Department of Child and Adolescent Psychiatry, Saarland University Hospital, Homburg, Germany.
BJU Int. 2010 Dec;106(11):1758-62. doi: 10.1111/j.1464-410X.2010.09457.x.
To assess and identify the frequency and type of urinary incontinence (UI), as well as associated symptoms in persons with Prader-Willi syndrome (PWS). PWS is characterized by mental retardation, short stature, obesity and hypogonadism. The behavioural phenotype includes eating problems, temper outbursts, affective disorders, stereotypies and speech abnormalities. UI is common in children with mental retardation in general, but has not been reported systematically in children with PWS so far.
The Dutch version of the 'Parental Questionnaire: Enuresis/Urinary Incontinence' was completed by 118 parents of children with PWS. This questionnaire includes items referring to day- and night-time wetting, toilet habits, observable voiding behaviours and reactions, urinary tract infections, stool habits and behavioural symptoms.
The rate of nocturnal enuresis in persons with PWS was 13.6% (16) at a mean age of 15.1 years. 3.8% (5) had additional daytime urinary incontinence, and 3.3% (4) had faecal incontinence. Lower urinary tract symptoms were commonly indicative of overactive bladder, dysfunctional voiding and postponement. Also, the rate of internalizing and externalizing behavioural problems was high.
Urinary incontinence is more common in persons with PWS than in typically developing children, adolescents and adults. As lower urinary tract symptoms are common, detailed assessment and specific treatment of UI should be part of the care of all persons with PWS.
评估和识别普瑞德-威利综合征（PWS）患者的尿失禁（UI）的频率和类型，以及相关症状。PWS 的特征为智力低下、身材矮小、肥胖和性腺功能减退。行为表型包括饮食问题、脾气发作、情感障碍、刻板行为和言语异常。UI 通常在一般智力低下的儿童中很常见，但迄今为止尚未在 PWS 儿童中系统报告。
118 名 PWS 儿童的家长完成了荷兰版的“家长问卷：遗尿/尿失禁”。该问卷包括日间和夜间湿、厕所习惯、可观察的排尿行为和反应、尿路感染、排便习惯和行为症状的项目。
15.1 岁时，PWS 患者的夜间遗尿率为 13.6%（16 人）。3.8%（5 人）有额外的日间尿失禁，3.3%（4 人）有粪便失禁。下尿路症状通常提示膀胱过度活动、排尿功能障碍和延迟。此外，内化和外化行为问题的发生率也很高。
尿失禁在 PWS 患者中比在典型发育的儿童、青少年和成人中更为常见。由于下尿路症状很常见，因此 UI 的详细评估和特定治疗应成为所有 PWS 患者护理的一部分。
