Open AccessCardiovascular Abnormalities in Egyptian Children with Mucopolysaccharidoses
Author(s) -
Laila Selim
Publication year - 2016
Publication title -
journal of clinical and diagnostic research
Language(s) - English
Resource type - Journals
eISSN - 2249-782X
pISSN - 0973-709X
DOI - 10.7860/jcdr/2016/21135.8851
Subject(s) - medicine , enzyme replacement therapy , cardiology , regurgitation (circulation) , pulmonary hypertension , mitral regurgitation , heart disease , stenosis , mitral valve , disease
The Mucopolysaccharidoses (MPS) are rare inherited metabolic disorders. They are characterized by the progressive systemic deposition of Glycosaminoglycans (GAGs). GAGs accumulate in the myocardium and the cardiac valves. Enzyme Replacement Therapy (ERT) is available for MPS I, II, and VI. However, ERT does not appear to improve cardiac valve disease in patients with valve disease present at the start of ERT.