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法布里病患者的心理方面。

Psychological aspects of patients with Fabry disease.

机构信息

Pacific Graduate School of Psychology at Palo Alto University, 1791 Arastradero Road, Palo Alto, CA, 94304, USA.

University of California, San Francisco, CA, USA.

出版信息

J Inherit Metab Dis. 2009 Dec;32(6):745-753. doi: 10.1007/s10545-009-1254-1. Epub 2009 Oct 31.

DOI:10.1007/s10545-009-1254-1
PMID:19924564
Abstract

The Minnesota Muliphasic Personality Inventory (MMPI-2) is widely used in chronic illness and chronic pain populations to assess psychological functioning. We report the results of the first investigation using the MMPI-2 to assess psychological aspects of patients with Fabry disease. Fabry disease, an X-linked lysosomal storage disorder, is a multisystem progressive disease affecting the kidney, heart, and central nervous system, and is particularly associated with chronic symptoms including pain. In this study, 28 patients with Fabry disease completed the MMPI-2 and a background questionnaire. Fabry disease patients scored significantly higher than the MMPI-2 normative sample on seven clinical scales (Hs, D, Hy, Pd, Pa, Pt, Sc) and two validity scales (L, F). Individuals with elevated scores on the Hs, D, and Hy scales tend to have somatic complaints, sadness, and emotional distress. Under stress, they may experience an increase in physical symptoms. Elevated Pd, Pa, Pt, and Sc scales suggest social maladjustment, suspiciousness, and feelings of isolation. An elevated L scale suggests defensiveness; a high score on F suggests emotional turmoil. When compared with cohorts of patients with Gaucher disease (GD), chronic heart disease (CRHD), and chronic pain, the Fabry disease patients had significantly higher scores than GD patients and CRHD patients on numerous clinical (Hs, D, Si), and validity (F) scales underscoring the relative amount of suffering and pain experienced by Fabry disease patients. No significant differences on any MMPI-2 scales were found between the Fabry disease patients and the pain patients, suggesting that Fabry disease patients may be comparable to pain patient populations.

摘要

明尼苏达多项人格测验(MMPI-2)广泛应用于慢性疾病和慢性疼痛人群,以评估心理功能。我们报告了首次使用 MMPI-2 评估法布里病患者心理方面的研究结果。法布里病是一种 X 连锁溶酶体贮积症,是一种多系统进行性疾病,影响肾脏、心脏和中枢神经系统,特别与慢性症状有关,包括疼痛。在这项研究中,28 名法布里病患者完成了 MMPI-2 和背景问卷。法布里病患者在七个临床量表(Hs、D、Hy、Pd、Pa、Pt、Sc)和两个效度量表(L、F)上的得分显著高于 MMPI-2 常模样本。Hs、D 和 Hy 量表得分较高的个体往往有躯体抱怨、悲伤和情绪困扰。在压力下,他们可能会出现更多的身体症状。Pd、Pa、Pt 和 Sc 量表得分较高表明社会适应不良、多疑和孤立感。L 量表得分升高表明防御性;F 量表得分高表明情绪波动。与戈谢病(GD)、慢性心脏病(CRHD)和慢性疼痛患者队列相比,法布里病患者在多个临床量表(Hs、D、Si)和效度量表(F)上的得分显著高于 GD 患者和 CRHD 患者,突出了法布里病患者所经历的相对痛苦和疼痛量。在任何 MMPI-2 量表上,法布里病患者与疼痛患者之间均无显著差异,这表明法布里病患者可能与疼痛患者群体相当。

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Mol Genet Metab. 2008 Feb;93(2):112-28. doi: 10.1016/j.ymgme.2007.09.013. Epub 2007 Nov 26.
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Depression in adults with Fabry disease: a common and under-diagnosed problem.法布里病成年患者的抑郁症:一个常见且诊断不足的问题。
J Inherit Metab Dis. 2007 Nov;30(6):943-51. doi: 10.1007/s10545-007-0708-6. Epub 2007 Nov 12.
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Int J Mol Sci. 2023 Mar 9;24(6):5246. doi: 10.3390/ijms24065246.
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Mol Genet Metab Rep. 2022 Aug 10;32:100899. doi: 10.1016/j.ymgmr.2022.100899. eCollection 2022 Sep.
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