Defining severe familial hypercholesterolaemia and the implications for clinical management: a consensus statement from the International Atherosclerosis Society Severe Familial Hypercholesterolemia Panel.
Identifieur interne : 002119 ( Main/Merge ); précédent : 002118; suivant : 002120Defining severe familial hypercholesterolaemia and the implications for clinical management: a consensus statement from the International Atherosclerosis Society Severe Familial Hypercholesterolemia Panel.
Auteurs : Raul D. Santos [Brésil] ; Samuel S. Gidding [États-Unis] ; Robert A. Hegele [Canada] ; Marina A. Cuchel [États-Unis] ; Philip J. Barter [Australie] ; Gerald F. Watts [Australie] ; Seth J. Baum [États-Unis] ; Alberico L. Catapano [Italie] ; M John Chapman [France] ; Joep C. Defesche [Pays-Bas] ; Emanuela Folco [Italie] ; Tomas Freiberger [République tchèque] ; Jacques Genest [Canada] ; G Kees Hovingh [Pays-Bas] ; Mariko Harada-Shiba [Japon] ; Steve E. Humphries [Royaume-Uni] ; Ann S. Jackson [États-Unis] ; Pedro Mata [Espagne] ; Patrick M. Moriarty [États-Unis] ; Frederick J. Raal [Afrique du Sud] ; Khalid Al-Rasadi [Oman] ; Kausik K. Ray [Royaume-Uni] ; Zelijko Reiner [Croatie] ; Eric J G. Sijbrands [Pays-Bas] ; Shizuya Yamashita [Japon]Source :
- The lancet. Diabetes & endocrinology [ 2213-8595 ] ; 2016.
Abstract
Familial hypercholesterolaemia is common in individuals who had a myocardial infarction at a young age. As many as one in 200 people could have heterozygous familial hypercholesterolaemia, and up to one in 300 000 individuals could be homozygous. The phenotypes of heterozygous and homozygous familial hypercholesterolaemia overlap considerably; the response to treatment is also heterogeneous. In this Review, we aim to define a phenotype for severe familial hypercholesterolaemia and identify people at highest risk for cardiovascular disease, based on the concentration of LDL cholesterol in blood and individuals' responsiveness to conventional lipid-lowering treatment. We assess the importance of molecular characterisation and define the role of other cardiovascular risk factors and advanced subclinical coronary atherosclerosis in risk stratification. Individuals with severe familial hypercholesterolaemia might benefit in particular from early and more aggressive cholesterol-lowering treatment (eg, with PCSK9 inhibitors). In addition to better tailored therapy, more precise characterisation of individuals with severe familial hypercholesterolaemia could improve resource use.
DOI: 10.1016/S2213-8587(16)30041-9
PubMed: 27246162
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Defining severe familial hypercholesterolaemia and the implications for clinical management: a consensus statement from the International Atherosclerosis Society Severe Familial Hypercholesterolemia Panel.</title><author><name sortKey="Santos, Raul D" sort="Santos, Raul D" uniqKey="Santos R" first="Raul D" last="Santos">Raul D. Santos</name><affiliation wicri:level="3"><nlm:affiliation>Lipid Clinic Heart Institute (InCor), University of São Paulo Medical School Hospital, and Preventive Medicine Centre and Cardiology Program, Hospital Israelita Albert Einstein, São Paulo, Brazil. Electronic address: rdsf@uol.com.br.</nlm:affiliation><country xml:lang="fr">Brésil</country><wicri:regionArea>Lipid Clinic Heart Institute (InCor), University of São Paulo Medical School Hospital, and Preventive Medicine Centre and Cardiology Program, Hospital Israelita Albert Einstein, São Paulo</wicri:regionArea><placeName><settlement type="city">São Paulo</settlement><region type="state">État de São Paulo</region></placeName></affiliation></author><author><name sortKey="Gidding, Samuel S" sort="Gidding, Samuel S" uniqKey="Gidding S" first="Samuel S" last="Gidding">Samuel S. Gidding</name><affiliation wicri:level="2"><nlm:affiliation>Nemours Cardiac Center, A I DuPont Hospital for Children, Wilmington, DE, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Nemours Cardiac Center, A I DuPont Hospital for Children, Wilmington, DE</wicri:regionArea><placeName><region type="state">Delaware</region></placeName></affiliation></author><author><name sortKey="Hegele, Robert A" sort="Hegele, Robert A" uniqKey="Hegele R" first="Robert A" last="Hegele">Robert A. 