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Mohammadinejad P1,
Pourhamdi S1, Abolhassani H1,2,
Mirminachi B1, Havaei A1, Masoom
SN1, Sadeghi B1, Ghajar A1,
Afarideh M1, Parvaneh N1, Mirsaeed-Ghazi
B3, Movahedi M4, Gharagozlou M4,
Chavoushzadeh Z5, Mahdaviani A6,
Zandieh F3, Sherkat R7,
Sadeghi-Shabestari M8, Faridhosseini R9,
Jabbari-Azad F9, Ahanchian H9,
Zandkarimi M9, Cherghi T10, Fayezi
A11, Mohammadzadeh I12, Amin R13,
Aleyasin S13, Moghtaderi M13,
Ghaffari J14, Bemanian M15,
Shafiei A3,15, Kalantari N16,
Ahmadiafshar A17, Khazaei HA18,
Mohammadi J19, Nabavi M20, Rezaei
N1,21, Aghamohammadi A1 |
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1Research Center for
Immunodeficiencies, Pediatrics Center of Excellence,
Childrens Medical Center, Tehran University of Medical
Sciences, Tehran, Iran
2Division of Clinical Immunology, Department
of Laboratory Medicine, Karolinska Institutet at
Karolinska University Hospital Huddinge, Stockholm,
Sweden
3Department of Immunology, Bahrami Hospital,
Tehran University of Medical Sciences, Tehran, Iran
4Department of Allergy and Clinical
Immunology, Pediatrics Center of Excellence, Childrens
Medical Center, Tehran University of Medical Sciences,
Tehran, Iran
5Pediatric Infectious Research Center, Mofid
Children's Hospital, Shahid Beheshti University of
Medical Sciences, Tehran, Iran
6Pediatric Respiratory Disease Research
Center, National Research Institute of Tuberculosis and
Lung Disease, Masih Daneshvari Hospital, Shahid Beheshti
University of Medical Sciences, Tehran, Iran
7Acquired Immunodeficiency Research Center,
Al-Zahra Hospital, Isfahan University of Medical
Sciences, Isfahan, Iran
8Department of Immunology and Allergy, Tabriz
University of Medical Sciences, Tabriz, Iran
9Allergy Research Center, School of Medicine,
Mashhad University of Medical Sciences, Mashhad, Iran
10Department of Pediatrics, 17th Shahrivar
Children's Hospital, Guilan University of Medical
Sciences, Rasht, Iran
11Department of Immunology and Allergy, Ahvaz
University of Medical Sciences, Ahvaz, Iran
12Noncommunicable Pediatric Diseases Research
Center, Amirkola Hospital, Babol University of Medical
Sciences, Babol, Iran
13Department of Pediatric Immunology and
Allergy, Namazi Hospital, Shiraz University of Medical
Sciences, Shiraz, Iran
14Department of Pediatrics, Mazandaran
University of Medical Sciences, Sari, Iran
15Department of Immunology and Allergy, Yazd
University of Medical Sciences, Yazd, Iran
16Department of Immunology and Allergy,
Golestan University of Medical Sciences, Gorgan, Iran
17Department of Immunology and Allergy,
Ghazvin University of Medical Sciences, Ghazvin, Iran
18Department of Immunology and Hematology,
Zahedan Medical Sciences University, Zahedan, Iran
19Department of Life Science, Faculty of New
Science and Technology, University of Tehran, Tehran,
Iran
20Department of Allergy and Clinical
Immunology, Rasool e Akram Hospital, Iran University of
Medical Sciences, Tehran, Iran
21Molecular Immunology Research Center and
Department of Immunology, School of Medicine, Tehran
University of Medical Sciences, Tehran, Iran |
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Abstract |
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Background:
Primary antibody
deficiency (PAD) is
the most common
group of primary
immunodeficiency
disorders (PID),
with a broad
spectrum of clinical
features ranging
from severe and
recurrent infections
to asymptomatic
disease.
Objectives:
The current study
was performed to
evaluate and compare
demographic and
clinical data in the
most common types of
PAD.
Materials and
Methods: We
performed a
retrospective review
of the medical
records of all PAD
patients with a
confirmed diagnosis
of common variable
immunodeficiency (CVID),
hyper IgM syndrome (HIgM),
selective IgA
deficiency (SIgAD),
and X-linked
agammaglobulinemia (XLA)
who were diagnosed
during the last 30
years at the
Childrens Medical
Center, Tehran,
Iran.
Results: A
total number of 280
cases of PAD (125
CVID, 32 HIgM, 63
SIgAD, and 60 XLA)
were enrolled in the
study. The median
(range) age at the
onset of disease in
CVID, HIgM, SIgAD,
and XLA was 2
(0-46), 0.91 (0-9),
1 (0-26), and 1
(0-10) years,
respectively.
Gastrointestinal
infections were more
prevalent in CVID
patients, as were
central nervous
system infections in
XLA patients.
Autoimmune
complications were
more prevalent in
HIgM patients,
malignancies in CVID
patients, and
allergies in SIgAD
patients. The
mortality rate for
CVID, HIgM, and XLA
was 27.2%, 28.1%,
and 25%,
respectively. No
deaths were reported
in SIgAD patients.
Conclusions:
SIgAD patients had
the best prognosis.
While all PAD
patients should be
monitored for
infectious
complications,
special attention
should be paid to
the finding of
malignancy and
autoimmune disorders
in CVID and HIgM
patients,
respectively.
Key words:
Common variable
immunodeficiency.
Immunoglobulin A
deficiency.
Infection. Hyper-IgM
syndrome. X-linked
agammaglobulinemia. |
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