AUTOIMMUNE ENTEROPATHY IN SCHIMKE IMMUNOOSSEOUS DYSPLASIA

The clinical phenotype of Schimke immunoosseous dysplasia (SID) is cha racterized by growth retardation, renal failure, recurrent infections, cerebral infarcts, and skin pigmentation beginning in childhood. We r eport here on a 4-year-old male child who had all characteristic sympt oms of SID, and, in addition, vomiting and prolonged diarrhea, The stu dy results suggest that malabsorption, demonstrated as increased serum immunoglobulin A anti-gliadin antibody, steatorrhea and partial villo us atrophy of the jejunal small bowel, is a previously unrecognized fe ature of SID. (C) 1998 Wiley-Liss, Inc.