevierStyleSup">38</span></a> We reported a case of LRBA deficiency, a rare autosomal recessive CVID-like syndrome,<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">39</span></a> which was complicated with bronchiolitis obliterans with organising pneumonia.</p><p id="par0190" class="elsevierStylePara elsevierViewall">The only patient reported with HIGM syndrome herein, was followed for 14 years and was 20 years old at the time of study, with no history of severe infection or disease related complication.</p><p id="par0195" class="elsevierStylePara elsevierViewall">Patients diagnosed with CID often die in infancy or the first few years of life, despite supportive therapy, unless treated with HSCT from an appropriate donor.<a class="elsevierStyleCrossRefs" href="#bib0325"><span class="elsevierStyleSup">25,26</span></a> In our cohort of CID patients, except for one who underwent HSCT, only three patients with milder phenotype survived for years.</p><p id="par0200" class="elsevierStylePara elsevierViewall">The major complications of patients with ATS are progressive neurologic deterioration, respiratory syndrome, chronic lung diseases and malignancies,<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">22,29</span></a> which rarely allow survival beyond the second or third decade of life. In our study, one patient died at 10 years of age due to poor family support and severe lung disease. The second patient was diagnosed with and treated for lymphoma at the time of study. DOCK8 deficiency has a relatively poor prognosis due to life threatening infections, malignancy or cerebral complications such as CNS vasculitis or stroke.<a class="elsevierStyleCrossRef" href="#bib0350"><span class="elsevierStyleSup">30</span></a> One of the patients with DOCK8 deficiency died of an undiagnosed brain mass at 11 years of age.</p><p id="par0205" class="elsevierStylePara elsevierViewall">One patient with heterozygous gain-of-function mutation in STAT1 developed chronic lung disease and pulmonary hypertension because of recurrent pneumonia. Severe phenotypes in these patients were reported previously.<a class="elsevierStyleCrossRef" href="#bib0400"><span class="elsevierStyleSup">40</span></a></p><p id="par0210" class="elsevierStylePara elsevierViewall">In our cohort of CGD patients, the majority developed chronic lung disease with severe bronchiectasis, cavity formation, pulmonary hypertension and clubbing. These morbidities were previously reported in patients with CGD<a class="elsevierStyleCrossRef" href="#bib0360"><span class="elsevierStyleSup">32</span></a>; however, unaffordable medicine prices and poor availability made these severe complications much more common among our patients. Without successful HSCT, infections are the major cause of death in patients with severe LAD1.<a class="elsevierStyleCrossRefs" href="#bib0290"><span class="elsevierStyleSup">18,26</span></a> In our study, one of the patients with moderate LAD1 died of lymphoma at 11 years of age. Two infants with undetectable CD18 levels are waiting for HSCT. The only patient with Kostmann disease was 12 years old and in good condition at the time of study with no severe complication. Patients with a good response to G-CSF have acceptable prognosis; however, they should be observed for infections and lymphoproliferative disorders.<a class="elsevierStyleCrossRef" href="#bib0305"><span class="elsevierStyleSup">21</span></a></p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Conclusions</span><p id="par0215" class="elsevierStylePara elsevierViewall">Physicians’ awareness of PID has been rising dramatically in certain regions of the world, ensuring an increasing number of patients being diagnosed and treated. However, a large number of patients do not have access to effective treatment services and suffer from lack of health insurance and weak or absent social supports.</p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Ethical disclosures</span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Protection of human and animal subjects</span><p id="par0220" class="elsevierStylePara elsevierViewall">The authors declare that no experiments were performed on humans or animals for this investigation.</p></span><span id="sec0060" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Confidentiality of data</span><p id="par0225" class="elsevierStylePara elsevierViewall">The authors declare that they have followed the protocols of their work centre on the publication of patient data and that all the patients included in the study have received sufficient information and have given their informed consent in writing to participate in this study.</p></span><span id="sec0065" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Right to privacy and informed consent</span><p id="par0230" class="elsevierStylePara elsevierViewall">The authors have obtained the informed consent of the patients mentioned in the article. The author for correspondence is in possession of this document.</p></span></span><span id="sec0070" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Conflict of interest</span><p id="par0235" class="elsevierStylePara elsevierViewall">The authors have no conflict of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres688850" "titulo" => "Abstract" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Purpose" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Method" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec694637" "titulo" => "Keywords" ] 2 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 3 => array:3 [ "identificador" => "sec0010" "titulo" => "Method" "secciones" => array:1 [ 0 => array:2 [ "identificador" => "sec0015" "titulo" => "Statistical analyses" ] ] ] 4 => array:3 [ "identificador" => "sec0020" "titulo" => "Results" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0025" "titulo" => "Predominantly Antibody Deficiencies" ] 1 => array:2 [ "identificador" => "sec0030" "titulo" => "Combined T-cell and B-cell immunodeficiencies" ] 2 => array:2 [ "identificador" => "sec0035" "titulo" => "Chronic Mucocutaneous Candidiasis" ] ] ] 5 => array:2 [ "identificador" => "sec0040" "titulo" => "Discussion" ] 6 => array:2 [ "identificador" => "sec0045" "titulo" => "Conclusions" ] 7 => array:3 [ "identificador" => "sec0050" "titulo" => "Ethical disclosures" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0055" "titulo" => "Protection of human and animal subjects" ] 1 => array:2 [ "identificador" => "sec0060" "titulo" => "Confidentiality of data" ] 2 => array:2 [ "identificador" => "sec0065" "titulo" => "Right to privacy and informed consent" ] ] ] 8 => array:2 [ "identificador" => "sec0070" "titulo" => "Conflict of interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2015-05-04" "fechaAceptado" => "2015-09-30" "PalabrasClave" => array:1 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec694637" "palabras" => array:7 [ 0 => "Consanguinity" 1 => "Complications" 2 => "Follow-up" 3 => "Iran" 4 => "Manifestations" 5 => "Mortality" 6 => "Primary immune deficiency" ] ] ] ] "tieneResumen" => true "resumen" => array:1 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Purpose</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">The aim was to describe the clinical manifestations, complications and long-term outcome of a cohort of Iranian patients with primary immune deficiency (PID).