ArticleJournal of inherited metabolic disease2009
Psychological aspects of patients with Fabry disease.
Article in Journal of inherited metabolic disease, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it, 34 citations in OpenAlex.
- Cognitive dysfunction and depression in Fabry disease: a systematic review.Journal of inherited metabolic disease · 2014Pooled it
- Fabry Disease and Central Nervous System Involvement: From Big to Small, from Brain to Synapse.International journal of molecular sciences · 2023Review
- Consensus recommendations for the treatment and management of patients with Fabry disease on migalastat: a modified Delphi study.Frontiers in medicine · 2023Article
- Shared decision making between patients with Fabry disease and physicians in Japan: An online survey.Molecular genetics and metabolism reports · 2022Article
- The potential consequences of bidirectional promoter methylation onBiomedical reports · 2022Article
- Unexplored regulatory sequences of divergently pairedExperimental and therapeutic medicine · 2021Article
- Fabry disease: GLA deletion alters a canonical splice site in a family with neuropsychiatric manifestations.Metabolic brain disease · 2021Article
- Depressive symptoms in Fabry disease: the importance of coping, subjective health perception and pain.Orphanet journal of rare diseases · 2020Article
- Treatment needs and expectations for Fabry disease in France: development of a new Patient Needs Questionnaire.Orphanet journal of rare diseases · 2019Article
- Treatment of Depression in Adults with Fabry Disease.JIMD reports · 2018Article
- Pain threshold reflects psychological traits in patients with chronic pain: a cross-sectional study.BioPsychoSocial medicine · 2017Article
- Efficacy of Enzyme and Substrate Reduction Therapy with a Novel Antagonist of Glucosylceramide Synthase for Fabry Disease.Molecular medicine (Cambridge, Mass.) · 2015Article
- Psychological health in adults with morquio syndrome.JIMD reports · 2015Article
- Early diagnosis of peripheral nervous system involvement in Fabry disease and treatment of neuropathic pain: the report of an expert panel.BMC neurology · 2011Review
- Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease.PloS one · 2010Article
- Fabry disease.Orphanet journal of rare diseases · 2010Review
- Psychiatric and cognitive profile in Anderson-Fabry patients: a preliminary study.Journal of inherited metabolic disease · 2010Article
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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.
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