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Catapano</name><affiliation wicri:level="3"><nlm:affiliation>Department of Pharmacological and Biomolecular Sciences, University of Milan, Milan, Italy; IRCCS Multimedica, Milan, Italy.</nlm:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Pharmacological and Biomolecular Sciences, University of Milan, Milan, Italy; IRCCS Multimedica, Milan</wicri:regionArea><placeName><settlement type="city">Milan</settlement><region nuts="2">Lombardie</region></placeName></affiliation></author><author><name sortKey="Chapman, M John" sort="Chapman, M John" uniqKey="Chapman M" first="M John" last="Chapman">M John Chapman</name><affiliation wicri:level="3"><nlm:affiliation>Pitié-Sâlpetrière University Hospital, Paris, France.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Pitié-Sâlpetrière University Hospital, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Defesche, Joep C" sort="Defesche, Joep C" uniqKey="Defesche J" first="Joep C" last="Defesche">Joep C. Defesche</name><affiliation wicri:level="4"><nlm:affiliation>University of Amsterdam, Academic Medical Center (AMC), Amsterdam, Netherlands.</nlm:affiliation><country xml:lang="fr">Pays-Bas</country><wicri:regionArea>University of Amsterdam, Academic Medical Center (AMC), Amsterdam</wicri:regionArea><placeName><settlement type="city">Amsterdam</settlement><region nuts="2" type="province">Hollande-Septentrionale</region><settlement type="city">Amsterdam</settlement></placeName><orgName type="university">Université d'Amsterdam</orgName></affiliation></author><author><name sortKey="Folco, Emanuela" sort="Folco, Emanuela" uniqKey="Folco E" first="Emanuela" last="Folco">Emanuela Folco</name><affiliation wicri:level="3"><nlm:affiliation>International Atherosclerosis Society, Milan, Italy.</nlm:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>International Atherosclerosis Society, Milan</wicri:regionArea><placeName><settlement type="city">Milan</settlement><region nuts="2">Lombardie</region></placeName></affiliation></author><author><name sortKey="Freiberger, Tomas" sort="Freiberger, Tomas" uniqKey="Freiberger T" first="Tomas" last="Freiberger">Tomas Freiberger</name><affiliation wicri:level="3"><nlm:affiliation>Molecular Genetics Lab, Centre for Cardiovascular Surgery and Transplantation, and Ceitec, Masaryk University, Brno, Czech Republic.</nlm:affiliation><country xml:lang="fr">République tchèque</country><wicri:regionArea>Molecular Genetics Lab, Centre for Cardiovascular Surgery and Transplantation, and Ceitec, Masaryk University, Brno</wicri:regionArea><placeName><settlement type="city">Brno</settlement><region>Moravie</region></placeName></affiliation></author><author><name sortKey="Genest, Jacques" sort="Genest, Jacques" uniqKey="Genest J" first="Jacques" last="Genest">Jacques Genest</name><affiliation wicri:level="1"><nlm:affiliation>McGill University Health Center, Royal Victoria Hospital, Montreal, QC, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>McGill University Health Center, Royal Victoria Hospital, Montreal, QC</wicri:regionArea><wicri:noRegion>QC</wicri:noRegion></affiliation></author><author><name sortKey="Hovingh, G Kees" sort="Hovingh, G Kees" uniqKey="Hovingh G" first="G Kees" last="Hovingh">G Kees Hovingh</name><affiliation wicri:level="4"><nlm:affiliation>University of Amsterdam, Academic Medical Center (AMC), Amsterdam, Netherlands.