Familial visceral myopathy with pseudo-obstruction, megaduodenum, Barrett's esophagus, and cardiac abnormalities

MUNGAN Z., AKYUZ F., Bugra Z. , YONAL O., OZTURK S. , ACAR A., ...Daha Fazla

AMERICAN JOURNAL OF GASTROENTEROLOGY, cilt.98, ss.2556-2560, 2003 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 98 Konu: 11
  • Basım Tarihi: 2003
  • Doi Numarası: 10.1016/j.amjgastroenterol.2003.08.013
  • Sayfa Sayıları: ss.2556-2560


This report describes a new subgroup of familial visceral myopathy. Three patients from within this family were admitted to the hospital with pseudo-obstruction. Barium x-ray, abdominal plain film, esophageal manometry, colonoscopy, gastroscopy, and echocardiography were performed in all siblings for diagnostic evaluation. Two of our patients had surgery because of suspicion of acute abdomen. In one of them, full-thickness biopsy, which was performed during laparotomy, revealed findings that were compatible with familial visceral myopathy. Three siblings from this family with visceral myopathy, in which the parents were consanguineous, had megaduodenum, long-segment Barrett's esophagus, and different cardiac abnormalities. (C) 2003 by Am. Coll. of Gastroenterology.