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INTESTINAL AGANGLIONOSIS IN THE SMITH-LEMLI-OPITZ SYNDROME |
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| Authors: | J. &# I&# KA,J. MARE&# OVÁ ,Z. KEREKE&# ,Z. NO&# I&# KA,V. JÜ TTNEROVÁ ,P. BALÍ CEK |
| Affiliation: | Departments of Medical Genetics;Paediatrics;Pathology, University Hospital, Hradec Králové, Czechoslovakia |
| Abstract: | ABSTRACT. Two unrelated cases with clinical and autopsy findings of the Smith-Lemli-Opitz syndrome are described. Narrowing of the terminal ileum and congenital intestinal aganglionosis was found in both. This is a rare association and the importance of microscopic examination of the intestine in cases of the Smith-Lemli-Opitz syndrome is emphasized. |
| Keywords: | Intestinal aganglionosis Smith-Lemli-Opitz syndrome narrowing of terminal ileum |