</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Method</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We retrospectively studied the demographic, clinical and immunological characteristics of the PID patients in a single tertiary centre, from January 1989 to July 2014. The patients were classified according to the International Union of Immunological Societies Expert Committee on PID.</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">98 patients were diagnosed with and followed-up for 15 disorders. The mean age at onset and diagnosis and the diagnostic delay were 8<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>10, 14.2<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>13.1 and 6.1<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>7 years, respectively. Parental consanguinity rate was 57%. Predominantly Antibody Deficiency was the most common diagnosis (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>63), followed by congenital defects of phagocytes (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>16), combined immunodeficiencies (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>12), well defined syndromes (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>4) and defects in innate immunity (<span class="elsevierStyleItalic">n</span><span class="elsevierStyleHsp" style=""></span>=<span class="elsevierStyleHsp" style=""></span>3). Recurrent sinopulmonary infection was the most common presentation. Active infections were treated appropriately, in addition to prophylactic therapy with IVIG and antimicrobials. Not all the patients were compliant with prophylactic regimens due to cost and unavailability. One SCID patient underwent successful bone marrow transplantation. The total mortality rate was 19% during the follow-up period (7.8<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>7.6 years). The mean age of living patients at the time of study was 23<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>11.7 years.</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conclusions</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Physicians awareness of PID has been rising dramatically in Iran, ensuring an increasing number of patients being diagnosed and treated. More effective treatment services, including health insurance coverage and drug availability are needed to improve the outcome of PID patients.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Purpose" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Method" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Conclusions" ] ] ] ] "multimedia" => array:5 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1361 "Ancho" => 1597 "Tamanyo" => 62400 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Number of newly diagnosed patients with primary immune deficiency at Rasool-E-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran between 1990 and 2014.</p>" ] ] 1 => array:7 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "tabla" => array:3 [ "leyenda" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleItalic">Note</span>. CVID: Common Variable Immune Deficiency; XLA: X Linked Agammaglobulinaemia; HIGM: Hyper-IgM; LRBA: LPS responsive beige-like anchor; SCID: severe combined immunodeficiency; ATS: Ataxia-Telangiectasia syndrome; DOCK8: Dedicator of Cytokinesis 8; CMCC: Chronic Mucocutaneous Candidiasis; CGD: chronic granulomatous disorder; LAD: leucocyte adhesion molecule deficiency; y.: years; m.: months; d.: days.</p>" "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="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Classification \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Subtype (<span class="elsevierStyleItalic">n</span>) \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Male (%) \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Age at onset (±SD) \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Age at diagnosis (±SD) \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Age at the time of study (±SD) \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Follow-up period \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Consanguinity (%)<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Mortality (%) \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry " rowspan="5" align="left" valign="middle">Predominantly Antibody Deficiency</td><td class="td" title="table-entry " align="left" valign="top">CVID (54) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">12.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>10.5<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">21<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>12<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">27.7<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>9.9<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7.5<span class="elsevierStyleHsp" style=""></span>y \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">44.5 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">11 \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">XLA (6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1.3<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>0.6<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">3.1<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>1.8<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">22.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>8.6<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">19.4<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">17 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \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">HIGM syndrome (1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">5<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">6<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">20<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">14<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \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">Good's syndrome (1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">45<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">48<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \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">LRBA deficiency (1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">6<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7.5<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1.5<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="5" align="left" valign="middle">Combined T-cell and B-cell Immunodeficiencies</td><td class="td" title="table-entry " align="left" valign="top">T− B+ SCID (6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">66 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1.8<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>1.3<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>8.5<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">45<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>55<span class="elsevierStyleHsp" style=""></span>m.