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Vol. 45. Issue 3.
Pages 244-250 (May - June 2017)
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Vol. 45. Issue 3.
Pages 244-250 (May - June 2017)
Original Article
DOI: 10.1016/j.aller.2016.11.001
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Primary immunodeficiency diseases in Northern Iran
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I. Mohammadzadeha,b, B. Moazzamia,b, J. Ghaffaric, A. Aghamohammadid,e, N. Rezaeid,f,g,
Corresponding author
rezaei_nima@tums.ac.ir

Corresponding author.
a Noncommunicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, Iran
b Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Babol, Iran
c Department of Pediatrics, School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran
d Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
e Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
f Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
g Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Boston, MA, USA
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Abstract
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Figures (1)
Tables (3)
Table 1. Frequency and demographic data of 94 PID patients by phenotype.
Table 2. Frequency of common infection in different phenotypes of 94 patients with primary immunodeficiency disorders.
Table 3. Distribution of some PID subtypes in different countries.
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Abstract
Introduction

Primary immunodeficiency diseases (PID) are a heterogeneous group of inherited disorders, characterised by recurrent severe infections, autoimmunity and lymphoproliferation. Despite impressive progress in identification of novel PID, there is an unfortunate lack of awareness among physicians in identification of patients with PID, especially in non-capital cities of countries worldwide.

Result

This study was performed in a single-centre paediatric hospital in Northern Iran during a 21-year period (1994–2015). Ninety-four patients were included in this study. The majority of cases had antibody deficiencies (37.23%), followed by well-defined syndromes with immunodeficiency in 16 (17.02%), phagocytic disorders in 15 patients (15.95%), complement deficiencies in 15 patients (15.95%), immunodeficiencies affecting cellular and humoral immunity in nine patients (9.57%), disease of immune dysregulation in three (3.19%), and defects in intrinsic and innate immunity in one (1.06%).

Conclusion

It seems that there are major variations in frequency of different types of PID in different regions of a country. Therefore, reporting local data could provide better ideas to improve the local health care system strategists and quality of care of PID patients.

Keywords:
Primary immunodeficiency diseases
Hypogammaglobulinaemia
Phagocyte defects
Combined immunodeficiencies
Frequency
Full Text
Introduction

Primary immunodeficiency diseases (PIDs) are a heterogeneous group of disorders, characterised by increased susceptibility to recurrent infections and malignancies.1,2 As a result in improvements in immunological and molecular diagnostic methods, more than 300 different PIDs have been currently identified.3–5 Despite the increasing trend in diagnosis of patients with PID, lack of awareness among physicians continues to play an important role in the delay of diagnosis in this group of patients.6–8 This in turn will delay the initiation of appropriate treatments and lead to increases in morbidity and mortality.6,7 Therefore, evaluation of immune function is required for patients who present with unusual, chronic or recurrent clinical manifestations such as severe systemic bacterial infection, serious upper and lower respiratory tract infections and liver or brain abscesses.

Since 1999, the Iranian Primary Immunodeficiency Registry (IPIDR) has collected data in order to estimate the prevalence of various types of PID in Iran.9 According to epidemiological studies, wide variations exist in geographical and racial prevalence as well as in the frequency of different types of PID.10–15 Therefore, regional data regarding the prevalence of various types of PID can work as a powerful tool in improving the local health care system strategies and quality of care of patients diagnosed with PID.16 The aim of our study was therefore to determine the frequency of PID in Northern Iran over a period of 21 years from 1994 to 2015.

Patients and methods

In the present study, we analysed the medical charts of 94 patients with PID who were referred to the allergy/immunology clinic in Amir-Kola Hospital in Babol, Mazandaran, province of Iran, over a period of 21 years (1994–2015). All patients were diagnosed with immunodeficiency according to Pan-American Group for Immunodeficiency (PAGID) and European society for immunodeficiencies (ESID) criteria.17

Secondary causes of immunodeficiency such as Patients with Human Immunodeficiency Virus (HIV) infection, organ transplantation or radiotherapy were excluded using standardised laboratory tests. A questionnaire was designed to collect information regarding demographic data including name, age, sex, consanguinity of parents, family history of any immunological disorders, age of onset, age at time of PID diagnosis and recurrent episodes of infections.

