Alexander R C, Greenswag L R, Nowak A J
Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City 52242.
Am J Med Genet. 1987 Dec;28(4):889-95. doi: 10.1002/ajmg.1320280414.
Inability to vomit has been cited as characteristic of Prader-Willi syndrome (PWS). Although post-prandial vomiting after gastric by-pass surgery has been reported, neither vomiting under "typical" circumstances or rumination have been described. Prompted by the discovery of several cases of vomiting and rumination, a questionnaire was sent to members of the PWS Association. Approximately 36% (113/313) of affected individuals reportedly experienced at least one episode of vomiting. Induced vomiting was unsuccessful in 9 of 14 cases in whom results were known. However, no complications of Ipecac were reported. We suggest that there is an alteration in the physiologic set-point at which vomiting occurs, leading to a decreased propensity to vomit. Liberal and strict definitions of rumination yielded 15.7% and 10.2% positive responses, respectively. Rumination was associated with a history of vomiting. Enamel deterioration consistent with rumination has been observed, and such changes should be looked for in all PWS children. In several instances, rumination was found to decrease when very strict weight control was lessened. Certain individuals may ruminate under too strict a weight control program, and weight control goals should be evaluated to achieve a reasonable compromise between ideal weight and obesity. Vomiting and rumination do not rule out the diagnosis of PWS.
无法呕吐被认为是普拉德-威利综合征(PWS)的特征。尽管有报道称胃旁路手术后会出现餐后呕吐,但尚未描述“典型”情况下的呕吐或反刍现象。在发现几例呕吐和反刍病例后,向PWS协会成员发送了一份问卷。据报道,约36%(113/313)的受影响个体至少经历过一次呕吐发作。在已知结果的14例病例中,有9例催吐未成功。然而,未报告吐根糖浆的并发症。我们认为,呕吐发生的生理设定点存在改变,导致呕吐倾向降低。对反刍的宽泛和严格定义分别产生了15.7%和10.2%的阳性反应。反刍与呕吐史有关。已观察到与反刍一致的牙釉质恶化,所有PWS儿童都应检查是否有此类变化。在一些情况下,当非常严格的体重控制放松时,反刍现象会减少。某些个体可能在过于严格的体重控制方案下出现反刍,应评估体重控制目标,以在理想体重和肥胖之间达成合理的平衡。呕吐和反刍并不排除PWS的诊断。