Endocardial Fibroelastosis
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چکیده
It is rare only 1-2% of all congenital heart diseases. The number of cases has fallen dramatically in recent years, possibly secondary to better antenatal scanning. It may be familial (10%) with a predominantly X-linked pattern. [3] It affects both sexes equally, usually presenting during the first 3-6 months of life in 80% of cases. Typical age of diagnosis is 2-12 months. It rarely is reported in adolescents and adults. It is an important cause of non-immune hydrops fetalis. [5]
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Endocardial fibroelastosis and Niemann-Pick disease.
The concurrence of endocardial fibroelastosis and Niemann-Pick disease is described. This appears to be the first described case of endocardial fibroelastosis in association with a lipid storage disorder.
متن کاملEndocardial fibroelastosis in infants with hydrops fetalis.
Endocardial fibroelastosis, defined as an endocardium in excess of 30 microns thick, was found in 10 out of 34 cases of hydrops fetalis in a review of 1589 perinatal necropsies carried out between 1976 and 1989. The infants comprised 16 cases of rhesus haemolytic disease, of whom three had endocardial fibroelastosis, and 18 cases of non-rhesus hydrops, of whom seven had endocardial fibroelastos...
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HE various theories of the pathogeii-e.sis of endocardial fibroelastosis, summarized by SCaphir,' include genetic, hypoxic, inflammatory, and mechanical factors. In the laboratory, chronic impairment of cardiac lymph flow in dogs leads to ventricular endocardial fibroelastosis, and a theory of the causcationi of human endocardial fibroelastosis has beeni presented.2 4 Up to now, however, the la...
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A case is presented of constrictive endocardial fibroelastosis without other cardiac abnormality in a newborn infant who was treated successfully by orthotopic heart transplantation.
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Andersen, D. H., and Kelly, J. (1956). Endocardial fibro-elastosis: I. endocardial fibro-elastosis associated with congenital malformations of the heart. Pediatrics, 18, 513, Dordick, J. R. (1951). Diffuse endocardial fibrosis and cardiac hypertrophy in infancy: report of two cases in consecutive siblings. American Journal of Clinical Pathology, 21, 743. Forfar, J. O., Miller, R. A., Bain, A. D...
متن کاملThe Relationship between Human Endocardial
HE various theories of the pathogeii-e.sis of endocardial fibroelastosis, summarized by SCaphir,' include genetic, hypoxic, inflammatory, and mechanical factors. In the laboratory, chronic impairment of cardiac lymph flow in dogs leads to ventricular endocardial fibroelastosis, and a theory of the causcationi of human endocardial fibroelastosis has beeni presented.2 4 Up to now, however, the la...
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