普拉德-威利综合征患者行胆胰转流术的结果。

Outcome of biliopancreatic diversion in subjects with Prader-Willi Syndrome.

作者信息

Marinari G M, Camerini G, Novelli G B, Papadia F, Murelli F, Marini P, Adami G F, Scopinaro N

机构信息

Semeiotica Chirurgica R, University of Genoa School of Medicine, Largo Rosanna Benzi 8, 16132 Genova, Italy.

出版信息

Obes Surg. 2001 Aug;11(4):491-5. doi: 10.1381/096089201321209404.

DOI:10.1381/096089201321209404

PMID:11501362

Abstract

BACKGROUND

In Prader-Willi Syndrome (PWS), mental retardation and compulsive hyperphagia cause early obesity, the co-morbidities of which lead to short life-expectancy, with death usually occurring in their 20s. Long-term weight loss is mandatory to lengthen the survival; therefore, the lack of compliance in voluntary food restriction requires a surgical malabsorptive approach.

METHODS

15 PWS subjects were submitted to biliopancreatic diversion (BPD) and followed (100%) for a mean period of 8.5 (4-13) years. BPD consists of a distal gastrectomy with a long Roux-en-Y reconstruction which, by delaying the meeting between food and biliopancreatic juices, causes an intestinal malabsorption. Indication for BPD was BMI > 40 or > 35 with metabolic complications. Preoperative mean age was 21 +/- 5 years, mean weight 127 +/- 26 kg, and mean Body Mass Index (BMI, kg/m2) 53 +/- 10. According to Holm's criteria, all of the subjects had a total score > or = 8. IQ assessment was performed in each subject, with a mean score of 72 +/- 10. An arbitrary lifestyle score was given to each subject.

RESULTS

No perioperative complications were observed. Percent excess weight loss (%EWL) was 59 +/- 15 at 2 years and 56 +/- 16 at 3 years, and then progressive regain occurred; at 5 years %EWL was 46 +/- 22 and at 10 years 40 +/- 27. Spearman rank test failed to demonstrate any correlation between weight loss at 5 years and patient data, except with lifestyle score (Spearman r = 0.8548, p < .0001). Current mean age is 31 +/- 7 years.

CONCLUSION

BPD has to be considered for its value in prolonging and qualitatively improving the PWS patient's life.

摘要

背景

在普拉德-威利综合征（PWS）中，智力发育迟缓与强迫性贪食导致早期肥胖，其合并症会导致预期寿命缩短，通常在20多岁时死亡。长期体重减轻对于延长生存期至关重要；因此，由于自愿限制饮食的依从性不足，需要采取手术吸收不良的方法。

方法

15名PWS患者接受了胆胰分流术（BPD），并进行了（100%）平均8.5（4 - 13）年的随访。BPD包括远端胃切除术及长袢Roux-en-Y重建术，通过延迟食物与胆胰液的混合，导致肠道吸收不良。BPD的适应症为BMI > 40或> 35且伴有代谢并发症。术前平均年龄为21±5岁，平均体重127±26 kg，平均体重指数（BMI，kg/m²）为53±10。根据霍尔姆标准，所有患者的总分≥8分。对每位患者进行了智商评估，平均分为72±10分。为每位患者给出了一个任意的生活方式评分。

结果

未观察到围手术期并发症。2年时超重体重减轻百分比（%EWL）为59±15，3年时为56±16，随后体重逐渐反弹；5年时%EWL为46±22，10年时为40±27。Spearman秩检验未能证明5年时的体重减轻与患者数据之间存在任何相关性，除了与生活方式评分相关（Spearman r = 0.8548，p <.0001）。目前平均年龄为31±7岁。