</nlm:affiliation><country xml:lang="fr">Pays-Bas</country><wicri:regionArea>University of Amsterdam, Academic Medical Center (AMC), Amsterdam</wicri:regionArea><placeName><settlement type="city">Amsterdam</settlement><region nuts="2" type="province">Hollande-Septentrionale</region><settlement type="city">Amsterdam</settlement></placeName><orgName type="university">Université d'Amsterdam</orgName></affiliation></author><author><name sortKey="Harada Shiba, Mariko" sort="Harada Shiba, Mariko" uniqKey="Harada Shiba M" first="Mariko" last="Harada-Shiba">Mariko Harada-Shiba</name><affiliation wicri:level="1"><nlm:affiliation>National Cerebral and Cardiovascular Center Research Institute, Suita, Osaka, Japan.</nlm:affiliation><country xml:lang="fr">Japon</country><wicri:regionArea>National Cerebral and Cardiovascular Center Research Institute, Suita, Osaka</wicri:regionArea><wicri:noRegion>Osaka</wicri:noRegion></affiliation></author><author><name sortKey="Humphries, Steve E" sort="Humphries, Steve E" uniqKey="Humphries S" first="Steve E" last="Humphries">Steve E. Humphries</name><affiliation wicri:level="3"><nlm:affiliation>Centre for Cardiovascular Genetics, Institute of Cardiovascular Science, University College of London, London, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Centre for Cardiovascular Genetics, Institute of Cardiovascular Science, University College of London, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Jackson, Ann S" sort="Jackson, Ann S" uniqKey="Jackson A" first="Ann S" last="Jackson">Ann S. Jackson</name><affiliation wicri:level="2"><nlm:affiliation>International Atherosclerosis Society, Houston, TX, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>International Atherosclerosis Society, Houston, TX</wicri:regionArea><placeName><region type="state">Texas</region></placeName></affiliation></author><author><name sortKey="Mata, Pedro" sort="Mata, Pedro" uniqKey="Mata P" first="Pedro" last="Mata">Pedro Mata</name><affiliation wicri:level="3"><nlm:affiliation>Fundación Hipercolesterolemia Familiar, Madrid, Spain.</nlm:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Fundación Hipercolesterolemia Familiar, Madrid</wicri:regionArea><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Moriarty, Patrick M" sort="Moriarty, Patrick M" uniqKey="Moriarty P" first="Patrick M" last="Moriarty">Patrick M. Moriarty</name><affiliation wicri:level="2"><nlm:affiliation>Atherosclerosis and Lipoprotein-Apheresis Center, University of Kansas Medical Center, Kansas City, KS, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Atherosclerosis and Lipoprotein-Apheresis Center, University of Kansas Medical Center, Kansas City, KS</wicri:regionArea><placeName><region type="state">Kansas</region></placeName></affiliation></author><author><name sortKey="Raal, Frederick J" sort="Raal, Frederick J" uniqKey="Raal F" first="Frederick J" last="Raal">Frederick J. Raal</name><affiliation wicri:level="4"><nlm:affiliation>Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.</nlm:affiliation><country xml:lang="fr">Afrique du Sud</country><wicri:regionArea>Faculty of Health Sciences, University of the Witwatersrand, Johannesburg</wicri:regionArea><orgName type="university">Université du Witwatersrand</orgName><placeName><settlement type="city">Johannesbourg</settlement><region type="region" nuts="2">Gauteng</region></placeName></affiliation></author><author><name sortKey="Al Rasadi, Khalid" sort="Al Rasadi, Khalid" uniqKey="Al Rasadi K" first="Khalid" last="Al-Rasadi">Khalid Al-Rasadi</name><affiliation wicri:level="1"><nlm:affiliation>Sultan Qaboos University Hospital, Muscat, Oman.