Laboratory analysis contained complete blood count performed by automated blood counting machine, Erythrocyte Sedimentation Rate (ESR) measured by Western Green Method, haemagglutinins measured by CA1600 and serum immunoglobulin levels (IgG, IgE, IgA, and IgM) measured by Enzyme-Linked ImmunoSorbent Assay (ELISA), reviewing Cluster of Differentiation (CD) markers done by flow cytometry, the level of serum complement components (C3, C4, CH50) measured by ELISA, delayed cutaneous hypersensitivity reactions test (purified protein derivative), lymphocyte transformation test, isohaemagglutinin tests, IgG subclasses serum level, evaluation of lymphocyte subtypes, chemotaxis evaluation, nitro blue tetrazolium dye test and haemolytic titration of complement (CH50) as needed.

Definitions of different immunodeficiency disorders were made according to the following criteria: Impaired T-cell function was defined by absence of lymphocyte proliferation cells in response to mitogens (e.g., PHA) and antigens, with normal T lymphocyte count. CD4 deficiency was defined as isolated absence of CD4 T-cell (or very low count) without any evidence of secondary immunodeficiency (HIV infection) or other known immunodeficiency (normal expression of HLADR). CD8 deficiency was defined as isolated absence of CD8 T-cell. Patients were considered to have HIGM if they had serum IgG concentrations at least two standard deviations below normal for their age and a confirmed mutation in any of the following genes: CD40LG (XHIGM) or AICDA, CD40, UNG (all ARHIGM), or PIK3CD (ADHIGM). The diagnosis of CVID was made with decreased serum IgG, IgM and/or IgA level, decreased response to vaccines, a B cell number more than 1% of total lymphocytes, age older than four years old at the time of diagnosis, and exclusion of other causes of hypogammaglobulinaemia. All DiGeorge syndrome cases were confirmed by chromosomal analysis that had been fully analysed by conventional and G-banding methods. The 22q11.2 deletion was confirmed using fluorescence in situ hybridisation (FISH) with a commercially-available region-specific probe (Vysis LSI D22S75 (N25 region) Spectrum Orange/LSI ARSA SpectrumGreen).

This study was approved by the Ethics Committee of the Medical University of Babol. All the data were analysed by statistical software SPSS-19.

Results

Ninety-four PID patients, 56 males and 38 females, were enrolled in our study. The male-to-female ratio was 1.47:1. As shown in Fig. 1, patients were classified into seven main groups of PID according to the International Union of Immunological Societies (IUIS) classification5 (Fig. 1). At the time of diagnosis, 84 patients (89.36%) were under the age of 16 years. The mean age of patients at the onset of symptoms was 4.52±9.68 years and the mean age at the time of diagnosis was 6.79±10.34 years. The youngest patient registered was one month old while the oldest presented at 69 years of age. The mean gap between diagnostic delay for a number of PIDs such as common variable immunodeficiency (CVID), X-linked agammaglobulinaemia (XLA) and chronic granulomatous disease (CGD) were 43.9 months, 19.5 months and 21 months, respectively.

Figure 1.

Frequency of different groups of primary immunodeficiency diseases.

(0.17MB).

The majority of cases in the present cohort had antibody deficiencies, being present in 35 patients (37.23%), followed by well-defined syndromes with immunodeficiency in 16 (17.02%), phagocytic disorders in 15 patients (15.95%), complement deficiencies in 15 patients (15.95%), immunodeficiencies affecting cellular and humoral immunity in nine patients (9.57%), disease of immune dysregulation in three (3.19%), and defects in intrinsic and innate immunity in one (1.06%) (Fig. 1).

Among all patients, 22 (23.40%) died, while three (3.19%) were inaccessible for their last follow up. The majority of patients belonged to the paediatric age group (93.2%). The frequency and demographic variations of different types of PID are demonstrated in Table 1.

Table 1.

Frequency and demographic data of 94 PID patients by phenotype.