<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">b</span></a> \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">26<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">66 \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">T− B− SCID(2) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">3.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>2.1<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">6<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>1.4<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">3<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \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">CD8 deficiency (2) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">3.8<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>4.7<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">5.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>4.9<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">11<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">42<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \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">CD4 deficiency (1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">11<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">60<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">12<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \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">Normal flow cytometry (1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">6<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">72<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">78<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">6<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="2" align="left" valign="middle">Other Well Defined Immunodeficiency Syndromes</td><td class="td" title="table-entry " align="left" valign="top">ATS (2) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>0.7<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>0.7<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \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">DOCK8 deficiency (2) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">21<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>21.2<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>4.2<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">2<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \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">Defects in Innate Immunity \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">CMCC (3) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">33 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">10.6<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>11.7<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8.5<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>1.2<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">20<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">11.5<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="3" align="left" valign="middle">Congenital Defects of Phagocyte Number and/or Function</td><td class="td" title="table-entry " align="left" valign="top">CGD (11) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">82 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">3.6<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>4.9<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8.9<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>9.2<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">21.2<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>9.9<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">11<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">18 \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">LAD1 (4) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">50 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">14.7<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>6.4<span class="elsevierStyleHsp" style=""></span>d. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">10.4<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>17<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">44<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>64<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">56<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">25 \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">Kostmann disease (1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">1<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">9<span class="elsevierStyleHsp" style=""></span>m. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">11<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">10<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">100 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">0 \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="9" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Total \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">98 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">59 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">8<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">14.2<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>13.1<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">23<span class="elsevierStyleHsp" style=""></span>±<span class="elsevierStyleHsp" style=""></span>11.7<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">7.6<span class="elsevierStyleHsp" style=""></span>y. \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">57 \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">19 \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1120648.png" ] ] ] "notaPie" => array:2 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Consanguinity: parents are first or second degree cousins.</p>" ] 1 => array:3 [ "identificador" => "tblfn0010" "etiqueta" => "b" "nota" => "<p class="elsevierStyleNotepara" id="npar0010">Two patients were alive; one of them had undergone bone marrow transplantation and was 7 years old at the time of study.</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Demographic characteristics and outcomes of ninety eight Iranian patients with primary immune deficiency.</p>" ] ] 2 => array:7 [ "identificador" => "tbl0010" "etiqueta" => "Table 2" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "tabla" => array:2 [ "leyenda" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleItalic">Note</span>. CVID: Common Variable Immune Deficiency; XLA: X Linked Agammaglobulinaemia; HIGM: Hyper-IgM; LRBA: LPS responsive beige-like anchor; CNS: central nervous system; UTI: urinary tract infection; ITP: idiopathic thrombocytopenic purpura; AHA: autoimmune haemolytic anaemia; IBD: inflammatory bowel disease; JRA: juvenile rheumatoid arthritis; GH: growth hormone; GVHD: graft versus host disease; FTT: failure to thrive; BOOP: bronchiolitis obliterans organising pneumonia.