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Health and Quality of Life Outcomes
Published in: Health and Quality of Life Outcomes 1/2016

Open Access 01-12-2016 | Research

Structural equation modeling of the quality of life for patients with marfan syndrome

Authors: Ju Ryoung Moon, Yong Ae Cho, June Huh, I-Seok Kang, Duk-Kyung Kim

Published in: Health and Quality of Life Outcomes | Issue 1/2016

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Abstract

Background

We used structural equation modeling to evaluate the quality of life (QOL) for patients with Marfan syndrome (MFS). The goal was to provide guidelines to facilitate the development of interventions and strategies to improve the QOL for patients with MFS.

Methods

The participants fulfilled the Ghent 2 criteria for MFS and they comprised patients who visited the cardiology outpatient department of a tertiary hospital in Seoul, Korea, between August 17, 2013 and April 17, 2014. Demographic, social support, disease-related factors, biobehavioral factors, and QOL data were collected in one-on-one interviews.

Results

The final analyses included 218 patients. Anxious and depressed patients comprised 63.8 and 71.5 % of the sample, respectively. For the hypothetical model, the goodness-of-fit index = 0.91, normal fit index = 0.93, and comparative fit index = 0.90. The outcome was suitable for the recommended level, so the hypothetical model appeared to fit the data. In patients with MFS, the QOL was affected significantly by social support, disease-related factors, and biobehavioral factors. These variables explained 72.4 % of the QOL in patients with MFS. Biobehavioral factors had the strongest and most direct effects on QOL.

