Endocardial fibroelastosis and Niemann-Pick disease.
نویسنده
چکیده
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.
منابع مشابه
بررسی یک مورد بیماری Niemann Pick تیپ A
Niemann Pick type A is a very rare hereditary disease with an incidence 1 in 20000-40000 live birth, which is calassified as a shingolipidoses. The disease is marked by the abnormal accumulation of sphingomyelin in most tissues, secondary to sphingomylinase deficiency. The most clinical manifestations are: Splenohepatomegaly–cherry red maculae-neuropathologic findings . This is a ...
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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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Background: The protein of Niemann-pick type C1 (NPC1) gene promotes the egress of cholesterol from late endosomes and lysosomes to other cellular compartments and contributes to a process known as reverse cholesterol transport. This study aimed to examine whether promoter methylation of NPC1 is associated with risk of cardiovascular disease (CVD). Methods: Fifty CVD patients and 50 healthy sub...
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Niemann-pick disease is a severe disorder in sphingolipid metabolism and esterification of cholesterol which results in accumulations of sphingomyelin in different tissues. This disease is characterized with hepatosplenomegaly, fever and foam cell appearance in microscopic examination of bone marrow, liver and spleen. The case presented in this study is a four-month-old female infant with chief...
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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عنوان ژورنال:
- British heart journal
دوره 39 12 شماره
صفحات -
تاریخ انتشار 1977