Genetic Architecture of Familial Hypercholesterolaemia

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Genetic Architecture of Familial Hypercholesterolaemia

PURPOSE OF REVIEW Familial hypercholesterolaemia (FH) is an inherited disorder of low-density lipoprotein cholesterol (LDL-C) which is characterised by a raised cholesterol level from birth and a high risk of premature coronary heart disease. In this paper, we review the genetic basis of FH and its impact on the clinical presentation. RECENT FINDINGS Mutations in any of three genes (LDLR, APO...

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Genetic diagnosis of familial hypercholesterolaemia by targeted next-generation sequencing

OBJECTIVES The aim of this study was to combine clinical criteria and next-generation sequencing (pyrosequencing) to establish a diagnosis of familial hypercholesterolaemia (FH). DESIGN, SETTING AND SUBJECTS A total of 77 subjects with a Dutch Lipid Clinic Network score of ≥ 3 (possible, probable or definite FH clinical diagnosis) were recruited from the Lipid Clinic at Sahlgrenska Hospital, ...

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Familial hypercholesterolaemia in Portugal.

Familial hypercholesterolaemia (FH) is characterised clinically by an increased level of circulating LDL cholesterol that leads to lipid accumulation in tendons and arteries, premature atherosclerosis and increased risk of coronary heart disease (CHD). Although Portugal should have about 20,000 cases, this disease is severely under-diagnosed in our country, this being the first presentation of ...

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Discovering familial hypercholesterolaemia.

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Nationwide screening for familial hypercholesterolaemia (FH), using family investigation and molecular testing, has been operative in The Netherlands since 1994. FH is a common inherited disorder of lipoprotein metabolism with a prevalence of 1 in 400 to 500 persons in western societies. Untreated, the disorder leads to hypercholesterolaemia, premature cardiovascular disease, and untimely death...

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ژورنال

عنوان ژورنال: Current Cardiology Reports

سال: 2017

ISSN: 1523-3782,1534-3170

DOI: 10.1007/s11886-017-0848-8