Chronic granulomatous disease

Chronic granulomatous disease is a rare group of inherited disorders characterized by impaired phagocyte oxidative metabolism caused by missing components or subunits of the NADPH oxidative complex, resulting in defective intracel-lular killing of catalase-positive micro-organisms. The most common form of chronic granulomatous disease is inherited as an X-linked recessive trait, although autosomal-type mutations have been described. The disease usually appears during the first two years of life with symptoms and signs of recurrent pyogenic infections, although milder forms of the disease have been described, with onset occurring in adulthood. Characteristic granulomas may develop in any organ system, including the skin, lungs, genitourinary, bone and reticoloendothelial systems. Gastrointestinal involvement has commonly been described. Narrowing of the gastric antrum, with signs of gastric outlet obstruction, such as vomiting, weight loss and epigastric pain, is a rare but distinctive feature of chronic granulomatous disease.52 Endoscopic features are usually not specific, whereas histology shows mild edema of the lamina propria, chronic inflammatory cells and granulomas characterized by phagocytes, giant cells and lipid-containing histiocytes. Upper gastrointestinal series reveal gastric rugae hypertrophy and narrowing of the antral lumen. Recent developments of nuclear medicine studies have increased the capability of evaluating thickness and inflammatory involvement of the gastric wall.

Eosinophilic gastritis 105

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