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采用胆胰转流十二指肠转位术治疗普拉德-威利综合征相关肥胖。病例报告及文献综述。

Prader-Willi syndrome-associated obesity treated by biliopancreatic diversion with duodenal switch. Case report and literature review.

作者信息

Papavramidis Spiros T, Kotidis Efstathios V, Gamvros Orestis

机构信息

Aristotle University of Thessaloniki, AHEPA University Hospital, Third Department of Surgery, Thessaloniki, 546 36, Macedonia, Greece.

出版信息

J Pediatr Surg. 2006 Jun;41(6):1153-8. doi: 10.1016/j.jpedsurg.2006.01.065.

DOI:10.1016/j.jpedsurg.2006.01.065
PMID:16769352
Abstract

BACKGROUND

Prader-Willi syndrome (PWS) is a congenital chromosomal disorder characterized by compulsive hyperphagia and the early development of obesity. Obesity is identified as the main cause of morbidity and mortality in PWS individuals. Thus, body weight reduction is of major importance for a prolonged survival. PATIENT-METHOD: A 20-year-old female patient with PWS was referred to our department for surgical treatment of her obesity. At admission, her body weight was 153 kg, and her body mass index (BMI) was 74.33 kg/m(2). The patient underwent biliopancreatic diversion with duodenal switch, as well as cholecystectomy and appendicectomy. The volume of the gastric remnant was 100 mL, and the lengths of the gastric and common limbs were 250 and 60 cm, respectively.

RESULTS

Eighteen months after the operation, the patient lost 63 kg with no considerable changes in her eating habits. Her sleep disturbances and sleep apnea disappeared, and her social life dramatically improved.

CONCLUSIONS

Biliopancreatic diversion with duodenal switch seems to be a good method for the treatment of PWS-associated obesity because it offers good results in weight loss without the need for revision, good quality of life, and a chance for a prolonged survival.

摘要

背景

普拉德-威利综合征(PWS)是一种先天性染色体疾病,其特征为强迫性多食和肥胖的早期发展。肥胖被认为是PWS患者发病和死亡的主要原因。因此,减轻体重对于延长生存期至关重要。

患者方法

一名20岁的PWS女性患者因肥胖接受手术治疗被转诊至我科。入院时,她的体重为153千克,体重指数(BMI)为74.33千克/平方米。该患者接受了十二指肠转位的胆胰分流术,以及胆囊切除术和阑尾切除术。胃残余容积为100毫升,胃和共同肢体的长度分别为250厘米和60厘米。

结果

术后18个月,患者体重减轻了63千克,饮食习惯无明显变化。她的睡眠障碍和睡眠呼吸暂停消失,社交生活显著改善。

结论

十二指肠转位的胆胰分流术似乎是治疗PWS相关肥胖的一种好方法,因为它在无需翻修的情况下减重效果良好,生活质量高,并有延长生存期的机会。

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Update on Diabetes Mellitus and Glucose Metabolism Alterations in Prader-Willi Syndrome.
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