Unresponsive enteropathy associated with circulating enterocyte autoantibodies in a boy with common variable hypogammaglobulinemia and type I diabetes.
A 13-year-old boy with common variable hypogammaglobulinemia and type I diabetes developed a severe enteropathy that proved to be unresponsive to any treatment, including total parenteral nutrition. No evidence of known etiologies of malabsorption and/or secretory diarrhea was found in this subject. A high titer of complement-fixing enterocyte autoantibody was persistently found in the patient's serum. These features suggest that the enteropathy of this primarily immunodeficient subject had an autoimmune origin. A cycle of cyclophosphamide failed to show any amelioration of the diarrhea or a significant decrease in the autoantibody titer.
D000293:Adolescent; D000361:Agammaglobulinemia / Q000276:immunology*; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000276:immunology*; D001706:Biopsy; D003922:Diabetes Mellitus, Type 1 / Q000276:immunology*; D003967:Diarrhea / Q000276:immunology; D005455:Fluorescent Antibody Technique; D006801:Humans; D007579:Jejunal Diseases / Q000276:immunology* / Q000473:pathology; D007583:Jejunum / Q000473:pathology; D008297:Male; D008855:Microscopy, Electron, Scanning; D010289:Parenteral Nutrition, Total
D001323:Autoantibodies