نتایج جستجو برای: familial hypercholesterolemia fh

تعداد نتایج: 68801  

Journal: :Clinical Pediatric Endocrinology 2021

Familial hypercholesterolemia (FH, OMIM number #143890), a life-threatening monogenic disorder characterized by high levels of low-density lipoprotein cholesterol (LDL-C), is classified into dominant and recessive types (1). The form FH may result from mutations in the LDLR, APOB, PCSK9 genes (2). However, receptor (LDLR) adaptor protein-1 (LDLRAP1) gene cause an autosomal inheritance pattern c...

Journal: :Journal of Atherosclerosis and Thrombosis 2017

2016

Genetic testing to confirm a diagnosis of familial hypercholesterolemia (FH) may be considered MEDICALLY NECESSARY when a definitive diagnosis is required as an eligibility criterion for specialty medications and when the following criteria are met:  Genetic testing is targeted to individuals who are in an uncertain category according to clinical criteria (personal and family history, physical...

2012
Damon A Bell Amanda J Hooper Gerald F Watts John R Burnett

Familial hypercholesterolemia (FH) is an autosomal dominant condition with a population prevalence of one in 300-500 (heterozygous) that is characterized by high levels of low-density lipoprotein (LDL) cholesterol, tendon xanthomata, and premature atherosclerosis and coronary heart disease (CHD). FH is caused mainly by mutations in the LDLR gene. However, mutations in other genes including APOB...

2012
Klaus G Parhofer

Familial hypercholesterolemia (FH) is an autosomal-dominant inherited disease with a prevalence of one in 500 (heterozygous) to one in 1,000,000 (homozygous). Mutations of the low-density lipoprotein (LDL) receptor gene, the apolipoprotein B100 gene, or the PCSK9 gene may be responsible for the disease. The resulting LDL hypercholesterolemia results in premature atherosclerosis as early as chil...

Journal: :Atherosclerosis 2011
Hiroshi Mabuchi Atsushi Nohara Tohru Noguchi Junji Kobayashi Masa-Aki Kawashiri Hayato Tada Chiaki Nakanishi Mika Mori Masakazu Yamagishi Akihiro Inazu Junji Koizumi

AIM Familial hypercholesterolemia (FH) is caused by mutations of FH genes, i.e. LDL-receptor (LDLR), PCSK9 and apolipoprotein B (ApoB) gene. We evaluated the usefulness of DNA analysis for the diagnosis of homozygous FH (homo-FH), and studied the frequency of FH in the Hokuriku district of Japan. METHODS Twenty-five homo-FH patients were recruited. LDLR mutations were identified using the Inv...

Journal: :Journal of the American College of Cardiology 2016
Amit V Khera Hong-Hee Won Gina M Peloso Kim S Lawson Traci M Bartz Xuan Deng Elisabeth M van Leeuwen Pradeep Natarajan Connor A Emdin Alexander G Bick Alanna C Morrison Jennifer A Brody Namrata Gupta Akihiro Nomura Thorsten Kessler Stefano Duga Joshua C Bis Cornelia M van Duijn L Adrienne Cupples Bruce Psaty Daniel J Rader John Danesh Heribert Schunkert Ruth McPherson Martin Farrall Hugh Watkins Eric Lander James G Wilson Adolfo Correa Eric Boerwinkle Piera Angelica Merlini Diego Ardissino Danish Saleheen Stacey Gabriel Sekar Kathiresan

BACKGROUND Approximately 7% of American adults have severe hypercholesterolemia (untreated low-density lipoprotein [LDL] cholesterol ≥190 mg/dl), which may be due to familial hypercholesterolemia (FH). Lifelong LDL cholesterol elevations in FH mutation carriers may confer coronary artery disease (CAD) risk beyond that captured by a single LDL cholesterol measurement. OBJECTIVES This study ass...

Journal: :Arteriosclerosis, thrombosis, and vascular biology 1999
G Zuliani M Arca A Signore G Bader S Fazio M Chianelli S Bellosta F Campagna A Montali M Maioli A Pacifico G Ricci R Fellin

We previously described a Sardinian family in which the probands had a severe form of hypercholesterolemia, suggestive of familial hypercholesterolemia (FH). However, low density lipoprotein (LDL) receptor activity in fibroblasts from these subjects and LDL binding ability were normal. The characteristics of the pedigree were consistent with an autosomal recessive trait. Sitosterolemia and pseu...

2005
Pekka V.I. Koivisto Ulla-Maija Koivisto Petri T. Kovanen Helena Gylling Tatu A. Miettinen Kimmo Kontula

We describe a mutation of the low-density lipoprotein (LDL) receptor gene, designated familial hypercholesterolemia (FH)-Espoo, which deletes exon 15 of the LDL receptor gene. The mutant receptor is predicted to lack 57 amino acids, including 18 serine and threonine residues, which are the sites of the clustered 0-linked sugars of the receptor. Studies on 10 carriers of this gene revealed that ...

2016
Andrzej Pajak Krystyna Szafraniec Maciej Polak Wojciech Drygas Walerian Piotrowski Tomasz Zdrojewski Piotr Jankowski

INTRODUCTION Familial hypercholesterolemia (FH) is a severely underdiagnosed and undertreated genetic disorder. Little is known about regional variation in the prevalence of FH, and information for Central and Eastern Europe (CEE) is scarce. This paper assesses the prevalence of FH and related cardiovascular disease (CVD) risk factors in Poland. MATERIAL AND METHODS We performed a meta-analys...

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