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戈谢病患者的心理并发症。

Psychological complications of patients with Gaucher disease.

作者信息

Packman W, Wilson Crosbie T, Riesner A, Fairley C, Packman S

机构信息

Pacific Graduate School of Psychology, 935 E. Meadow Drive, Palo Alto, CA 94303, USA.

出版信息

J Inherit Metab Dis. 2006 Feb;29(1):99-105. doi: 10.1007/s10545-006-0154-x.

DOI:10.1007/s10545-006-0154-x
PMID:16601875
Abstract

The Minnesota Multiphasic Personality Inventory (MMPI-2) is commonly used in chronic illness and chronic pain populations to assess psychological functioning. We report the results of the first study employing the MMPI-2 to assess psychological aspects of patients with Gaucher disease, type I (GD) is an inborn error of metabolism with unique features as a chronic illness: the disorder often presents with mild symptoms, and is frequently diagnosed in later childhood or adulthood; the treatment is highly efficacious, but, that same treatment is intrusive and expensive and requires that patients restructure their work and personal schedules. In this study, 28 patients with GD completed the MMPI-2 and a background questionnaire. GD patients scored significantly higher than the MMPI-2 normative sample on MMPI-2 scales of Validity (K), Hypochondriasis (Hs), Depression (D), Hysteria (Hy), Psychasthenia (Pt) and Schizophrenia (Sc). Individuals with elevated scores on the Hs, D and Hy scales tend to have somatic concerns and depressed mood. Under stress, they are likely to report physical symptoms. Elevated Pt and Sc scales suggest psychological turmoil and, possibly feelings of isolation. An elevated K scale indicates a tendency for individuals to deny psychopathology. The length of time the patient with GD had been on enzyme replacement therapy was not significantly related to any of the 13 MMPI-2 scales. Cohorts of patients with chronic heart disease (CRHD) and cohorts of patients with chronic pain were utilized as comparative populations in this investigation. The elevated scores of the GD patients on MMPI-2 scales Hs, D and Hy were similar to those of the CRHD population. The chronic pain patients also showed elevations on MMPI-2 scales Hs, D and Hy, which were elevated in the GD patients; the elevations in the chronic pain patients were higher than those shown by the GD patients. We conclude that patients with GD exhibit moderate to severe psychological complications, similar to patients with other long-term chronic illnesses.

摘要

明尼苏达多相人格调查表(MMPI - 2)常用于慢性病和慢性疼痛人群以评估心理功能。我们报告了第一项使用MMPI - 2评估1型戈谢病(GD)患者心理状况的研究结果。1型戈谢病是一种先天性代谢紊乱疾病,作为慢性病具有独特特征:该疾病通常症状较轻,常在儿童晚期或成年期被诊断出来;治疗效果显著,但治疗具有侵入性且费用高昂,还要求患者重新安排工作和个人日程。在本研究中,28名GD患者完成了MMPI - 2测试和一份背景调查问卷。GD患者在MMPI - 2效度量表(K)、疑病量表(Hs)、抑郁量表(D)、癔症量表(Hy)、精神衰弱量表(Pt)和精神分裂症量表(Sc)上的得分显著高于MMPI - 2常模样本。Hs、D和Hy量表得分升高的个体往往存在躯体不适和情绪低落。在压力下,他们可能会报告身体症状。Pt和Sc量表得分升高表明心理紊乱,可能还有孤独感。K量表得分升高表明个体有否认精神病理学问题的倾向。GD患者接受酶替代治疗的时间长短与13个MMPI - 2量表中的任何一个均无显著相关性。在本调查中,慢性心脏病(CRHD)患者队列和慢性疼痛患者队列被用作对照人群。GD患者在MMPI - 2量表Hs、D和Hy上的得分升高与CRHD人群相似。慢性疼痛患者在MMPI - 2量表Hs、D和Hy上也有升高,GD患者同样如此;慢性疼痛患者的得分升高幅度高于GD患者。我们得出结论,GD患者表现出中度至重度的心理并发症,与其他长期慢性病患者相似。

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