Peutz-Jeghers' syndrome--juvenile intestinal polyposis--review of five cases.
During the past 30 years, authors observed and followed 5 patients with Peutz-Jeghers' syndrome. Four of them had diffuse polyposis of stomach, small bowel and colon. They also had severe clinical presentation of the disease, with recurring colicky pain, haemorrhage, anaemia and intussusception, all of which necessitated frequent surgical treatment. Excised polyps presented as benign hamartomas, without malignant alteration. Mucocutaneous pigmentations were present in 4 patients. Family history was revealing in only 2 cases. One patient, a girl aged 2 years, died due to the complications of the surgical intervention (intestinal obstruction). She has had the most severe form of the disease with diffuse polyposis.
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007414:Intestinal Neoplasms / Q000235:genetics* / Q000601:surgery; D007443:Intussusception / Q000235:genetics; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D010375:Pedigree; D010580:Peutz-Jeghers Syndrome / Q000235:genetics* / Q000601:surgery; D012086:Reoperation; D013274:Stomach Neoplasms / Q000235:genetics