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DeCS
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Descriptor English:
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Endocardial Fibroelastosis
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Descriptor Spanish:
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Fibroelastosis Endocárdica
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Descriptor Portuguese:
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Fibroelastose Endocárdica
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Synonyms English:
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Endocardial Fibroelastoses
Endomyocardial Fibroelastosis
Fibroelastoses, Endocardial
Fibroelastosis, Endocardial
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Tree Number:
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C14.280.238.281
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Definition English:
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A condition characterized by the thickening of ENDOCARDIUM due to proliferation of fibrous and elastic tissue, usually in the left ventricle leading to impaired cardiac function (CARDIOMYOPATHY, RESTRICTIVE). It is most commonly seen in young children and rarely in adults. It is often associated with congenital heart anomalies (HEART DEFECTS CONGENITAL;) INFECTION; or gene mutation. Defects in the tafazzin protein, encoded by TAZ gene, result in a form of autosomal dominant familial endocardial fibroelastosis. |
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Allowable Qualifiers English:
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Record Number:
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4776
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Unique Identifier:
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D004695
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Occurrence in VHL:
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Similar:
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DeCS
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