X-linked agammaglobulinemia: a survey of 33 Iranian patients.
In order to determine the clinical and laboratory features of X-linked agammaglobulinemia, the records of 33 male patients with XLA were reviewed during 22 years (1980-2002) in the Iranian referral center of primary immunodeficiency disorders. The patients' ages ranged from 20 to 360 months (median 113 months). The median age at the onset of the disease was 8 months and the median age of diagnosis was 48 months, with a median diagnosis delay of 33 months. Almost all of the patients presented common infectious diseases, which were: pneumonia, otitis, diarrhea, sinusitis, and arthritis. During the course of illness, infections in the respiratory tract, gastrointestinal tract, central nervous system, and musculoskeletal system were seen in 93.9%, 75.8%, 33.3%, and 21.2% of XLA patients, respectively. The most common complications of these patients were chronic infections in 75.8% of them, including: chronic otitis media, chronic sinusitis, chronic diarrhea, and bronchiectasis.
Moin
Mostafa
M
Department of Allergy and Clinical Immunology of Children's Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran. rezaei_nima@yahoo.com
Aghamohammadi
Asghar
A
Farhoudi
Abolhasan
A
Pourpak
Zahra
Z
Rezaei
Nima
N
Movahedi
Masoud
M
Gharagozlou
Mohammad
M
Ghazi
Bahram Mir Saeid
BM
Zahed
Ali
A
Abolmaali
Kamran
K
Mahmoudi
Maryam
M
Emami
Leila
L
Bashashati
Mohammad
M
D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000453:epidemiology / Q000235:genetics* / Q000276:immunology*; D017668:Age of Onset; D016175:B-Lymphocyte Subsets; D002648:Child; D002675:Child, Preschool; D003141:Communicable Diseases / Q000097:blood / Q000209:etiology* / Q000276:immunology; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000453:epidemiology / Q000276:immunology*; D006801:Humans; D007136:Immunoglobulins / Q000097:blood / Q000172:deficiency; D007223:Infant; D007492:Iran / Q000453:epidemiology; D018655:Lymphocyte Count; D008297:Male; D012008:Recurrence; D016176:T-Lymphocyte Subsets
D007136:Immunoglobulins