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探讨成年普拉德-威利综合征患者中不受欢迎行为的模式。

Exploring patterns of unwanted behaviours in adults with Prader-Willi syndrome.

机构信息

Psychology Laboratory, IRCCS Istituto Auxologico Italiano, Piancavallo, Italy.

出版信息

J Appl Res Intellect Disabil. 2013 Nov;26(6):568-77. doi: 10.1111/jar.12047. Epub 2013 Apr 24.

DOI:10.1111/jar.12047
PMID:23613474
Abstract

BACKGROUND

Obsessive-compulsive (O-C) traits, and excessive food intake are well known behavioural manifestations among individuals with Prader-Willi Syndrome (PWS). Other unwanted behaviours are also frequently observed, but they need a more specific investigation, especially in the adult population.

METHODS

The behaviour of 31 PWS adults was investigated via the Symptom Checklist-90-Revised (SCL-90-R), the Yale-Brown Obsessive Compulsive Scale Symptom Checklist (Y-BOCS-SC), and the Prader-Willi Behavioural Checklist (PBC). The PBC is a quick screening questionnaire prompted specifically for the investigation on adults with PWS.

RESULTS

Statistical clustering revealed two patterns of unwanted behaviours from the PBC. Behaviours belonging to the first cluster (e.g., Excessive food intake, Skin picking) appear to be linked to the usual phenotypic manifestation of PWS. By contrast, many other behaviours (e.g., some O-C symptoms and aggressive actions) could show a relationship also to individual psychopathologies.

CONCLUSIONS

Both internal (Anxiety and Depression) and external (Hostility) difficulties in managing impulses should account for individually distinct behaviours in adults with PWS.

摘要

背景

强迫症(O-C)特征和过度进食是普拉德-威利综合征(PWS)患者中众所周知的行为表现。其他不受欢迎的行为也经常被观察到,但它们需要更具体的调查,特别是在成年人群体中。

方法

通过症状清单 90 修订版(SCL-90-R)、耶鲁-布朗强迫症量表症状清单(Y-BOCS-SC)和普拉德-威利行为清单(PBC)对 31 名 PWS 成年患者的行为进行了调查。PBC 是一个快速筛查问卷,专门针对 PWS 成年患者的调查而提示。

结果

统计聚类从 PBC 中揭示了两种不受欢迎的行为模式。属于第一个聚类的行为(例如,过度进食,皮肤搔抓)似乎与 PWS 的常见表型表现有关。相比之下,许多其他行为(例如,一些 O-C 症状和攻击行为)也可能与个体的心理病理有关。

结论

管理冲动的内部(焦虑和抑郁)和外部(敌意)困难都应该解释 PWS 成年患者个体独特的行为。

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