Peutz-Jeghers Syndrome: A Clinical Study of a Large Family in Two Generations [Das Peutz-Jeghers-Syndrom: Klinische Studie von 2 Generationen einer gro±en Familie]

Abstract

A family group of 15 cases in two generations with Peutz-Jeghers syndrome has been studied and a review of the history and features of the syndrome is presented. This syndrome was detected in 10 of 15 family members in two generations. One family member underwent repeated surgery after small bowel obstruction was diagnosed. In addition, multiple polyps were removed from the rectum. Rectal bleeding dominated the clinical picture in two patients. Five of the family members had small bowel polyps, five of the siblings had polyps in the colon. Colonic polyp was also found in one patient who had a small bowel polyp. Four of the ten affected family members in two generations had mucocutaneous pigmentation. One of the siblings had died during the early postoperative period. © 1986, Georg Thieme Verlag KG Stuttgart, New York. All rights reserved.