PMID: 2123245Oct 1, 1990Paper

Congenital short gut, malrotation, and dysmotility of the small bowel

Journal of Pediatric Gastroenterology and Nutrition
I B KernT Bohane

Abstract

A familial syndrome of congenital short small bowel, malrotation, and functional bowel obstruction is presented and illustrated in three male siblings with one long-term survivor. Including the present three cases there have been 18 similar cases found in the literature. This disorder appears to be a sex-linked recessive inheritance. Early barium meal and follow through in siblings of patients with this condition will detect malrotation that should be surgically corrected in order to prevent mid-gut volvulus. Intravenous alimentation and early introduction of enteral feeding is important, not only to maintain nutrition but to encourage adaptation of the associated short and dysmotile bowel. These measures aim to improve the outcome of this frequently, but not invariably, fatal disorder.

Citations

Oct 6, 2012·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Christine S van der WerfRobert M W Hofstra
Jan 1, 1997·Journal of Pediatric Gastroenterology and Nutrition·C D RudolphJ A Vanderhoof
May 19, 2006·Journal of Pediatric Gastroenterology and Nutrition·Paulina OrdonezEdward J Hoffenberg
Oct 10, 2006·Genetics·Karl W BromanGary A Churchill
Mar 7, 2013·Development·Michael K Dush, Nanette M Nascone-Yoder
Feb 7, 2016·Seminars in Cell & Developmental Biology·Mandy WombleNanette Nascone-Yoder
Oct 1, 2010·Neurogastroenterology and Motility : the Official Journal of the European Gastrointestinal Motility Society·L GalmicheA Rötig
Aug 19, 2015·Biochimica Et Biophysica Acta·Christine S van der WerfRobert M W Hofstra
Aug 25, 2015·BMC Developmental Biology·Jelly Hm SoffersWouter H Lamers
Oct 11, 2016·Clinics and Research in Hepatology and Gastroenterology·Lucile GonnaudHugues Patural
Mar 24, 2005·Annales de pathologie·Françoise BomanFrançois Paraf

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