Source: Annual Congress 2001 - Immune deficiencies and the lung in childhood
Disease area: Respiratory infections

Abstract

Background: Chronic Granulomatous Disease (CGD) represents a group of inherited disorders of phagocytic system, involving recurrent infections at different sites especially respiratory system. Objective: To determine the clinical spectrum of lung manifestations in CGD patients. Method: In this retrospective study, we used data from Iranian PID registry. The diagnosis was based upon WHO criteria for CGD. Results: We reviewed the records of 38 patients (26males,12 females), belonged to 33 families, 73% of which were consanguineous. The median age at the time of study was 12yrs (3mo-22yrs). The median onset age of symptoms was 4 months (1mo–12yrs), and that of diagnostic age was 5yrs (1mo-20yrs), with a diagnostic delay of 4.15 yrs,on average. Sixty three percent of our patients had lung involvement in the course of their illness, including pneumonia (18pts,75%), tuberculosis (11pts,46%), aspergillosis (3pts,12.5%), lung abscess (3pts,12.5%), and bronchiectasis (1pt,4%). Only 4 of our patients were presented with lung problems as their first manifestation. Conclusion: Lung was the second site of involvement in CGD patients; however, it is noteworthy that only in a few of our patients, it was the first manifestation of disease. So especial concern should be made to the pulmonary complications on managing this disease.

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A. Aghamohammadi, A. Farhoudi, M. Moein, M. Movahedi, N. Shahnavaz, A. Babaei Jandaghi, N. Rezaei, K. Abolmaali (Tehran, Iran). Lung manifestations of chronic granulomatous disease; a clinical survey of patients from the Iranian primary immunodeficiency registry. Eur Respir J 2001; 16: Suppl. 31, 3431

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