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Research Journal of Pharmacology and Pharmacodynamics
Year : 2019, Volume : 11, Issue : 4
First page : ( 137) Last page : ( 139)
Print ISSN : 0975-4407. Online ISSN : 2321-5836.
Article DOI : 10.5958/2321-5836.2019.00024.7

Marfans Syndrome A Case Report

Menon Aathira1, Roshni P. R2, Dr. Natarajan K. U.3,*

1Student, Pharm D, Department of Pharmacy Practice, Amrita School of Pharmacy, Amrita Vishwa Vidyapeetham, Kochi, 682041, Kerala, India

2Assistant Professor, Department of Pharmacy Practice, Amrita School of Pharmacy, Amrita Vishwa Vidyapeetham, Kochi, 682041, Kerala, India

3Professor and HOD, Department of Cardiology, Amrita Institute of Medical Sciences and Research Centre, Kochi, 682 041, Kerala, India

*Corresponding Author E-mail: kunatarajan@aims.amrita.edu

Online published on 31 December, 2019.

Abstract

It is an autosomal dominant, generalized disorder, affecting the connective tissue, which imparts rigidity, support, and flexibility to the cartilage, tendons, blood vessels, heart valves and other vital parts of the body. It is predominantly caused by chromosomal abnormality1 in the extracellular matrix protein fibrillin. This disease affects multiple systems.2 About 1 in 5000 are affected with the disease and around 26% of the cases have no family history. It manifests mainly as proximal aortic aneurysm, ocular lens displacement, bone overgrowth, especially the long bones. Few people diagnosed with the syndrome may experience a mitral valve prolapse, a condition that may be associated with uneven or rapid heartbeats and breathlessness. Life expectancy of patients with Marfans syndrome is mainly determined by the severity of cardiovascular involvement and has improved substantially as a result of medical and surgical management.3 Early detection and appropriate treatment is critical for patients with Marfans syndrome, as they are at an increased risk of life threatening complications of the cardiovascular system such as aortic dissection and rupture.4The patients may require lifelong treatment, representing a major commitment for themselves as well as care givers.

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Keywords

Proximal aortic aneurysm, aortic dissection, ocular lens displacement.

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