Female  Male  Age of study (range)  Percentage  Frequency  PIDs categories and subtypes 
11  24  2m–17y  37.23%  35  Predominantly antibody deficiency 
2m–17y  11.7%  11  Common variable
Immunodeficiency 
6m–6y  9.57%  X-linked agammaglobulinaemia 
1.5–4.5y  4.25%  Hyper IgM syndromes 
1–7y  10.63%  10  Selective IgA deficiency 
1–2y  1.06%  μ Heavy Chain deficiency 
1m–8y  9.57%  Immunodeficiencies affecting cellular and humoral immunity 
2–8y  1.06%  LRBA deficiency 
2–18m  8.51%  Severe combined
Immunodeficiency 
1–5y  1.06%  Defects in intrinsic and innate immunity 
1–5y  1.06%  Chronic mucocutaneous
Candidiasis 
1m–12y  15.95%  15  Congenital defects of phagocyte number, function, or both 
1m–7y  8.51%  Chronic granulomatous disease 
1–2m  3.19%  Leucocyte adhesion defect 
1–4y  2.12%  Cyclic neutropenia 
12y  1.06%  Severe congenital
Neutropenia's 
6–8m  1.06%  Kostman disease 
6m–12y  17.02%  16  Well-defined syndromes with immunodeficiency 
7m–7y  2.12%  Hyper IgE syndrome 
6m–12y  10.63%  10  Ataxia telangiectasia 
6m–12y  3.19%  Wiskott-Aldrich syndrome 
1–1m  1.06%  DiGeorge syndrome 
6m–2y  3.19%  Diseases of immune dysregulation 
6m–2y  1.06%  Chediak-Higashi syndrome 
6–10m  2.12%  Griscelli syndrome type II 
4–69y  15.95%  15  Complement deficiencies 
4–69y  15.95%  15  C1 inhibitor deficiency 
38  56  1m–69y  100%  94  Total 

m: months; y: years.

From all patients registered in this study, antibody deficiencies were the most prevalent immunodeficiency disorders, diagnosed in 35 cases which consisted of common variable immunodeficiency (CVID) in 11 cases, X-linked agammaglobulinaemia (XLA) in nine cases, selective IgA deficiency in 10 cases, hyper-IgM syndrome in four patients and μ Heavy Chain deficiency in only one patient. Among these patients, the most frequent infectious manifestation of CVID patients was pneumonia followed by diarrhoea, otitis media and upper respiratory tract infections, respectively (Table 2). For patients diagnosed with XLA, severe upper and lower respiratory tract infections were the most common infectious manifestation. The main symptom of Hyper-IgM syndrome was sinopulmonary infections.

Table 2.

Frequency of common infection in different phenotypes of 94 patients with primary immunodeficiency disorders.

Neutropenia  Angio-oedema  Eczema  Lymphoma  BCGosis  Candidiasis  Deep abscess  Superficial abscess  Meningitis/encephalitis  Septic arthritis  Sinusitis  Osteomyelitis  Diarrhoea  Otitis media  Pneumonia   
1 (2.85%)  0 (0%)  0 (0%)  1 (2.85%)  1 (2.85%)  0 (0%)  0 (0%)  0 (0%)  2 (5.71%)  2 (5.71%)  10 (28.57%)  0 (0%)  11 (31.42%)  14 (40%)  23 (65.7%)  Humoral def (n=35) 
CVID (n=11) 
XLA (n=9) 
Selective IgA def (n=10) 
Hyper-IgM syndrome (n=4)
Selective 
μ Heavy Chain deficiency (n=1) 
0 (0%)  0 (0%)  0 (0%)  0 (0%)  2 (22.22%)  6 (66.66%)  0 (0%)  0 (0%)  0 (0%)  1 (11.11%)  0 (0%)  0 (0%)  6 (66.66%)  1 (11.11%)  5 (55.55%)  Immunodeficiencies affecting cellular and humoral immunity (n=9) 
SCID (n=8) 
LRBA (n=1) 
0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  1 (100%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  Defects in innate immunity (n=1) 
CMCC (n=1) 
0 (0%)  0 (0%)  0 (0%)  0 (0%)  5 (33.33%)  0 (0%)  2 (13.33%)  1 (6.66%)  3 (20%)  0 (0%)  0 (0%)  4 (26.66%)  3 (20%)  1 (6.66%)  9 (60%)  Phagocytic def (n=15) 
CGD (n=8) 
LAD (n=3) 
Cyclic neutropenia (n=2) 
Severe congenital neutropenia's (n=1) 
Kostman disease (n=1) 
1 (6.25%)  0 (0%)  4 (25%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  6 (37.5%)  0 (0%)  7 (43.75%)  0 (0%)  0 (0%)  5 (31.25%)  10 (62.5%)  Well-defined syndromes with immunodeficiency (n=16) 
Hyper IgE syndrome (n=2) 
AT (n=10) 
Wiskott-Aldrich syndrome (n=3) 
DiGeorge syndrome (n=1) 
0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  1 (33.33%)  2 (66.66%)  Diseases of immune dysregulation (n=3) 
Chediak-Higashi syndrome (n=1) 
Griscelli syndrome type II (n=2) 
0 (0%)  15 (100%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  0 (0%)  Complement deficiencies (n=15) 
15  Hereditary angio-oedema (n=15) 

