Journal of Cardiovascular Disease Research
Saga of multiple teeth – Case report of Marfan Syndrome
Dr. Bhavika Vhatkar Dr. Naveenkumar Shetty Dr. Hemant Bhutani Dr. Vagisha Choudhary Dr. Janhavi Chandure Dr. Sagar kumar
JCDR. 2021: 638-643
Abstract
Marfan syndrome is a rare autosomal dominant disorder of the connective tissue, which shows skeletal, ligamentous, oro-oculofacial, pulmonary, neurological manifestations. The incidence of the syndrome is approximately 1:5000. There is no reported difference based on the gender, ethnicity and geographic groups. Marfan Syndrome is caused by pathogenic variants of FBN1gene, which encodes a large protein fibrillin-1, which is a major structural component of the extracellular matrix . The diagnosis of the syndrome becomes difficult as it is essentially a clinical one, relying mainly on family history, physical examination and investigation of involved organ systems. Marfan syndrome is mostly accounted by the dentists at an early age. The present case illustrates the typical features of Marfan syndrome and its treatment carried out .
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