</p>" "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="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Infections (%) \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Auto-inflammatory diseases (%) \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Complications (%) \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry " align="center" valign="middle">CVID \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Sinopulmonary (98%), gastrointestinal (30%), osteomyelitis/arthritis (20%), skin (18%), CNS (11%), visceral abscess (5.5%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ITP (25%), AHA (16%), thyroiditis (15%), IBD (15%), rheumatologic (24%), vitiligo (9%), pernicious anaemia (4%), alopecia (2%), hepatitis (2%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Bronchiectasis (67%), splenomegaly (32%), malignancy (11%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="center" valign="middle">XLA \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Sinopulmonary (100%), gastrointestinal (50%), osteomyelitis/arthritis (50%), CNS (17%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">JRA (17%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Bronchiectasis (50%); GH deficiency (17%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="center" valign="middle">HIGM syndrome \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Sinopulmonary, BCG adenitis, gastrointestinal, UTI \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="center" valign="middle">Good's syndrome \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Oral mucositis, gingivitis, urogenital, skin \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Alopecia, myasthenia gravis, recurrent oral and genital aphthous ulcers \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">GVHD, aplastic anaemia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " align="center" valign="middle">LRBA deficiency \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Sinopulmonary, gastrointestinal, CNS \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">ITP, AHA \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Bronchiectasis, splenomegaly, hepatomegaly, FTT, BOOP \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1120651.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Clinical manifestations and complications of 63 Iranian patients with predominantly antibody deficiency.</p>" ] ] 3 => array:7 [ "identificador" => "tbl0015" "etiqueta" => "Table 3" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "tabla" => array:2 [ "leyenda" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">CID: combined immunodeficiency disorders; SCID: severe combined immunodeficiency disorders; FTT: failure to thrive; BCG: bacillus calmette-guerin.</p>" "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="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">CID type (number of patients) \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Sinopulmonary infections \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Gastroenteritis \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Viral infection \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">FTT \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Disseminated BCG \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Autoimmunity \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Oral thrush \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">T− B+ SCID (6) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">6 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">4 (66%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">3 (50%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">5 (83%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (33%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (16%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">4 (66%) \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">T− B− SCID (2) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (50%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (50%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \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">CD4 deficiency (1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (100%) \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">CD8 low (2) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (50%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (50%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (100%) \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">Normal flow cytometry (1) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">– \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (100%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " colspan="8" align="left" valign="top"><span class="elsevierStyleVsp" style="height:0.5px"></span></td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Total (12) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">12 (100%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">9 (75%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">4 (33%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">9 (75%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">3 (25%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">3 (25%) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">8 (66%) \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1120650.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Common clinical manifestations in twelve Iranian combined immunodeficiency disorders.</p>" ] ] 4 => array:7 [ "identificador" => "tbl0020" "etiqueta" => "Table 4" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "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="table-head " align="" valign="top" scope="col" style="border-bottom: 2px solid black"> \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Manifestations \t\t\t\t\t\t\n \t\t\t\t</th><th class="td" title="table-head " align="left" valign="top" scope="col" style="border-bottom: 2px solid black">Number (%) \t\t\t\t\t\t\n \t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry " rowspan="9" align="center" valign="middle">Infections</td><td class="td" title="table-entry " align="left" valign="top">Sinopulmonary infections \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">10 (91%) \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">Skin abscess \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">8 (73%) \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">Lymphadenitis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">7 (64%) \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">Visceral abscess \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">5 (45%) \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">Disseminated BCG \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">4 (36%) \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">Chronic/recurrent diarrhoea (inflammatory bowel disorder excluded) \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">3 (27%) \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">Oral thrush \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">3 (27%) \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">Mycobacterium tuberculosis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (18%) \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">Osteomyelitis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (18%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="3" align="center" valign="middle">Auto-inflammatory</td><td class="td" title="table-entry " align="left" valign="top">Hepatosplenomegaly \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">2 (18%) \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">Psoriasis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (9%) \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">Eczema \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">1 (9%) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="5" align="center" valign="middle">Complications</td><td class="td" title="table-entry " align="left" valign="top">Bronchiectasis \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">9 (82%) \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">Pulmonary cavity \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">8 (73%) \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">Clubbing \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">7 (64%) \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">Pulmonary hypertension \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">7 (64%) \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">Failure to thrive \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="char" valign="top">4 (36%) \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab1120649.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Clinical manifestations and complications of eleven Iranian patients with chronic granulomatous disease.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:40 [ 0 => array:3 [ "identificador" => "bib0205" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Aghamohammadi" 1 => "P. Mohammadinejad" 2 => "H. Abolhassani" 3 => "B. Mirminachi" 4 => "M. Movahedi" 5 => "M. Gharagozlou" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10875-014-0001-z" "Revista" => array:6 [ "tituloSerie" => "J Clin Immunol" "fecha" => "2014" "volumen" => "34" "paginaInicial" => "478" "paginaFinal" => "490" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24659230" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0210" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Population prevalence of diagnosed primary immunodeficiency diseases in the United States" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J. Boyle" 1 => "R. Buckley" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10875-007-9103-1" "Revista" => array:6 [ "tituloSerie" => "J Clin Immunol" "fecha" => "2007" "volumen" => "27" "paginaInicial" => "497" "paginaFinal" => "502" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17577648" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0215" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Autoimmune disease in primary antibody deficiencies" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "E. Sarmiento" 1 => "R. Mora" 2 => "M. Rodríguez-Mahou" 3 => "J. Rodríguez-Molina" 4 => "E. Fernández-Cruz" 5 => "J. Carbone" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Allergol Immunopathol (Madr)" "fecha" => "2004" "volumen" => "33" "paginaInicial" => "69" "paginaFinal" => "73" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0220" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The prevalences and patient characteristics of primary immunodeficiency diseases in Turkey—two centers study" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "S.S. Kilic" 1 => "M. Ozel" 2 => "D. Hafizoglu" 3 => "N.E. Karaca" 4 => "G. Aksu" 5 => "N. Kutukculer" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10875-012-9763-3" "Revista" => array:6 [ "tituloSerie" => "J Clin Immunol" "fecha" => "2013" "volumen" => "33" "paginaInicial" => "74" "paginaFinal" => "83" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22983506" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0225" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "I. Quinti" 1 => "A. Soresina" 2 => "G. Spadaro" 3 => "S. Martino" 4 => "S. Donnanno" 5 => "C. Agostini" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10875-007-9075-1" "Revista" => array:6 [ "tituloSerie" => "J Clin Immunol" "fecha" => "2007" "volumen" => "27" "paginaInicial" => "308" "paginaFinal" => "316" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17510807" "web" => "Medline" ] ] ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0230" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Common variable immunodeficiency: crossroads between infections, inflammation and autoimmunity" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "S. Baldovino" 1 => "D. Montin" 2 => "S. Martino" 3 => "S. Sciascia" 4 => "E. Menegatti" 5 => "D. Roccatello" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Autoimmunity Rev" "fecha" => "2013" "volumen" => "12" "paginaInicial" => "796" "paginaFinal" => "801" ] ] ] ] ] ] 6 => array:3 [ "identificador" => "bib0235" "etiqueta" => "7" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "W. Al-Herz" 1 => "A. Bousfiha" 2 => "J.-L. Casanova" 3 => "T. Chatila" 4 => "M.E. Conley" 5 => "C. Cunningham-Rundles" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:3 [ "tituloSerie" => "Front Immunol" "fecha" => "2014" "paginaInicial" => "5" ] ] ] ] ] ] 7 => array:3 [ "identificador" => "bib0240" "etiqueta" => "8" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Diagnostic criteria for primary immunodeficiencies" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "M.E. Conley" 1 => "L.D. Notarangelo" 2 => "A. Etzioni" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1006/clim.1999.4799" "Revista" => array:6 [ "tituloSerie" => "Clin Immunol" "fecha" => "1999" "volumen" => "93" "paginaInicial" => "190" "paginaFinal" => "197" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/10600329" "web" => "Medline" ] ] ] ] ] ] ] ] 8 => array:3 [ "identificador" => "bib0245" "etiqueta" => "9" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Physicians awareness on primary immunodeficiency disorders in Iran" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "K. Nourijelyani" 1 => "A. Aghamohammadi" 2 => "M.S. Sadaghiani" 3 => "N. Behniafard" 4 => "H. Abolhassani" 5 => "S. Pourjabar" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "011.01/ijaai.5764" "Revista" => array:6 [ "tituloSerie" => "Iran J Allergy Asthma Immunol" "fecha" => "2012" "volumen" => "11" "paginaInicial" => "57" "paginaFinal" => "64" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22427477" "web" => "Medline" ] ] ] ] ] ] ] ] 