Peutz-Jeghers sendromu

Kalıtsal - AD

Burun polipleri

Dudaklarda koyu renkli çiller

Yanak mukozasında koyu renkli çiller

Bronş polipleri

Sertoli hücreli tümör ve jinekomasti

Erkek çocuklarında erken puberte

Safra yolları polipleri

Mide-rektum arasında hamartomatöz polipler

GİS poliplerinde ağrı

İleus (intussusception)

Ovaryum kistleri

Üreter papillomları

Mesane papillomları

El derisinde hiperpigmante çiller

Café-au-lait

Demir eksikliği anemisi

Tümör riski: karsinom (GİS, meme, tiroid, akciğer, pankreas, uterus), ovaryum ve testis tümörleri

Bkz Levy-Jeghers sendromu

 

  • Brinster DR, Raper SE. Synchronous colon and pancreatic cancers in a patient with Peutz-Jeghers syndrome: report of a case and review of the literature. Surgery, 135(3):352-354, 2004

  • Hearle N, Schumacher V, Menko FH, et al. Frequency and spectrum of cancers in the Peutz-Jeghers syndrome. Clinical Cancer Research, 12(10):3209-3215, 2006

  • Kopacova M, Tacheci I, Rejchrt S, Bures J. Peutz-Jeghers syndrome: diagnostic and therapeutic approach. World Journal of Gastroenterology, 15(43):5397-5408, 2009

  • Beggs AD, Latchford AR, Vasen HF, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut, 59:975–986, 2010

  • van Lier MG, Wagner A, Mathus-Vliegen EMH, et al. High cancer risk in Peutz-Jeghers syndrome: a systematic review and surveillance recommendations. The American Journal of Gastroenterology, 105:1258–1264, 2010