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Netherton 综合征；儿童和成年患者及其父母的神经心理学和社会心理功能。

Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents.

机构信息

Erasmus University Medical Center-Sophia Children's Hospital, The Netherlands.

University of Amsterdam, The Netherlands.

出版信息

J Health Psychol. 2020 Nov-Dec;25(13-14):2296-2316. doi: 10.1177/1359105318790052. Epub 2018 Aug 21.

DOI:10.1177/1359105318790052
PMID:30129381
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7583452/
Abstract

BACKGROUND

Netherton syndrome is a rare severe skin disease. Clinical experience showed considerable psychosocial burdens among Netherton syndrome patients/families. Their (neuro)psychological functioning has never been investigated.

OBJECTIVE

To investigate neuropsychological/psychosocial functioning of Netherton syndrome patients and parents.

METHODS

A total of 12 Netherton syndrome patients and/or parents completed neuropsychological tests, semi-structured-interviews, and psychological-questionnaires.

RESULTS

Intelligence results showed disharmonic profiles, with below-average scores on processing speed. Neuropsychological problems and unfavorable outcomes on health-related quality of life, illness-appearance-related problems, and negative social consequences among patients/parents were found. Psychopathological (emotional) problems were reported; stigmatization, bullying was common among Netherton syndrome patients.

CONCLUSION

Compared with normative data, Netherton syndrome patients showed neuropsychological and psychosocial problems. Standard follow-up is necessary to identify problems at early stage.

摘要

背景

Netherton 综合征是一种罕见的严重皮肤病。临床经验表明，Netherton 综合征患者/家庭存在相当大的心理社会负担。他们的（神经）心理功能从未被调查过。

目的

调查 Netherton 综合征患者及其父母的神经心理/心理社会功能。

方法

共有 12 名 Netherton 综合征患者和/或父母完成了神经心理学测试、半结构化访谈和心理问卷。

结果

智力测试结果显示，患者存在不平衡的表现，在处理速度方面得分低于平均水平。患者/父母存在与健康相关的生活质量、疾病外貌相关问题和负面社会后果相关的神经心理问题和不良结果。报告了精神病理（情绪）问题；Netherton 综合征患者中常见的污名化和欺凌现象。

结论

与常模数据相比，Netherton 综合征患者存在神经心理和心理社会问题。需要进行标准随访以早期识别问题。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df72/7583452/573c95602fcd/10.1177_1359105318790052-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df72/7583452/67d66c86033f/10.1177_1359105318790052-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df72/7583452/5ea219ec038a/10.1177_1359105318790052-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df72/7583452/877abfe92df5/10.1177_1359105318790052-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df72/7583452/573c95602fcd/10.1177_1359105318790052-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df72/7583452/67d66c86033f/10.1177_1359105318790052-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df72/7583452/5ea219ec038a/10.1177_1359105318790052-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df72/7583452/877abfe92df5/10.1177_1359105318790052-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/df72/7583452/573c95602fcd/10.1177_1359105318790052-fig4.jpg

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