Clinical characteristics and basic research development of Peutz-Jeghers syndrome.
OBJECTIVE
To improve clinical knowledge of Peutz-Jeghers syndrome.


METHODS
Eight patients with Peutz-Jeghers syndrome from 1984 to 1998 in our hospital were retrospectively reviewed and analyzed in the present study.


RESULTS
The result of this analysis showed that there were 4 patients appeared with family histories of Peutz-Jeghers syndrome. All of the included patients admitted to the hospital with various complications, and eventually received surgical interventions for these complications, among which, 6 of them had intestinal obstructions mostly (5/6) due to small bowel intussusception, and 2 of them suffered with hemafecia. Post-operative recoveries were generally satisfactory with zero mortality.


CONCLUSIONS
Peutz-Jeghers syndrome is an uncommon digestive dominant hereditary disease. The diagnosis of it with history, symptoms, signs, and proper examinations usually is not difficult Surgical interventions are necessary once complications occur.
He
X
X
Department of Surgery, Peking Union Medical College Hospital, CAMS & PUMC, Beijing 100730.
Mao
Y
Y
Ben
Y
Y
Ma
C
C
Zhang
Z
Z
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D007443:Intussusception / Q000209:etiology / Q000601:surgery; D008297:Male; D009780:Occult Blood; D010375:Pedigree; D010580:Peutz-Jeghers Syndrome / Q000150:complications / Q000235:genetics / Q000601:surgery; D012189:Retrospective Studies