Progressive esophageal dysfunction in chronic granulomatous disease.
Chronic granulomatous disease of childhood (CGD), a hereditary disorder of neutrophil function, affects the gastrointestinal tract in a variety of ways. Esophageal involvement has only rarely been reported. An 11-year-old boy with CGD and progressive esophageal dysmotility is described. Repeated radiographic, endoscopic, and motility studies revealed a markedly atonic esophagus with varying function of the lower esophageal sphincter. Pharmacologic therapy and esophageal dilatations were unsuccessful in establishing adequate esophageal function. A feeding gastrostomy was required for nutritional support.
D002648:Child; D004935:Esophageal Diseases / Q000209:etiology*; D004943:Esophagogastric Junction / Q000503:physiopathology; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D006801:Humans; D008297:Male; D011312:Pressure