Immunoproliferative Small Intestinal Disease

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Abstract
To the Editor: Khojasteh and colleagues described immunoproliferative small intestinal disease (IPSID) as a "Third-World lesion" (June 9 issue).1 We would like to share our experience with two cases of IPSID that occurred in North American children with long-standing immunodeficiency.Patient 1 had recurrent bacterial infections due to a defect in the alternative pathway of complement. Weight loss and diarrhea developed when she was 11 months old. A jejunal biopsy showed a lymphoplasmacytic infiltration into the lamina propria. Laparotomy was undertaken seven years later when the patient had an elevated IgA level and ascites. Massive mesenteric adenopathy was seen. Microscopical . . .