Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
4128982 | Annales de Pathologie | 2008 | 4 Pages |
Abstract
Chronic granulomatous disease (GCD) of childhood is a rare inherited immunodeficiency. It is characterized clinically by the occurrence of severe and recurrent uncontrollable infections, which often lead to death in early childhood. The underlying biologic anomaly is a defective microbicidal capacity of phagocytosis with abnormal oxidative response during phagocytosis. Histologically, the GCD is characterized by a spectrum of histopathological features in a wide range of tissue specimens, often demonstrating features of active chronic inflammation, with or without non-caseating granuloma formation. The presence of numerous pigmented macrophages in association with such an inflammation should raise suspicion of the diagnosis. We report a case of a GCD in an 11-year-old boy and study the anatomoclinic features of this rare entity.
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Authors
Samia Hannachi Sassi, Zohra Fitouri, Emna Braham, Karima Mrad, Nadia Mattousi, Imen Abbes, Rym Dhouib, Saida Ben Becher, Khaled Ben Romdhane,