Psychiatric disorders in a cohort of individuals with Prader-Willi syndrome
Psychiatric disorders in a cohort of individuals with Prader-Willi syndrome
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DOI：
10.1016/j.eurpsy.2017.03.007
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发表时间：
2017-07-01
影响因子：
7.8
通讯作者：
Benarroch, F.
中科院分区：
文献类型：
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作者：
Shriki-Tal, L.;Avrahamy, H.;Benarroch, F.
背景：普拉德-威利综合征（PWS）的精神病学表现很常见，并且往往是这些个体中最令人衰弱的问题。我们提出了一个流行病学的全国范围内的调查，在PWS人口的精神病诊断，基于全方位的精神interviews.Methods：我们研究了精神病诊断的分布（而不是一个基于ESTA的方法）在以色列国家队列的青少年和成年人与PWS。共有53例患者（32例男性），年龄在12岁及以上。所有的个人和他们的照顾者进行了采访，使用标准化的精神病学问卷。人口统计学和临床变量，临床总体印象（CGI）评分，智商，暴食症的严重程度和生活质量（QOL）也进行了评估和相关性与NPD（精神病诊断数）calculated.Results：绝大多数（89%）的研究参与者至少有一个精神病诊断。最常见的是破坏性行为障碍（DBD）（68%），强迫症（OCD）（45%）和皮肤采摘（35%）。DBD患者患强迫症和皮肤采摘的风险增加。11%的人患有精神疾病。NPD对生活质量有显著的负向影响。NPD与BMI、智商、食欲过盛严重程度、激素谱或遗传亚型之间没有相关性。结论：精神病诊断在PWS中非常常见，并且强烈影响QOL。此外，描述PWS中精神共病的特征对于规划有效的干预措施至关重要。PWS中精确的行为表型结合明确的遗传病因学可能有助于将生物学相关因素与行为联系起来的生物学研究。(C)2017年Elsevier Masson SAS。All rights reserved.
Background: Psychiatric manifestations in Prader-Willi Syndrome (PWS) are common and often are the most debilitating problem in these individuals. We present an epidemiological nation-wide survey of psychiatric diagnoses in the PWS population, based on full-range psychiatric interviews.Methods: We studied the distribution of psychiatric diagnoses (as opposed to a symptom-based approach) in the Israel national cohort of adolescents and adults with PWS. There was a total of 53 (32 males) ages 12 years and older. All individuals and their caretakers were interviewed using standardized psychiatric questionnaires. Demographic and clinical variables, Clinical Global Impression (CGI) score, IQ, severity of hyperphagia and quality of life (QOL) were also assessed and correlations with NPD (number of psychiatric diagnoses) calculated.Results: An overwhelming majority (89%) of the study participants had at least one psychiatric diagnosis. The most common were disruptive behavior disorders (DBD) (68%), obsessive compulsive disorder (OCD) (45%) and skin picking (35%). Individuals with DBD were at increased risk for OCD and skin picking. Psychotic disorders were found in 11%. NPD had a significant negative influence on QOL. There was no correlation between NPD and BMI, IQ, hyperphagia severity, hormonal profile or genetic subtypes.Conclusions: Psychiatric diagnoses are very frequent in PWS and strongly influence QOL. Furthermore, characterizing the profile of psychiatric comorbidity in PWS is crucial for planning effective interventions. Precise behavioral phenotyping in PWS in combination with a well-defined genetic etiology may aid biological research linking biological correlates to behavior. (C) 2017 Elsevier Masson SAS. All rights reserved.