Peutz-Jeghers综合征29例诊治分析

Chinese Journal of Practical Surgery ›› 2009, Vol. 29 ›› Issue (08) : 665-666.

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PDF(342 KB)
Chinese Journal of Practical Surgery ›› 2009, Vol. 29 ›› Issue (08) : 665-666.
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Abstract

Diagnosis and treatment of Peutz-Jeghers syndrome DU Xiao, ZHAO Yu-pei, HE Xiao-dong, et al. Department of General Surgery, Peking Union Medical College Hospital , Peking Union Medical College , Chinese Academy of Medical Science , Beijing 100730 , China Corresponding author : ZHAO Yu-pei , E-mail: zhao8028@263.net Abstact Objective To investigate the clinical feature and treatment of Peutz-Jeghers syndrome(PJS). Methods Twenty-nine cases of PJS admitted in Peking Union Medical College Hospital from 1984 to 2008 were reviewed retrospectively and their clinical characters have been summarized. Results All the 29 patients had polyps in gastrointestinal tract, 27 patients had melanin pigmentation. Definite family history of Peutz-Jeghers syndrome were found in 10 cases (34%). Abdominal pain and hemafecia were the major clinical presentations of this syndrome. 12 cases (41%) complicated with intussusception and 11 cases (38%) with bowel obstructions. Operations were performed in 19 cases, 6 cases underwent polypectomy by endoscopy and 4 patients were given conservative therapy. 26 cases have been proven to be hamartoma of Peutz-Jeghers syndrome by pathologic examination, in which 3 cases had atypical hyperplasia. Conclusion Peutz-Jeghers syndrome has a specific clinical manifestation, and easy to complicated with bowel obstruction, intussusception and digestive tract bleeding. Surgical procedure associating with polypectomy by endoscopy is an appropriate method of treatment. Regular follow-up is indispensable to patients due to the high risk of developing malignant tumors.

Key words

Peutz-Jeghers syndrome / polyps

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