The frequency of the well-defined immunodeficiency syndromes was 16 (17.02%), including ataxia telangiectasia (AT) in 10 patients. Their main clinical presentations were pneumonia (62.5%) followed by sinusitis (43.75%).

Fifteen patients (15.95%) had phagocytic disorders including chronic granulomatous disease in eight patients, Leucocyte adhesion defect in three, cyclic neutropenia in two, severe congenital neutropenia in one and Kostman disease in one. The most common clinical features among this group were pneumonia, BCGosis, osteomyelitis, diarrhoea, meningitis and otitis media respectively.

Immunodeficiencies affecting cellular and humoral immunity were diagnosed in nine patients (9.57%) with the majority diagnosed with severe combined immunodeficiency (SCID) with candidiasis being the most predominant symptom in six patients (66.66%). Other symptoms including diarrhoea and pneumonia were detected in six and four patients, respectively.

Among patients with complement deficiencies, 15 patients (15.95%) were diagnosed with hereditary angio-oedema. In almost all of these patients, no significant infectious manifestation was present, with angio-oedema being their only clinical presentation at the time of diagnosis.

Disease of immune dysregulation involved three cases (3.19%), including two cases of Griscelli syndrome type 2 and one case of Chediak-Higashi syndrome. Both cases of Griscelli syndrome type 2 died due to the severe respiratory tract infection.

Discussion

In the present report, we evaluated the frequency and aetiology of 94 patients who were identified as PID according to the latest IUIS classification at our immunology/allergy clinic during a period of 21 years.5 These patients were referred to Amir-kola Pediatrics hospital, the main referral hospital in Babol, Mazandaran province. Mazandaran is a northern state in Iran with a population of 3,074,000 located to the south of the Caspian Sea, indicating a PID prevalence of 30.5 per 1,000,000 populations. This is significantly higher compared to overall prevalence of PID in Iran which was estimated to be 9.7 per 1,000,000 population during the years 2006 to 2013.18

The major aim of this study was to determine the prevalence of various types of PID in the North of Iran and provide local demographic information. It should be emphasised that the result of the current report does not necessarily reflect the true prevalence of PID, which may be due to lack of awareness among physicians and inadequate available diagnostic capabilities. In severe cases of PID such as SCID, many patients die in their early childhood before the exact diagnosis is made and their cause of death remains unknown.9,19 In addition, patients with mild forms of the disorder are usually managed by general physicians or other specialists and are not referred to any tertiary centres.20–22 Early diagnosis and management of PID has a crucial role in diminishing the rate of morbidity and mortality, and increasing the quality of life in PID patients.18,23,24

The overwhelming majority of our PID cases were diagnosed in children (93.2%), which was higher than previous reports from Iran. This contrast in age range among our PID patients with other reports may be due to our lower patient population study and also a poor referral system for adult patients (20).

As shown in Table 3, the spectrum of PID observed in the present study was similar to previous studies.6,25–30 Patients with antibody deficiency were identified in 65% of the PID population. This is consistent with prior international reports including data from Europe (54.82%), Turkey (74%), Australia (71%), Korea (53.3%), Japan (52.9%), and Switzerland (66.2%).27,29–32 Among patients with antibody deficiency, our results indicated CVID to be the most prevalent disorder followed by IgA deficiency and XLA. These findings are in concordance with the study carried out by the Iranian Primary Immunodeficiency Registry (IPIDR), indicating CVID to be the most prevalent antibody disorder in the country.9 While many other registries have shown IgA deficiency to be the most common phenotype of PID, we could only identify 10 patients (10.63%) in our cohort with IgA deficiency.33,34 This discrepancy in low incidence of IgA deficiency among our patients’ population may be resulting from asymptomatic presentation of IgA deficiency in most of the cases, leading to a lower rate of diagnosis for this type of PID.9

Table 3.

Distribution of some PID subtypes in different countries.

