Psychological aspects of patients with Fabry disease.

Journal of Inherited Metabolic Disease
T Wilson CrosbieS Packman

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 di...Continue Reading

References

Jan 1, 1987·Psychotherapy and Psychosomatics·B D Kirkcaldy, E Kobylinska
May 25, 1967·The New England Journal of Medicine·R O BradyL Laster
Jan 1, 1993·International Journal of Psychiatry in Medicine·R P Grewal
May 23, 1998·Diabetes Research and Clinical Practice·B B AbuaishaA J Boulton
Jul 10, 1999·Journal of Inherited Metabolic Disease·L Varvogli, S E Waisbren
Jul 6, 2000·Journal of Personality Assessment·R E McGrathT Piacentini
Dec 6, 2000·JAMA : the Journal of the American Medical Association·R O Brady, R Schiffmann
Dec 16, 2000·Journal of Personality Assessment·K GrossR L Greene
Jun 21, 2001·JAMA : the Journal of the American Medical Association·R SchiffmannR O Brady
Jan 5, 2002·Expert Opinion on Biological Therapy·Mark C Glassy, Keiji Koda
Mar 14, 2002·Expert Opinion on Biological Therapy·Gregory M Pastores, Ravi Thadhani
May 9, 2003·Journal of Genetic Counseling·Robin L BennettRavi Thadhani
May 12, 2004·Neuroscience Letters·Ji-Sheng Han
Jul 16, 2004·The Journal of Alternative and Complementary Medicine : Research on Paradigm, Practice, and Policy·Kenneth D PhillipsGregory A Hand
Dec 15, 2004·Mayo Clinic Proceedings·Hsiupei ChenRandall P Brewer
Apr 8, 2006·Journal of Inherited Metabolic Disease·W PackmanS Packman
Mar 16, 2007·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·S SchröderJ H Greten
Nov 13, 2007·Journal of Inherited Metabolic Disease·A L ColeR H Lachmann
Nov 27, 2007·Molecular Genetics and Metabolism·William R WilcoxUNKNOWN Fabry Registry

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Citations

Jun 16, 2010·Journal of Inherited Metabolic Disease·Perri SegalAnnick Raas-Rothschild
Jul 20, 2010·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·I SchermulyA Fellgiebel
Nov 26, 2010·Orphanet Journal of Rare Diseases·Dominique P Germain
Aug 21, 2013·Journal of Inherited Metabolic Disease·Fay E BolsoverRobin H Lachmann
Apr 26, 2012·Molecular Genetics and Metabolism·Stacy L WeberWendy Packman
Dec 6, 2019·Orphanet Journal of Rare Diseases·Esther NoëlOlivier Lidove
Jan 30, 2020·Orphanet Journal of Rare Diseases·Simon KörverMirjam Langeveld
May 17, 2017·BioPsychoSocial Medicine·Fumie KatoMikihiko Fukunaga
Nov 7, 2020·Metabolic Brain Disease·Patrícia VarelaJoão Bosco Pesquero

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