</nlm:affiliation><country xml:lang="fr">Oman</country><wicri:regionArea>Sultan Qaboos University Hospital, Muscat</wicri:regionArea><wicri:noRegion>Muscat</wicri:noRegion></affiliation></author><author><name sortKey="Ray, Kausik K" sort="Ray, Kausik K" uniqKey="Ray K" first="Kausik K" last="Ray">Kausik K. Ray</name><affiliation wicri:level="3"><nlm:affiliation>School of Public Health, Imperial College London, London, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>School of Public Health, Imperial College London, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Reiner, Zelijko" sort="Reiner, Zelijko" uniqKey="Reiner Z" first="Zelijko" last="Reiner">Zelijko Reiner</name><affiliation wicri:level="1"><nlm:affiliation>European Association for Cardiovascular Prevention and Rehabilitations, Zagreb, Croatia.</nlm:affiliation><country xml:lang="fr">Croatie</country><wicri:regionArea>European Association for Cardiovascular Prevention and Rehabilitations, Zagreb</wicri:regionArea><wicri:noRegion>Zagreb</wicri:noRegion></affiliation></author><author><name sortKey="Sijbrands, Eric J G" sort="Sijbrands, Eric J G" uniqKey="Sijbrands E" first="Eric J G" last="Sijbrands">Eric J G. Sijbrands</name><affiliation wicri:level="3"><nlm:affiliation>Department of Internal Medicine, Erasmus MC, Rotterdam, Netherlands.</nlm:affiliation><country xml:lang="fr">Pays-Bas</country><wicri:regionArea>Department of Internal Medicine, Erasmus MC, Rotterdam</wicri:regionArea><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Yamashita, Shizuya" sort="Yamashita, Shizuya" uniqKey="Yamashita S" first="Shizuya" last="Yamashita">Shizuya Yamashita</name><affiliation wicri:level="1"><nlm:affiliation>Osaka University Graduate School of Medicine, Suita, Osaka, Japan.</nlm:affiliation><country xml:lang="fr">Japon</country><wicri:regionArea>Osaka University Graduate School of Medicine, Suita, Osaka</wicri:regionArea><wicri:noRegion>Osaka</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2016">2016</date><idno type="RBID">pubmed:27246162</idno><idno type="pmid">27246162</idno><idno type="doi">10.1016/S2213-8587(16)30041-9</idno><idno type="wicri:Area/PubMed/Corpus">001760</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">001760</idno><idno type="wicri:Area/PubMed/Curation">001737</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">001737</idno><idno type="wicri:Area/PubMed/Checkpoint">001737</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">001737</idno><idno type="wicri:Area/Ncbi/Merge">003617</idno><idno type="wicri:Area/Ncbi/Curation">003617</idno><idno type="wicri:Area/Ncbi/Checkpoint">003617</idno><idno type="wicri:Area/Main/Merge">002119</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Defining severe familial hypercholesterolaemia and the implications for clinical management: a consensus statement from the International Atherosclerosis Society Severe Familial Hypercholesterolemia Panel.</title><author><name sortKey="Santos, Raul D" sort="Santos, Raul D" uniqKey="Santos R" first="Raul D" last="Santos">Raul D. Santos</name><affiliation wicri:level="3"><nlm:affiliation>Lipid Clinic Heart Institute (InCor), University of São Paulo Medical School Hospital, and Preventive Medicine Centre and Cardiology Program, Hospital Israelita Albert Einstein, São Paulo, Brazil. Electronic address: rdsf@uol.com.br.</nlm:affiliation><country xml:lang="fr">Brésil</country><wicri:regionArea>Lipid Clinic Heart Institute (InCor), University of São Paulo Medical School Hospital, and Preventive Medicine Centre and Cardiology Program, Hospital Israelita Albert Einstein, São Paulo</wicri:regionArea><placeName><settlement type="city">São Paulo</settlement><region type="state">État de São Paulo</region></placeName></affiliation></author><author><name sortKey="Gidding, Samuel S" sort="Gidding, Samuel S" uniqKey="Gidding S" first="Samuel S" last="Gidding">Samuel S. Gidding</name><affiliation wicri:level="2"><nlm:affiliation>Nemours Cardiac Center, A I DuPont Hospital for Children, Wilmington, DE, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Nemours Cardiac Center, A I DuPont Hospital for Children, Wilmington, DE</wicri:regionArea><placeName><region type="state">Delaware</region></placeName></affiliation></author><author><name sortKey="Hegele, Robert A" sort="Hegele, Robert