9 => array:3 [ "identificador" => "bib0250" "etiqueta" => "10" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Parental consanguinity and the risk of primary immunodeficiency disorders: report from the Kuwait National Primary Immunodeficiency Disorders Registry" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "W. Al-Herz" 1 => "K.K. Naguib" 2 => "L.D. Notarangelo" 3 => "R.S. Geha" 4 => "A. Alwadaani" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1159/000319212" "Revista" => array:6 [ "tituloSerie" => "Int Arch Allergy Immunol" "fecha" => "2010" "volumen" => "154" "paginaInicial" => "76" "paginaFinal" => "80" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20664281" "web" => "Medline" ] ] ] ] ] ] ] ] 10 => array:3 [ "identificador" => "bib0255" "etiqueta" => "11" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Consanguinity in primary immunodeficiency disorders; the report from Iranian Primary Immunodeficiency Registry" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "N. Rezaei" 1 => "Z. Pourpak" 2 => "A. Aghamohammadi" 3 => "A. Farhoudi" 4 => "M. Movahedi" 5 => "M. Gharagozlou" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1600-0897.2006.00409.x" "Revista" => array:6 [ "tituloSerie" => "Am J Reprod Immunol" "fecha" => "2006" "volumen" => "56" "paginaInicial" => "145" "paginaFinal" => "151" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16836617" "web" => "Medline" ] ] ] ] ] ] ] ] 11 => array:3 [ "identificador" => "bib0260" "etiqueta" => "12" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Effects of family history and consanguinity in primary immunodeficiency diseases in children in Qatar" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "M. Ehlayel" 1 => "A. Bener" 2 => "M.A. Laban" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "Open J Immunol" "fecha" => "2013" "volumen" => "3" "paginaInicial" => "47" ] ] ] ] ] ] 12 => array:3 [ "identificador" => "bib0265" "etiqueta" => "13" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Parental consanguinity is associated with a severe phenotype in common variable immunodeficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "C. Rivoisy" 1 => "L. Gérard" 2 => "D. Boutboul" 3 => "M. Malphettes" 4 => "C. Fieschi" 5 => "I. Durieu" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10875-011-9604-9" "Revista" => array:6 [ "tituloSerie" => "J Clin Immunol" "fecha" => "2012" "volumen" => "32" "paginaInicial" => "98" "paginaFinal" => "105" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22002594" "web" => "Medline" ] ] ] ] ] ] ] ] 13 => array:3 [ "identificador" => "bib0270" "etiqueta" => "14" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Phenotyping and follow up of forty-seven Iranian patients with common variable immunodeficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "S. Arshi" 1 => "M. Nabavi" 2 => "M.H. Bemanian" 3 => "R. Shakeri" 4 => "B. Taghvaei" 5 => "B. Ghalebaghi" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:2 [ "tituloSerie" => "Allergol Immunopathol (Madr)" "fecha" => "2015" ] ] ] ] ] ] 14 => array:3 [ "identificador" => "bib0275" "etiqueta" => "15" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Correlation between common variable immunodeficiency clinical phenotypes and parental consanguinity in children and adults" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "A. Aghamohammadi" 1 => "H. Abolhassani" 2 => "K. Moazzami" 3 => "N. Parvaneh" 4 => "N. Rezaei" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "J Investig Allergol Clin Immunol" "fecha" => "2010" "volumen" => "20" "paginaInicial" => "372" "paginaFinal" => "379" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20945602" "web" => "Medline" ] ] ] ] ] ] ] ] 15 => array:3 [ "identificador" => "bib0280" "etiqueta" => "16" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Parental consanguinity is associated with a severe phenotype in common variable immunodeficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "C. Rivoisy" 1 => "L. Gerard" 2 => "D. Boutboul" 3 => "M. Malphettes" 4 => "C. Fieschi" 5 => "I. Durieu" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10875-011-9604-9" "Revista" => array:6 [ "tituloSerie" => "J Clin Immunol" "fecha" => "2012" "volumen" => "32" "paginaInicial" => "98" "paginaFinal" => "105" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22002594" "web" => "Medline" ] ] ] ] ] ] ] ] 16 => array:3 [ "identificador" => "bib0285" "etiqueta" => "17" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Chronic granulomatous disease: overview and hematopoietic stem cell transplantation" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "E.M. Kang" 1 => "B.E. Marciano" 2 => "S. DeRavin" 3 => "K.A. Zarember" 4 => "S.M. Holland" 5 => "H.L. Malech" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jaci.2011.03.028" "Revista" => array:6 [ "tituloSerie" => "J Allergy Clin Immunol" "fecha" => "2011" "volumen" => "127" "paginaInicial" => "1319" "paginaFinal" => "1326" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21497887" "web" => "Medline" ] ] ] ] ] ] ] ] 17 => array:3 [ "identificador" => "bib0290" "etiqueta" => "18" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical and laboratory findings in Iranian patients with leukocyte adhesion deficiency (study of 15 cases)" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Movahedi" 1 => "N. Entezari" 2 => "Z. Pourpak" 3 => "S. Mamishi" 4 => "Z. Chavoshzadeh" 5 => "M. Gharagozlou" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s10875-006-9069-4" "Revista" => array:6 [ "tituloSerie" => "J Clin Immunol" "fecha" => "2007" "volumen" => "27" "paginaInicial" => "302" "paginaFinal" => "307" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17294145" "web" => "Medline" ] ] ] ] ] ] ] ] 18 => array:3 [ "identificador" => "bib0295" "etiqueta" => "19" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Severe combined immunodeficiency: a cohort of 40 patients" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Yeganeh" 1 => "M. Heidarzade" 2 => "Z. Pourpak" 3 => "N. Parvaneh" 4 => "N. Rezaei" 5 => "M. Gharagozlou" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1399-3038.2007.00647.x" "Revista" => array:6 [ "tituloSerie" => "Pediatr Allergy Immunol" "fecha" => "2008" "volumen" => "19" "paginaInicial" => "303" "paginaFinal" => "306" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/18093084" "web" => "Medline" ] ] ] ] ] ] ] ] 19 => array:3 [ "identificador" => "bib0300" "etiqueta" => "20" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: an Italian multicenter study" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Plebani" 1 => "A. Soresina" 2 => "R. Rondelli" 3 => "G.M. Amato" 4 => "C. Azzari" 5 => "F. Cardinale" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Clin Immunol" "fecha" => "2002" "volumen" => "104" "paginaInicial" => "221" "paginaFinal" => "230" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12217331" "web" => "Medline" ] ] ] ] ] ] ] ] 20 => array:3 [ "identificador" => "bib0305" "etiqueta" => "21" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pathophysiology and management of inherited bone marrow failure syndromes" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "A. Shimamura" 1 => "B.P. Alter" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.blre.2010.03.002" "Revista" => array:6 [ "tituloSerie" => "Blood Rev" "fecha" => "2010" "volumen" => "24" "paginaInicial" => "101" "paginaFinal" => "122" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20417588" "web" => "Medline" ] ] ] ] ] ] ] ] 21 => array:3 [ "identificador" => "bib0310" "etiqueta" => "22" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Ataxia-telangiectasia in Iran: clinical and laboratory features of 104 patients" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M. Moin" 1 => "A. Aghamohammadi" 2 => "A. Kouhi" 3 => "S. Tavassoli" 4 => "N. Rezaei" 5 => "S.