[Autoimmune enteropathy causing protracted diarrhea].
A 3-month old female infant was transferred from another hospital where she had been hospitalized from the age of 1 month for protracted secretory diarrhea. The diarrhea had begun at birth and was unresponsive to various therapeutic formulas and to total parenteral nutrition (TPN). The parents were consanguineous. There were 6 normal siblings, while 3 siblings had died in infancy, including a sister who had succumbed to protracted diarrhea at the age of 6 months. In our patient duodenal biopsy showed flattening of villi and proliferation of mononuclear cells in the lamina propria. Specific circulating IgG antibodies against gut epithelium were found, as well as thyroglobulin antibodies. Repeated trials of oral feeding were unsuccessful and TPN was required for 8 months. Complications included septicemia, osteomyelitis and acute renal failure. Therapeutic trials with intravenous hydrocortisone, zinc sulphate and metronidazole were unsuccessful and the infant died at the age of 11 months. Intestinal tissue taken postmortem showed nearly absolute flattening of intestinal villi. This is the first report in Israel of intractable infantile diarrhea due to autoantibodies to intestinal epithelium.
Naveh
Y
Y
Dept. of Pediatrics B, Rambam Medical Center, Haifa.
Ben-Arieh
Y
Y
Greif
Z
Z
Berant
M
M
D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases; D003968:Diarrhea, Infantile / Q000209:etiology* / Q000276:immunology; D004848:Epithelium / Q000276:immunology; D005260:Female; D006801:Humans; D007223:Infant; D007410:Intestinal Diseases / Q000150:complications* / Q000276:immunology; D007422:Intestines / Q000276:immunology
D001323:Autoantibodies