A" uniqKey="Hegele R" first="Robert A" last="Hegele">Robert A. Hegele</name><affiliation wicri:level="1"><nlm:affiliation>Department of Medicine and Robarts Research Institute, Schulich School of Medicine, Western University, London, ON, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Department of Medicine and Robarts Research Institute, Schulich School of Medicine, Western University, London, ON</wicri:regionArea><wicri:noRegion>ON</wicri:noRegion></affiliation></author><author><name sortKey="Cuchel, Marina A" sort="Cuchel, Marina A" uniqKey="Cuchel M" first="Marina A" last="Cuchel">Marina A. Cuchel</name><affiliation wicri:level="2"><nlm:affiliation>Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA</wicri:regionArea><placeName><region type="state">Pennsylvanie</region></placeName></affiliation></author><author><name sortKey="Barter, Philip J" sort="Barter, Philip J" uniqKey="Barter P" first="Philip J" last="Barter">Philip J. Barter</name><affiliation wicri:level="1"><nlm:affiliation>School of Medical Sciences, University of New South Wales, Sydney, NSW, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>School of Medical Sciences, University of New South Wales, Sydney, NSW</wicri:regionArea><wicri:noRegion>NSW</wicri:noRegion></affiliation></author><author><name sortKey="Watts, Gerald F" sort="Watts, Gerald F" uniqKey="Watts G" first="Gerald F" last="Watts">Gerald F. Watts</name><affiliation wicri:level="1"><nlm:affiliation>Lipid Disorders Clinic, Royal Perth Hospital, The University of Western Australia, Perth, WA, Australia.</nlm:affiliation><country xml:lang="fr">Australie</country><wicri:regionArea>Lipid Disorders Clinic, Royal Perth Hospital, The University of Western Australia, Perth, WA</wicri:regionArea><wicri:noRegion>WA</wicri:noRegion></affiliation></author><author><name sortKey="Baum, Seth J" sort="Baum, Seth J" uniqKey="Baum S" first="Seth J" last="Baum">Seth J. Baum</name><affiliation wicri:level="2"><nlm:affiliation>Preventive Cardiology, Christine E Lynn Women's Health & Wellness Institute, Boca Raton Regional Hospital, Boca Raton, FL, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Preventive Cardiology, Christine E Lynn Women's Health & Wellness Institute, Boca Raton Regional Hospital, Boca Raton, FL</wicri:regionArea><placeName><region type="state">Floride</region></placeName></affiliation></author><author><name sortKey="Catapano, Alberico L" sort="Catapano, Alberico L" uniqKey="Catapano A" first="Alberico L" last="Catapano">Alberico L. Catapano</name><affiliation wicri:level="3"><nlm:affiliation>Department of Pharmacological and Biomolecular Sciences, University of Milan, Milan, Italy; IRCCS Multimedica, Milan, Italy.</nlm:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Pharmacological and Biomolecular Sciences, University of Milan, Milan, Italy; IRCCS Multimedica, Milan</wicri:regionArea><placeName><settlement type="city">Milan</settlement><region nuts="2">Lombardie</region></placeName></affiliation></author><author><name sortKey="Chapman, M John" sort="Chapman, M John" uniqKey="Chapman M" first="M John" last="Chapman">M John Chapman</name><affiliation wicri:level="3"><nlm:affiliation>Pitié-Sâlpetrière University Hospital, Paris, France.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Pitié-Sâlpetrière University Hospital, Paris</wicri:regionArea><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Defesche, Joep C" sort="Defesche, Joep C" uniqKey="Defesche J" first="Joep C" last="Defesche">Joep C. Defesche</name><affiliation wicri:level="4"><nlm:affiliation>University of Amsterdam, Academic Medical Center (AMC), Amsterdam, Netherlands.