ESID database26
No. (16,547) 
Iceland41
No. (66) 
Switzerland29
No. (348) 
Egypt40
No. (206) 
Tunisia39
No. (710) 
Morocco38
No. (421) 
Qatar36
No. (131) 
Oman37
No. (90) 
Kuwait19
No. (76) 
Turkey27
No. (1435) 
Iran, current study  Category 
56.4%  39%  62.4%  14%  17.7%  22.7%  23.7%  18%  30%  73.9%  37.23%  Predominantly antibody deficiency 
14.9%  15%  8.3%  18%  22.7%  27.4%  22.9%  13%  30%  5.5%  17.02%  Well-defined syndromes with immunodeficiency 
7.78%    6.6%  41%  28.6%  20.6%  19.1%  12%  21%  2%  9.57%  Combined immunodeficiency diseases 
8.4%  12%  8.9%  10%  25.4%  17.5%  19.1%  42%  8%  3.5%  15.95%  Phagocyte defects 
  29%  4.6%  1%  0.40%  0.95%    6%  4%  0.4%  15.95%  Complement deficiencies 

In the present study, phagocytic disorders comprised 15.95% of our PID populations with CGD being the most common form (8.51%), which was shown to be similar to results from Latin American countries and Japan (8.6% and 11.9%, respectively), but significantly higher than European studies.26,32,33

In patients with autosomal recessive disorders, such as Chediak-Higashi syndrome, Griscelli syndrome, CGD and severe congenital neutropenia (SCN), consanguinity marriages seem to be a major risk factor.16,35 The overall average of the consanguinity rate found in our study was 24.7%, which is higher than in other studies, but still lower than the overall consanguinity rate of the country's population (38.6%).16 The consanguinity rate among our patients diagnosed with immune dysregulation was 100%, which is higher in comparison with other reports such as in Europe and other Asian countries which have been shown to have lower rates of consanguinity marriages.6,26,28

The present study is the first report of PID in Northern Iran. Variations in frequency of different types of PID exist in different regions of Iran. Therefore, collecting local data can be used to improve the local health care system strategists and quality of care of patients diagnosed with PID.

Ethical disclosuresConfidentiality of data

The authors declare that no patient data appears in this article.

Right to privacy and informed consent

The authors have obtained the informed consent of the patients and/or subjects mentioned in the article. The author for correspondence is in possession of this document.

Protection of human subjects and animals in research

The authors declare that the procedures followed were in accordance with the regulations of the responsible Clinical Research Ethics Committee and in accordance with those of the World Medical Association and the Helsinki Declaration.

Conflict of interest

The authors have no conflict of interest to declare.

