Peutz-Jeghers syndrome: is family screening needed?

Citation
G. Baumgartner et al., Peutz-Jeghers syndrome: is family screening needed?, PEDIAT SURG, 16(5-6), 2000, pp. 437-439
Citations number
12
Categorie Soggetti
Pediatrics
Journal title
PEDIATRIC SURGERY INTERNATIONAL
ISSN journal
01790358 → ACNP
Volume
16
Issue
5-6
Year of publication
2000
Pages
437 - 439
Database
ISI
SICI code
0179-0358(200007)16:5-6<437:PSIFSN>2.0.ZU;2-6
Abstract
In a 7-year-old boy, a small-bowel polyp was found intraoperatively as a le ad point of an intussusception. Histologically, a hamartoma was found and t he clinical work-up revealed Peutz-Jeghers syndrome (PJS). Additionally, al l four asymptomatic siblings showed intestinal polyposis. All children in a family with PJS should be properly investigated. In case of an intussuscep tion with a polyp in a critical location, a surgical procedure should follo w.