Delayed Gastric Emptying in Patients with Prader Willi Syndrome

作者：Arenz T; Schwarzer A; Pfluger Th; Koletzko S; Schmidt H^*

来源：Journal of Pediatric Endocrinology & Metabolism, 2010, 23(9): 867-871.

DOI：10.1515/jpem.2010.140

• Prader-Willi Syndrome
• gastric emptying
• gastric rupture

Background: A 15 year old girl with Prader-Willi Syndrome (PWS) died of gastric rupture. Systematic literature research revealed seven case reports of PWS patients with acute gastric dilatation, two had a lethal course. The objective of this study was to determine if delayed gastric emptying in PWS patients might contribute to gastric dilatation.
Methods: Gastric emptying was measured in eight patients with PWS by nucleotid scintigraphy after a standardized test meal.
Results: Median age was 17.8 years (range 10.1-19.5). Median BMI of the male patients was 29.5 (range 18.4-34.8), of the female patients 28 (range 20.0-44.8). Half time of gastric emptying was delayed in five of the eight patients (median 78.5 minutes, range 59-134).
Conclusion: Scintigraphic measurement of gastric emptying in eight PWS patients revealed delay in comparison to normal values. This might be a risk factor for gastric dilatation and rupture in patients with PWS.

• 出版日期2010-9