-R. Ghaffari" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.pediatrneurol.2007.03.002" "Revista" => array:6 [ "tituloSerie" => "Pediatr Neurol" "fecha" => "2007" "volumen" => "37" "paginaInicial" => "21" "paginaFinal" => "28" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17628218" "web" => "Medline" ] ] ] ] ] ] ] ] 22 => array:3 [ "identificador" => "bib0315" "etiqueta" => "23" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Infectious and non-infectious complications among undiagnosed patients with common variable immunodeficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Aghamohammadi" 1 => "M. Tavassoli" 2 => "H. Abolhassani" 3 => "N. Parvaneh" 4 => "K. Moazzami" 5 => "A. Allahverdi" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Iran J Pediatrics" "fecha" => "2009" "volumen" => "19" "paginaInicial" => "367" "paginaFinal" => "375" ] ] ] ] ] ] 23 => array:3 [ "identificador" => "bib0320" "etiqueta" => "24" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Primary immunodeficiency diseases associated with increased susceptibility to viral infections and malignancies" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "N. Rezaei" 1 => "M. Hedayat" 2 => "A. Aghamohammadi" 3 => "K.E. Nichols" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jaci.2011.01.057" "Revista" => array:4 [ "tituloSerie" => "J Allergy Clin Immunol" "fecha" => "2011" "volumen" => "127" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21377717" "web" => "Medline" ] ] ] ] ] ] ] ] 24 => array:3 [ "identificador" => "bib0325" "etiqueta" => "25" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Early vs. delayed diagnosis of severe combined immunodeficiency: a family perspective survey" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "A. Chan" 1 => "C. Scalchunes" 2 => "M. Boyle" 3 => "J.M. Puck" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.clim.2010.09.010" "Revista" => array:6 [ "tituloSerie" => "Clin Immunol" "fecha" => "2011" "volumen" => "138" "paginaInicial" => "3" "paginaFinal" => "8" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21035402" "web" => "Medline" ] ] ] ] ] ] ] ] 25 => array:3 [ "identificador" => "bib0330" "etiqueta" => "26" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Practice parameter for the diagnosis and management of primary immunodeficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "F.A. Bonilla" 1 => "I.L. Bernstein" 2 => "D.A. Khan" 3 => "Z.K. Ballas" 4 => "J. Chinen" 5 => "M.M. Frank" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Ann Allergy Asthma Immunol" "fecha" => "2005" "volumen" => "94" "paginaInicial" => "S1" "paginaFinal" => "S63" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/15945566" "web" => "Medline" ] ] ] ] ] ] ] ] 26 => array:3 [ "identificador" => "bib0335" "etiqueta" => "27" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Bacillus Calmette-Guerin (BCG) complications associated with primary immunodeficiency diseases" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "S. Norouzi" 1 => "A. Aghamohammadi" 2 => "S. Mamishi" 3 => "S.D. Rosenzweig" 4 => "N. Rezaei" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "J Infection" "fecha" => "2012" "volumen" => "64" "paginaInicial" => "543" "paginaFinal" => "554" "itemHostRev" => array:3 [ "pii" => "S0196070913002573" "estado" => "S300" "issn" => "01960709" ] ] ] ] ] ] ] 27 => array:3 [ "identificador" => "bib0340" "etiqueta" => "28" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Susceptibility to mycobacterial infections in children with X-linked chronic granulomatous disease: a review of 17 patients living in a region endemic for tuberculosis" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "P.P. Lee" 1 => "K.-W. Chan" 2 => "L. Jiang" 3 => "T. Chen" 4 => "C. Li" 5 => "T.-L. Lee" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Pediatric Infect Dis J" "fecha" => "2008" "volumen" => "27" "paginaInicial" => "224" "paginaFinal" => "230" ] ] ] ] ] ] 28 => array:3 [ "identificador" => "bib0345" "etiqueta" => "29" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Immunodeficiency and infections in ataxia-telangiectasia" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "A. Nowak-Wegrzyn" 1 => "T.O. Crawford" 2 => "J.A. Winkelstein" 3 => "K.A. Carson" 4 => "H.M. Lederman" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "J Pediatrics" "fecha" => "2004" "volumen" => "144" "paginaInicial" => "505" "paginaFinal" => "511" ] ] ] ] ] ] 29 => array:3 [ "identificador" => "bib0350" "etiqueta" => "30" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "DOCK8 (dedicator of cytokinesis 8) deficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "H.C. Su" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1097/ACI.0b013e32833fd718" "Revista" => array:5 [ "tituloSerie" => "Curr Opin Allergy Clin Immunol" "fecha" => "2010" "volumen" => "10" "paginaInicial" => "515" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20864884" "web" => "Medline" ] ] ] ] ] ] ] ] 30 => array:3 [ "identificador" => "bib0355" "etiqueta" => "31" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Update on the treatment of primary immunodeficiencies" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "J. García" 1 => "T. Español" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Allergol Immunopathol (Madr)" "fecha" => "2007" "volumen" => "35" "paginaInicial" => "184" "paginaFinal" => "192" ] ] ] ] ] ] 31 => array:3 [ "identificador" => "bib0360" "etiqueta" => "32" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical, functional, and genetic characterization of chronic granulomatous disease in 89 Turkish patients" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "M.Y. Köker" 1 => "Y. Camcıoğlu" 2 => "K. van Leeuwen" 3 => "Kılıç SŞ" 4 => "I. Barlan" 5 => "M. Yılmaz" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:3 [ "tituloSerie" => "J Allergy Clin Immunol" "fecha" => "2013" "volumen" => "132" ] ] ] ] ] ] 32 => array:3 [ "identificador" => "bib0365" "etiqueta" => "33" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Development of cancer in patients with primary immunodeficiencies" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "K. Salavoura" 