Chronic granulomatous disease: a clinical survey of 41 patients from the Iranian primary immunodeficiency registry.
BACKGROUND
Chronic granulomatous disease (CGD) represents a group of inherited disorders of the phagocytic system, involving recurrent infections at different sites, especially the respiratory system. The present study was accomplished in order to determine the clinical spectrum of Iranian patients with CGD.


METHODS
Forty-one patients (29 males and 12 females) with CGD, who had already been referred to two immunodeficiency referral centers in Iran, were reviewed during a 22-year period (1980-2002).


RESULTS
These patients belonged to 34 families, and 56% of them were consanguineous. The median age at the time of study was 12 years (3 months to 22 years). The median age at onset of symptoms was 4 months (1 month to 12 years), and the median diagnostic age was 5.5 years (2 months to 20 years), with a diagnostic delay of 3 years on average. The most common presenting complaint in our CGD patients was lymphadenopathy (seen in 11 patients, 26.8%). The most common manifestations of CGD (in descending order) were lymphadenopathy (75.6%), pulmonary infections (65.9%) and skin involvement (63.4%) during their illness, followed by gastrointestinal (56.1%), skeletal (29.3%), upper respiratory tract (26.8%) and central nervous system (2.4%) involvement.


CONCLUSIONS
Early diagnosis of the disease is crucial. In view of the possibility of timely treatment, i.e. prophylactic treatment of infection, CGD should be excluded in any patient with unexplained infections or granulomas.
Movahedi
Masoud
M
Immunology, Asthma and Allergy Research Institute, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Aghamohammadi
Asghar
A
Rezaei
Nima
N
Shahnavaz
Nikrad
N
Jandaghi
Ali Babaei
AB
Farhoudi
Abolhasan
A
Pourpak
Zahra
Z
Moin
Mostafa
M
Gharagozlou
Mohammad
M
Mansouri
Davoud
D
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000175:diagnosis / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007492:Iran; D008206:Lymphatic Diseases / Q000209:etiology; D008297:Male; D011379:Prognosis; D012042:Registries