Document Detail


Health-related quality of life in Marfan syndrome: a cross-sectional study of Short Form 36 in 84 adults with a verified diagnosis.
MedLine Citation:
PMID:  20613543     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PURPOSE: To explore health-related quality of life as measured with Short Form 36 in adults with verified Marfan syndrome and to compare with the general population, other groups with chronic problems and studies on Marfan syndrome. Furthermore, to study potential correlations between the scores on the subscales of Short Form 36 and the presence of biomedical criteria and symptoms of Marfan syndrome.
METHOD: Cross-sectional study. Short Form 36 was investigated in 84 adults with verified Marfan syndrome.
RESULTS: The study group had reduced scores on all eight subscales of Short Form 36 compared with the general population, comparable with other groups with chronic diseases. Compared with earlier Short Form 36 results in Marfan syndrome, we found lower scores for social function, vitality, general health, bodily pain, and role physical. No correlations of substantial explanatory values were found between the Short Form 36 subscales and gender, body mass index, ascending aortic surgery, use of beta-blockers, visual acuity, joint hypermobility, fulfillment of the five major Ghent criteria, and number of major criteria fulfilled. Potential explanations are discussed.
CONCLUSION: Persons with Marfan syndrome have reduced scores for health-related quality of life as measured with Short Form 36, comparable with those in other chronic disorders and disabilities. The reduction does not seem to be related to biomedical criteria or symptoms of Marfan syndrome.
Authors:
Svend Rand-Hendriksen; Heidi Johansen; Svein Ove Semb; Odd Geiran; Johan K Stanghelle; Arnstein Finset
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Genetics in medicine : official journal of the American College of Medical Genetics     Volume:  12     ISSN:  1530-0366     ISO Abbreviation:  Genet. Med.     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-08-12     Completed Date:  2010-11-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9815831     Medline TA:  Genet Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  517-24     Citation Subset:  IM    
Affiliation:
TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, Nesoddtangen, Norway. svend.rand-hendriksen@sunnaas.no
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MeSH Terms
Descriptor/Qualifier:
Adult
Body Constitution / physiology
Body Mass Index
Cross-Sectional Studies
Humans
Linear Models
Marfan Syndrome / pathology*,  psychology*
Mental Health
Norway
Quality of Life / psychology*
Questionnaires*
Statistics, Nonparametric

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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