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Steatorrhea and Absent Gastric Antral Motility in Three Female Siblings with Familial Deafness.

A. Groll, M.R.C.P.; and B. I. Hirschowitz, M.D., F.A.C.P.
Ann Intern Med. 1968;68(5):1147-1148. doi:10.7326/0003-4819-68-5-1147_3
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The last three of six siblings were all affected with familial eighth nerve deafness at age 9, 3, and 13 years. Also, all three have been affected with an additional syndrome presenting at ages 14 to 15 with malnutrition, diarrhea, and vomiting from gastric retention (24 to 72 hr) due to absent antral motility, poorly relieved in the two surviving sisters by drainage procedures without vagotomy. All had normal gastric and pancreatic secretion studies, small-bowel X rays, duodenal biopsies, and lipoprotein and amino acid studies. Severe malnutrition with edema in the two older siblings was related to marked steatorrhea and


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