Marfan's syndrome: a family affair

David Strider, Terri Moore, Jane Guarini, Beth Fallin, Jan Ivey, Irving Kron

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Marfan's syndrome (MFS), a heritable connective tissue disorder, may result in cardiac valvular insufficiency, aortic aneurysm or dissection, dislocated lens, and musculoskeletal abnormalities. During a 20-month period (1994-96), an interdisciplinary health care team at a central Virginia medical center evaluated the histories of 112 persons from 15 different families for the presence of MFS-related traits. Seventy-five had at least one MFS-related trait, and 27 subjects underwent echocardiography to evaluate for aortic root dilatation and valvular lesions. Forty-three patients (57.3%) in the above cohort demonstrated significant cardiovascular lesions, with 20 undergoing cardiac surgery. Thirty-one patients (41.3%) were initially seen with significant ocular lesions, and 38 (50.7%) displayed orthopedic deformities. The health care team developed strategies for long-term management of persons with MFS, including antihypertensive therapy, periodic testing, risk-factor modification, genetic counseling, and surgery for appropriate patients. Proactive, consistent management of MFS families will improve long-term health outcomes for this patient population.

Original languageEnglish (US)
Pages (from-to)91-98
Number of pages8
JournalJournal of Vascular Nursing
Volume14
Issue number4
DOIs
StatePublished - Dec 1 1996
Externally publishedYes

Fingerprint

Marfan Syndrome
Patient Care Team
Family Health
Genetic Testing
Preschool Children
Pedigree
Musculoskeletal Abnormalities
Aortic Aneurysm
Genetic Counseling
Connective Tissue
Antihypertensive Agents
Lenses
Thoracic Surgery
Orthopedics
Echocardiography
Dissection
Dilatation
Health
Population

ASJC Scopus subject areas

  • Medical–Surgical

Cite this

Strider, D., Moore, T., Guarini, J., Fallin, B., Ivey, J., & Kron, I. (1996). Marfan's syndrome: a family affair. Journal of Vascular Nursing, 14(4), 91-98. https://doi.org/10.1016/S1062-0303(96)80024-0

Marfan's syndrome : a family affair. / Strider, David; Moore, Terri; Guarini, Jane; Fallin, Beth; Ivey, Jan; Kron, Irving.

In: Journal of Vascular Nursing, Vol. 14, No. 4, 01.12.1996, p. 91-98.

Research output: Contribution to journalArticle

Strider, D, Moore, T, Guarini, J, Fallin, B, Ivey, J & Kron, I 1996, 'Marfan's syndrome: a family affair', Journal of Vascular Nursing, vol. 14, no. 4, pp. 91-98. https://doi.org/10.1016/S1062-0303(96)80024-0
Strider D, Moore T, Guarini J, Fallin B, Ivey J, Kron I. Marfan's syndrome: a family affair. Journal of Vascular Nursing. 1996 Dec 1;14(4):91-98. https://doi.org/10.1016/S1062-0303(96)80024-0
Strider, David ; Moore, Terri ; Guarini, Jane ; Fallin, Beth ; Ivey, Jan ; Kron, Irving. / Marfan's syndrome : a family affair. In: Journal of Vascular Nursing. 1996 ; Vol. 14, No. 4. pp. 91-98.
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