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. Copyright 2004 S. Karger AG, Basel
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 CGDpatients 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. Copyright 2004 S. Karger AG, Basel
Authors: Davood Mansouri; Parisa Adimi; Mehdi Mirsaedi; Nahal Mansouri; Payam Tabarsi; Majid Amiri; Hamid R Jamaati; Masoud Motavasseli; Noushin Baghaii; Ali Cheraghvandi; Reza Rouhi; Navid A Roozbahany; Soheila Zahirifard; Forouzan Mohammadi; Mohammad R Masjedi; Ali A Velayati; Jean L Casanova; David P Speert; R Kevin Elwood; Robert Schellenberg; Stuart E Turvey Journal: J Clin Immunol Date: 2005-07 Impact factor: 8.317
Authors: Meghna Alimchandani; Jin-Ping Lai; Phyu Phyu Aung; Sajneet Khangura; Natasha Kamal; John I Gallin; Steven M Holland; Harry L Malech; Theo Heller; Markku Miettinen; Martha M Quezado Journal: Am J Surg Pathol Date: 2013-09 Impact factor: 6.394