[Chronic granulomatous disease in childhood].
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.
Hannachi Sassi
Samia
S
Service d'anatomie et de cytologie pathologiques, institut Salah-Azaiez, Bab Saadoun, Tunis-1006, Tunisie. samia.hannachi@rns.tn
Fitouri
Zohra
Z
Braham
Emna
E
Mrad
Karima
K
Mattousi
Nadia
N
Abbes
Imen
I
Dhouib
Rym
R
Ben Becher
Saida
S
Ben Romdhane
Khaled
K
D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D002648:Child; D003106:Colon / Q000473:pathology; D003967:Diarrhea / Q000209:etiology; D004804:Eosinophils / Q000473:pathology; D006105:Granulomatous Disease, Chronic / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007249:Inflammation / Q000209:etiology / Q000473:pathology; D007413:Intestinal Mucosa / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D016896:Treatment Outcome
D000900:Anti-Bacterial Agents