1 => "A. Kolialexi" 2 => "G. Tsangaris" 3 => "A. Mavrou" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:7 [ "tituloSerie" => "Anticancer Res" "fecha" => "2008" "volumen" => "28" "numero" => "2B" "paginaInicial" => "1263" "paginaFinal" => "1269" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/18505064" "web" => "Medline" ] ] ] ] ] ] ] ] 33 => array:3 [ "identificador" => "bib0370" "etiqueta" => "34" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Mortality and morbidity in common variable immunodeficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Aghamohammadi" 1 => "N. Pouladi" 2 => "N. Parvaneh" 3 => "M. Yeganeh" 4 => "M. Movahedi" 5 => "M. Gharagolou" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1093/tropej/fml077" "Revista" => array:6 [ "tituloSerie" => "J Trop Pediatr" "fecha" => "2007" "volumen" => "53" "paginaInicial" => "32" "paginaFinal" => "38" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17166933" "web" => "Medline" ] ] ] ] ] ] ] ] 34 => array:3 [ "identificador" => "bib0375" "etiqueta" => "35" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Comparison of pulmonary diseases in common variable immunodeficiency and X-linked agammaglobulinaemia" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Aghamohammadi" 1 => "A. Allahverdi" 2 => "H. Abolhassani" 3 => "K. Moazzami" 4 => "H. Alizadeh" 5 => "M. Gharagozlou" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1440-1843.2009.01679.x" "Revista" => array:6 [ "tituloSerie" => "Respirology" "fecha" => "2010" "volumen" => "15" "paginaInicial" => "289" "paginaFinal" => "295" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20051045" "web" => "Medline" ] ] ] ] ] ] ] ] 35 => array:3 [ "identificador" => "bib0380" "etiqueta" => "36" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Molecular analysis of X-linked agammaglobulinemia with growth hormone deficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "M.E. Conley" 1 => "A.W. Burks" 2 => "H.G. Herrod" 3 => "J.M. Puck" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "J Pediatrics" "fecha" => "1991" "volumen" => "119" "paginaInicial" => "392" "paginaFinal" => "397" ] ] ] ] ] ] 36 => array:3 [ "identificador" => "bib0385" "etiqueta" => "37" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Recurrent pyogenic meningitis in a 17-year-old: a delayed presentation of X-linked agammaglobulinemia with growth hormone deficiency" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "G.R. Sabnis" 1 => "N.D. Karnik" 2 => "S.A. Chavan" 3 => "D.S. Korivi" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.4103/0028-3886.82768" "Revista" => array:5 [ "tituloSerie" => "Neurol India" "fecha" => "2011" "volumen" => "59" "paginaInicial" => "435" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21743178" "web" => "Medline" ] ] ] ] ] ] ] ] 37 => array:3 [ "identificador" => "bib0390" "etiqueta" => "38" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "What is Good's syndrome? Immunological abnormalities in patients with thymoma" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "P. Kelleher" 1 => "S. Misbah" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "J Clin Pathol" "fecha" => "2003" "volumen" => "56" "paginaInicial" => "12" "paginaFinal" => "16" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12499426" "web" => "Medline" ] ] ] ] ] ] ] ] 38 => array:3 [ "identificador" => "bib0395" "etiqueta" => "39" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Lipopolysaccharide responsive beige-like anchor subcellular localization involving in vesicle trafficking responsive to lipopolysaccharide" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "M. Reiser" 1 => "K. Li" 2 => "R. Lockey" 3 => "Wang. Jia-Wang" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:4 [ "tituloSerie" => "Austin J Clin Immunol" "fecha" => "2014" "volumen" => "1" "paginaInicial" => "8" ] ] ] ] ] ] 39 => array:3 [ "identificador" => "bib0400" "etiqueta" => "40" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Fatal combined immunodeficiency associated with heterozygous mutation in <span class="elsevierStyleItalic">STAT1</span>" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "N. Sharfe" 1 => "A. Nahum" 2 => "A. Newell" 3 => "H. Dadi" 4 => "B. Ngan" 5 => "S.L. Pereira" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jaci.2013.09.032" "Revista" => array:6 [ "tituloSerie" => "J Allergy Clin Immunol" "fecha" => "2014" "volumen" => "133" "paginaInicial" => "807" "paginaFinal" => "817" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/24239102" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/03010546/0000004400000004/v1_201607130013/S0301054615001512/v1_201607130013/en/main.assets" "Apartado" => array:4 [ "identificador" => "6702" "tipo" => "SECCION" "es" => array:2 [ "titulo" => "Original articles" "idiomaDefecto" => true ] "idiomaDefecto" => "es" ] "PDF" => "https://static.elsevier.es/multimedia/03010546/0000004400000004/v1_201607130013/S0301054615001512/v1_201607130013/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0301054615001512?idApp=UINPBA00004N" ]
| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 7 | 6 | 13 |
| 2024 October | 24 | 15 | 39 |
| 2024 September | 45 | 13 | 58 |
| 2024 August | 48 | 12 | 60 |
| 2024 July | 27 | 3 | 30 |
| 2024 June | 29 | 5 | 34 |
| 2024 May | 22 | 8 | 30 |
| 2024 April | 35 | 7 | 42 |
| 2024 March | 51 | 11 | 62 |
| 2024 February | 24 | 12 | 36 |
| 2024 January | 57 | 7 | 64 |
| 2023 December | 23 | 7 | 30 |
| 2023 November | 28 | 9 | 37 |
| 2023 October | 52 | 5 | 57 |
| 2023 September | 63 | 8 | 71 |
| 2023 August | 51 | 7 | 58 |
| 2023 July | 29 | 4 | 33 |
| 2023 June | 40 | 7 | 47 |
| 2023 May | 76 | 6 | 82 |
| 2023 April | 77 | 3 | 80 |
| 2023 March | 37 | 4 | 41 |
| 2023 February | 49 | 6 | 55 |
| 2023 January | 19 | 8 | 27 |
| 2022 December | 30 | 5 | 35 |
| 2022 November | 33 | 9 | 42 |
| 2022 October | 22 | 19 | 41 |
| 2022 September | 22 | 15 | 37 |
| 2022 August | 41 | 13 | 54 |
| 2022 July | 8 | 5 | 13 |
| 2022 June | 22 | 8 | 30 |
| 2022 May | 19 | 8 | 27 |
| 2022 April | 25 | 7 | 32 |
| 2022 March | 21 | 8 | 29 |
| 2022 February | 22 | 9 | 31 |
| 2022 January | 20 | 14 | 34 |
| 2021 December | 15 | 11 | 26 |
| 2021 November | 21 | 19 | 40 |
| 2021 October | 22 | 7 | 29 |
| 2021 September | 19 | 10 | 29 |
| 2021 August | 11 | 6 | 17 |
| 2021 July | 24 | 8 | 32 |
| 2021 June | 33 | 10 | 43 |
| 2021 May | 36 | 13 | 49 |
| 2021 April | 40 | 15 | 55 |
| 2021 March | 27 | 8 | 35 |
| 2021 February | 24 | 13 | 37 |
| 2021 January | 27 | 10 | 37 |
| 2020 December | 1 | 0 | 1 |
| 2020 October | 1 | 0 | 1 |
| 2020 July | 0 | 1 | 1 |
| 2018 February | 6 | 4 | 10 |
| 2018 January | 6 | 3 | 9 |
| 2017 December | 6 | 2 | 8 |
| 2017 November | 8 | 2 | 10 |
| 2017 October | 17 | 7 | 24 |
| 2017 September | 7 | 3 | 10 |
| 2017 August | 6 | 0 | 6 |
| 2017 July | 3 | 3 | 6 |
| 2017 June | 3 | 9 | 12 |
| 2017 May | 7 | 7 | 14 |
| 2017 April | 1 | 1 | 2 |
| 2016 July | 4 | 2 | 6 |
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