References
[1]
A. Aghamohammadi, M. Moein, A. Farhoudi, Z. Pourpak, N. Rezaei, K. Abolmaali, et al.
Primary immunodeficiency in Iran: first report of the National Registry of PID in Children and Adults.
J Clin Immunol, 22 (2002), pp. 375-380
[2]
M. Ebadi, A. Aghamohammadi, N. Rezaei.
Primary immunodeficiencies: a decade of shifting paradigms, the current status and the emergence of cutting-edge therapies and diagnostics.
Expert Rev Clin Immunol, 11 (2015), pp. 117-139
[3]
F.A. Bonilla, R.S. Geha.
Primary immunodeficiency diseases.
J Allergy Clin Immunol, 111 (2003), pp. S571-S581
[4]
A. Bousfiha, L. Jeddane, W. Al-Herz, F. Ailal, Casanova Jl, T. Chatila, et al.
The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies.
J Clin Immunol, 35 (2015), pp. 727-738
[5]
C. Picard, W. Al-Herz, A. Bousfiha, J.L. Casanova, T. Chatila, M.E. Conley, et al.
Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency 2015.
J Clin Immunol, 35 (2015), pp. 696-726
[6]
W. Al-Herz, M.E. Zainal, M. Salama, W. Al-Ateeqi, K. Husain, M. Abdul-Rasoul, et al.
Primary immunodeficiency disorders: survey of pediatricians in Kuwait.
J Clin Immunol, 28 (2008), pp. 379-383
[7]
K. Nourijelyani, A. Aghamohammadi, M. Salehi Sadaghiani, N. Behniafard, H. Abolhassani, S. Pourjabar, et al.
Physicians awareness on primary immunodeficiency disorders in Iran.
Iran J Allergy Asthma Immunol, 11 (2012), pp. 57-64
[8]
N. Rezaei, P. Mohammadinejad, A. Aghamohammadi.
The demographics of primary immunodeficiency diseases across the unique ethnic groups in Iran, and approaches to diagnosis and treatment.
Ann NY Acad Sci, 1238 (2011), pp. 24-32
[9]
N. Rezaei, A. Aghamohammadi, M. Moin, Z. Pourpak, M. Movahedi, M. Gharagozlou, et al.
Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian Primary Immunodeficiency Registry.
J Clin Immunol, 26 (2006), pp. 519-532
[10]
A. Aghamohammadi, M. Moin, N. Rezaei.
History of primary immunodeficiency diseases in Iran.
Iran J Pediatr, 20 (2010), pp. 16-34
[11]
J.M. Boyle, R.H. Buckley.
Population prevalence of diagnosed primary immunodeficiency diseases in the United States.
J Clin Immunol, 27 (2007), pp. 497-502
[12]
H.C. Gooi.
Primary immunodeficiency register, United Kingdom: update 1992.
Immunodeficiency, 4 (1993), pp. 191-192
[13]
H. Hayakawa, T. Iwata, J. Yata, N. Kobayashi.
Primary immunodeficiency syndrome in Japan. I. Overview of a nationwide survey on primary immunodeficiency syndrome.
J Clin Immunol, 1 (1981), pp. 31-39
[14]
G. Luzi, L. Businco, F. Aiuti.
Primary immunodeficiency syndromes in Italy: a report of the national register in children and adults.
J Clin Immunol, 3 (1983), pp. 316-320
[15]
O. Ryser, A. Morell, W.H. Hitzig.
Primary immunodeficiencies in Switzerland: first report of the national registry in adults and children.
J Clin Immunol, 8 (1988), pp. 479-485
[16]
N. Rezaei, Z. Pourpak, A. Aghamohammadi, A. Farhoudi, M. Movahedi, M. Gharagozlou, et al.
Consanguinity in primary immunodeficiency disorders; the report from Iranian Primary Immunodeficiency Registry.
Am J Reprod Immunol, 56 (2006), pp. 145-151
[17]
M.E. Conley, L.D. Notarangelo, A. Etzioni.
Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies).
Clin Immunol, 93 (1999), pp. 190-197
[18]
A. Aghamohammadi, P. Mohammadinejad, H. Abolhassani, B. Mirminachi, M. Movahedi, M. Gharagozlou, et al.
Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry.
J Clin Immunol, 34 (2014), pp. 478-490
[19]
W. Al-Herz, M.E. Zainal, H.M. Alenezi, K. Husain, S.H. Alshemmari.
Performance status and deaths among children registered in Kuwait National Primary Immunodeficiency Disorders Registry.
Asian Pac J Allergy Immunol, 28 (2010), pp. 141-146
[20]
J.M. Garcia, P. Gamboa, A. de la Calle, M.D. Hernández, M.T. Caballero, B.E. García, et al.
Diagnosis and management of immunodeficiencies in adults by allergologists.
J Investig Allergol Clin Immunol, 20 (2010), pp. 185-194
[21]
J. Litzman, D. Stikarovska, Z. Pikulova, T. Pavlik, S. Pesak, V. Thon, et al.
Change in referral diagnoses and diagnostic delay in hypogammaglobulinaemic patients during 28 years in a single referral centre.
