Dataset Information

Health-related quality of life in Marfan syndrome: a 10-year follow-up.

ABSTRACT: Background: Marfan syndrome, a rare hereditary connective tissue disorder caused by mutations in fibrillin-1, can affect many organ systems, especially the cardiovascular system. Previous research has paid less attention to health-related quality of life and prospective studies on this topic are needed. The aim of this study was to assess changes in health-related quality of life after 10 years in a Norwegian Marfan syndrome cohort.

Methods: Forty-seven Marfan syndrome patients ??18 years were investigated for all organ manifestations in the 1996 Ghent nosology and completed the self-reported questionnaire, Short-Form-36 Health Survey, at baseline in 2003-2004 and at follow-up in 2014-2015. Paired sample t tests were performed to compare means and multiple regression analyses were performed with age, sex, new cardiovascular and new non-cardiovascular pathology as predictors.

Results: At 10-year follow-up: a significant decline was found in the physical domain. The mental domain was unchanged. Older age predicted a larger decline in physical health-related quality of life. None of the chosen Marfan-related variables predicted changes in any of the subscales of the Short-Form 36 Health Survey or in the physical or the mental domain.

Conclusion: Knowledge of decline in the physical domain, not related to organ affections, may be important in the follow-up of Marfan syndrome patients.

SUBMITTER: Vanem TT

PROVIDER: S-EPMC7706277 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature

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Health-related quality of life in Marfan syndrome: a 10-year follow-up.

Vanem Thy Thy TT Rand-Hendriksen Svend S Brunborg Cathrine C Geiran Odd Ragnar OR Røe Cecilie C

Health and quality of life outcomes 20201201 1

<h4>Background</h4>Marfan syndrome, a rare hereditary connective tissue disorder caused by mutations in fibrillin-1, can affect many organ systems, especially the cardiovascular system. Previous research has paid less attention to health-related quality of life and prospective studies on this topic are needed. The aim of this study was to assess changes in health-related quality of life after 10 years in a Norwegian Marfan syndrome cohort.<h4>Methods</h4>Forty-seven Marfan syndrome patients ≥ 18 ...[more]

PMID: 33256748

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Dataset Information

Health-related quality of life in Marfan syndrome: a 10-year follow-up.

Publications

Health-related quality of life in Marfan syndrome: a 10-year follow-up.

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