Conclusion

To improve QOL in patients with MFS, comprehensive interventions are necessary to assess and manage biobehavioral factors, social support, and disease-related factors.
Literature
1.
Loeys BL, Dietz HC, Braverman AC, Callewaert BL, De Backer J, Devereux RB, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet. 2010;47:476–85.CrossRefPubMed
2.
Gray JR, Bridges AB, West RR, McLeish L, Stuart AG, Dean JC, et al. Life expectancy in British Marfan syndrome populations. Clin Genet. 1998;54:124–8.CrossRefPubMed
3.
Velvin G, Bathen T, Rand-Hendriksen S, Geirdal AǾ. Systematic review of the psychosocail aspects of living with Marfan syndrome. Glin Genet. 2015;87:109–16.
4.
Peters KF, Kong F, Horne R, Frandcomano CA, Bisescker BB. Living with Marfan syndrome I. Perceptation of the condition. Glin Genet. 2001;620:273–82.
5.
Rand-Hendriksen S, Sorensen I, Holmstrom H, Andersson S, Finset A. Fatigue, cognitive functioning and psychological distress in Marfan syndrome, a pilot study. Psychol Health Med. 2007;12:305–13.CrossRefPubMed
6.
Peters KF, Kong F, Hanslo M, Biesecker BB. Living with Marfan syndrome III. Quality of life and reproductive planning. Clin Genet. 2002;62:110–20.CrossRefPubMed
7.
Peters KF, Apse KA, Blackford A, McHugh B, Michalic D, Biesecker BB. Social and behavior research in clinical genetics. Living with marfan syndrome: coping with stigma. Glin Genet. 2005;68:6–14.
8.
De Bie S, De Paepe A, Delvaux I, Davies S, Hennekam RC. Marfan syndrome in Europe. A questionnaire study on patients perception. Community Genet. 2004;7:216–26.CrossRefPubMed
9.
Fusar-Poli P, Klersy C, Stramesi F, Callegari A, Arbustini E, Politi P. Determinants of quality of life in Marfan syndrome. Psychosomatics. 2008;49:243–8.CrossRefPubMed
10.
Van Tongerloo A, De Paepe A. PSYCHOSOCIAL adaptation in adolescents and young adults with MARFNA syndrome: AN exploratory study. J Med Genet. 1998;35:405–09.CrossRefPubMedPubMedCentral
11.
Mercuro G, Carpiniello G, Ruscazio M, Zoncu S, Montisci R, Rudas N, Cherchi A. Association between psychiatric disorders and Marfan’s syndrome in a large Sardinian family with a high prevalence of cared abormalities. Clin Cardiol. 1997;20:243–45.CrossRefPubMed
12.
Wanson L, Godfroid IO. Psychiatric symptoms and marfan: part of the syndrome or incidental to it? World J Biol Psychiatry. 2002;3:229–30.CrossRefPubMed
13.
Rand-Hendriksen S, Johansen H, Semb SO, Geiran O, Stanghelle JK, Finset A. Health-related quality of life in Marfan syndrome: a cross-sectional study of Short Form 36 in 84 adults with a verified diagnosis. Genet Med. 2010;12:517–24.CrossRefPubMed
14.
Bathen T, Velvin G, Rand-Hendriksen S, Robinson HS. Fatigue in adults with Marfan syndrome, occurrence and associations to pain and other factors. Am J Med Genet. 2014;part A 164 A:1931–39.CrossRef
15.
Peters KF, Horne T, Kong F, Frascomano CA, Biesecker BB. Living with Marfan syndrome II. Medication adherence and physical activity modification. Glin Genet. 2001;60:283–92.
16.
17.
Weil J. Psychosocial Genetic Counseling. New York: Oxford University Press; 2000.
18.
Bae BR. Structural Equation Modeling with AMOS 17.0. Seoul: Chungram; 2009.
19.
Woo JP. The Concept and Understanding of Structural Equation Modeling with AMOS 4.0–20.0. Seoul: Hanna Rae; 2012.
20.
Ware Jr JE, Sherbourne CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care. 1992;30:473–83.CrossRefPubMed
21.
Ware JE, Kosinski M. SF-36 physical & mental health summary scales : a manual for users of version 1. 2nd ed. Lincoln, RI: QualityMetric; 2001.
22.
Nam BH, Lee SU. Testing the validity of the Korean SF-36 health survey. J Korean Soc Health Iinform Health Stat. 2003;2:3–24.
23.
Ware JE, Kosinski M, Dewey JE. How to score version 2 of the SF-36 health survey: standars & acute forms. Lincolin, RI: QualityMetric; 2001.
24.
Han CW, Lee EJ, Iwaya T, Kataoka H, Kohzuki M. Development of the Korean version of Short-Form 36-Item Health Survey: health related QOL of healthy elderly people and elderly patients in Korea. Tohoku J Exp Med. 2004;203:189–94.CrossRefPubMed
25.
Zimet GD, Dahlem NW, Zimet SG, Farley GK. The multidimensional scale of perceived social support. J Pers Assess. 1988;52:30–41.CrossRef
26.
Shin JS, Lee YB. The effects of social supports on psychosocial well-being of the unemployed. Korean J Soc Welfare. 1999;37:241–69.
27.
Kim HW, Choi-Kwon S. Structural equation modeling on quality of life in pre-dialysis patients with chronic kidney disease. J Korean Acad Nurs. 2012;42:699–708.CrossRefPubMed
28.
29.
Zigmond AS, Snaith RP. The hospital anxiety and depression scale. Acta Psychiatr Scand. 1983;67:361–70.CrossRefPubMed
30.
Oh HS, Kim DS. An exploratory study on the concept of uncertainty. J Korean Acad Adult Nurs. 1999;11:831–44.
31.
Krupp LB, LaRocca NG, Muir-Nash J, Steinberg AD. The fatigue severity scale. Application to patients with multiple sclerosis and systemic lupus erythematosus. Arch Neurol. 1989;46:1121–3.CrossRefPubMed
32.
Cash TF, Fleming EC, Alindogan J, Steadman L, Whitehead A. Beyond body image as a trait: the development and validation of the body image states scale. Eat Disord. 2002;10:103–13.CrossRefPubMed
33.
Brislin RW. Back-translation for cross-cultural research. J Cross-Cult Psychol. 1970;1:185–216.CrossRef
34.
Oh J, Yi M. Structural equation modeling on quality of life in older adults with osteoarthritis. J Korean Acad Nurs. 2014;44:75–85.CrossRefPubMed
35.
Suh M, Choi-Kwon S. Structural equation modeling on quality of life in stroke survivors. J Korean Acad Nurs. 2010;40:533–41.CrossRefPubMed
36.
Treasure T, Takkenberg JJ, Golesworthy T, Requ F, Petrou M, Rosendahl U, et al. Personalized external aortic root support (PEARS) in Marfan syndrome: analysis of 1–9 year outcomes by intention –to treat in a cohort of the first 30 consecutive patients to receive a novel tissue and valve-conserving procedure, compared with the published results of aortic root replacement. Heart. 2014;100:969–75.CrossRefPubMedPubMedCentral
37.
Chiu HH, Wu MH, Chen HC, Kao FY, Huang SK. Epidemiological profile of Marfan syndrome in a general population: a national database study. Mayo Clin Proc. 2014;89:34–42.CrossRefPubMed
Metadata
Title
Structural equation modeling of the quality of life for patients with marfan syndrome
Authors
Ju Ryoung Moon
Yong Ae Cho
June Huh
I-Seok Kang
Duk-Kyung Kim
Publication date
01-12-2016
Publisher
BioMed Central
Published in
Health and Quality of Life Outcomes / Issue 1/2016
Electronic ISSN: 1477-7525
DOI
https://doi.org/10.1186/s12955-016-0488-5

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