Int Arch Allergy Immunol, 153 (2010), pp. 95-101
[22]
S. Mamishi, A.N. Eghbali, N. Rezaei, H. Abolhassani, N. Parvaneh, A. Aghamohammadi.
A single center 14 years study of infectious complications leading to hospitalization of patients with primary antibody deficiencies.
Braz J Infect Dis, 14 (2010), pp. 351-355
[23]
H. Abolhassani, A. Aghamohammadi, F. Abolhassani, H. Eftekhar, M. Heidarnia, N. Rezaei.
Health policy for common variable immunodeficiency: burden of the disease.
J Investig Allergol Clin Immunol, 21 (2011), pp. 454-458
[24]
K. Zebracki, T.M. Palermo, R. Hostoffer, K. Duff, D. Drotar.
Health-related quality of life of children with primary immunodeficiency disease: a comparison study.
Ann Allergy Asthma Immunol, 93 (2004), pp. 557-561
[25]
M. Abuzakouk, C. Feighery.
Primary immunodeficiency disorders in the Republic of Ireland: first report of the national registry in children and adults.
J Clin Immunol, 25 (2005), pp. 73-77
[26]
Immunodeficiencies ESf. ESID database statistics. 2013.
[27]
S.S. Kilic, M. Ozel, D. Hafizoglu, N.E. Karaca, G. Aksu, N. Kutukculer.
The prevalences [correction] and patient characteristics of primary immunodeficiency diseases in Turkey – two centers study.
J Clin Immunol, 33 (2013), pp. 74-83
[28]
P. Kirkpatrick, S. Riminton.
Primary immunodeficiency diseases in Australia and New Zealand.
J Clin Immunol, 27 (2007), pp. 517-524
[29]
K. Marschall, M. Hoernes, M. Bitzenhofer-Gruber, P. Jandus, A. Duppenthaler, W.A. Wuillemin, et al.
The Swiss National Registry for Primary Immunodeficiencies: report on the first 6 years’ activity from 2008 to 2014.
Clin Exp Immunol, 182 (2015), pp. 45-50
[30]
J.W. Rhim, K.H. Kim, D.S. Kim, B.S. Kim, J.S. Kim, C.H. Kim, et al.
Prevalence of primary immunodeficiency in Korea.
J Korean Med Sci, 27 (2012), pp. 788-793
[31]
K.W. Baumgart, W.J. Britton, A. Kemp, M. French, D. Roberton.
The spectrum of primary immunodeficiency disorders in Australia.
J Allergy Clin Immunol, 100 (1997), pp. 415-423
[32]
M. Ishimura, H. Takada, T. Doi, K. Imai, Y. Sasahara, H. Kanegane, et al.
Nationwide survey of patients with primary immunodeficiency diseases in Japan.
J Clin Immunol, 31 (2011), pp. 968-976
[33]
L.E. Leiva, M. Zelazco, M. Oleastro, M. Carneiro-Sampaio, A. Condino-Neto, B.T. Costa-Carvalho, et al.
Primary immunodeficiency diseases in Latin America: the second report of the LAGID registry.
J Clin Immunol, 27 (2007), pp. 101-108
[34]
M. Zelazko, M. Carneiro-Sampaio, M. Cornejo de Luigi, D. Garcia de Olarte, O. Porras Madrigal, R. Berrón Perez, et al.
Primary immunodeficiency diseases in Latin America: first report from eight countries participating in the LAGID. Latin American Group for Primary Immunodeficiency Diseases.
J Clin Immunol, 18 (1998), pp. 161-166
[35]
A. Bittles.
Consanguinity and its relevance to clinical genetics.
Clin Genet, 60 (2001), pp. 89-98
[36]
M.S. Ehlayel, A. Bener, M.A. Laban.
Primary immunodeficiency diseases in children: 15 year experience in a tertiary care medical center in Qatar.
J Clin Immunol, 33 (2013), pp. 317-324
[37]
S. Al-Tamemi, E. Ibtisam, D. Dennison.
Primary immunodeficiency diseases in Oman: five years’ experience at Sultan Qaboos University Hospital.
World Allergy Organ J, 5 (2012), pp. 52-56
[38]
A.A. Bousfiha, L. Jeddane, N. El Hafidi, N. Benajiba, N. Rada, J. El Bakkouri, et al.
First report on the Moroccan registry of primary immunodeficiencies: 15 years of experience (1998–2012).
J Clin Immunol, 34 (2014), pp. 459-468
[39]
F. Mellouli, I.B. Mustapha, M.B. Khaled, H. Besbes, M. Ouederni, N. Mekki, et al.
Report of the Tunisian Registry of Primary Immunodeficiencies: 25-years of experience (1988–2012).
J Clin Immunol, 35 (2015), pp. 745-753
[40]
M.R. Barbouche, N. Galal, I. Ben-Mustapha, L. Jeddane, F. Mellouli, F. Ailal, et al.
Primary immunodeficiencies in highly consanguineous North African populations.
Ann NY Acad Sci, 1238 (2011), pp. 42-52
[41]
B.R. Ludviksson, S.T. Sigurdardottir, J.H. Johannsson, A. Haraldsson, T.O. Hardarson.
Epidemiology of primary immunodeficiency in Iceland.
J Clin Immunol, 35 (2015), pp. 75-79
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