AUTOIMMUNE ENTEROPATHY IN INFANTS - PATHOLOGICAL-STUDY OF THE DISEASEIN 2 FAMILIAL CASES

In two brothers with autoimmune enteropathy there was total villous at rophy in the small intestine and marked lymphoid cell infiltration in the lamina propria of the entire digestive tract, discovered at autops y in one of these patients. In addition, the pancreas showed diffuse i nterstitial infiltration by lymphocytes. The liver was enlarged, with extensive haematopoiesis and cholestasis. Similar lesions in the diges tive tract were noticed in the second boy, but on immunosuppressive th erapy his diarrhoea gradually disappeared. When he was 16 months of ag e, percutaneous biopsies showed moderately aggressive chronic hepatiti s and a focal interstitial lymphoid infiltrate in the kidney. After 3 years of immunosuppressive therapy (prednisone, cyclosporin), the chil d ate well and total parenteral nutrition was discontinued. The intest inal lesions had regressed but fibrotic lesions of the liver persisted .