</nlm:affiliation><country xml:lang="fr">Pays-Bas</country><wicri:regionArea>University of Amsterdam, Academic Medical Center (AMC), Amsterdam</wicri:regionArea><placeName><settlement type="city">Amsterdam</settlement><region nuts="2" type="province">Hollande-Septentrionale</region><settlement type="city">Amsterdam</settlement></placeName><orgName type="university">Université d'Amsterdam</orgName></affiliation></author><author><name sortKey="Folco, Emanuela" sort="Folco, Emanuela" uniqKey="Folco E" first="Emanuela" last="Folco">Emanuela Folco</name><affiliation wicri:level="3"><nlm:affiliation>International Atherosclerosis Society, Milan, Italy.</nlm:affiliation><country xml:lang="fr">Italie</country><wicri:regionArea>International Atherosclerosis Society, Milan</wicri:regionArea><placeName><settlement type="city">Milan</settlement><region nuts="2">Lombardie</region></placeName></affiliation></author><author><name sortKey="Freiberger, Tomas" sort="Freiberger, Tomas" uniqKey="Freiberger T" first="Tomas" last="Freiberger">Tomas Freiberger</name><affiliation wicri:level="3"><nlm:affiliation>Molecular Genetics Lab, Centre for Cardiovascular Surgery and Transplantation, and Ceitec, Masaryk University, Brno, Czech Republic.</nlm:affiliation><country xml:lang="fr">République tchèque</country><wicri:regionArea>Molecular Genetics Lab, Centre for Cardiovascular Surgery and Transplantation, and Ceitec, Masaryk University, Brno</wicri:regionArea><placeName><settlement type="city">Brno</settlement><region>Moravie</region></placeName></affiliation></author><author><name sortKey="Genest, Jacques" sort="Genest, Jacques" uniqKey="Genest J" first="Jacques" last="Genest">Jacques Genest</name><affiliation wicri:level="1"><nlm:affiliation>McGill University Health Center, Royal Victoria Hospital, Montreal, QC, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>McGill University Health Center, Royal Victoria Hospital, Montreal, QC</wicri:regionArea><wicri:noRegion>QC</wicri:noRegion></affiliation></author><author><name sortKey="Hovingh, G Kees" sort="Hovingh, G Kees" uniqKey="Hovingh G" first="G Kees" last="Hovingh">G Kees Hovingh</name><affiliation wicri:level="4"><nlm:affiliation>University of Amsterdam, Academic Medical Center (AMC), Amsterdam, Netherlands.</nlm:affiliation><country xml:lang="fr">Pays-Bas</country><wicri:regionArea>University of Amsterdam, Academic Medical Center (AMC), Amsterdam</wicri:regionArea><placeName><settlement type="city">Amsterdam</settlement><region nuts="2" type="province">Hollande-Septentrionale</region><settlement type="city">Amsterdam</settlement></placeName><orgName type="university">Université d'Amsterdam</orgName></affiliation></author><author><name sortKey="Harada Shiba, Mariko" sort="Harada Shiba, Mariko" uniqKey="Harada Shiba M" first="Mariko" last="Harada-Shiba">Mariko Harada-Shiba</name><affiliation wicri:level="1"><nlm:affiliation>National Cerebral and Cardiovascular Center Research Institute, Suita, Osaka, Japan.</nlm:affiliation><country xml:lang="fr">Japon</country><wicri:regionArea>National Cerebral and Cardiovascular Center Research Institute, Suita, Osaka</wicri:regionArea><wicri:noRegion>Osaka</wicri:noRegion></affiliation></author><author><name sortKey="Humphries, Steve E" sort="Humphries, Steve E" uniqKey="Humphries S" first="Steve E" last="Humphries">Steve E. Humphries</name><affiliation wicri:level="3"><nlm:affiliation>Centre for Cardiovascular Genetics, Institute of Cardiovascular Science, University College of London, London, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Centre for Cardiovascular Genetics, Institute of Cardiovascular Science, University College of London, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Jackson, Ann S" sort="Jackson, Ann S" uniqKey="Jackson A" first="Ann S" last="Jackson">Ann S. Jackson</name><affiliation wicri:level="2"><nlm:affiliation>International Atherosclerosis Society, Houston, TX, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>International Atherosclerosis Society, Houston, TX</wicri:regionArea><placeName><region type="state">Texas</region></placeName></affiliation></author><author><name sortKey="Mata, Pedro" sort="Mata, Pedro" uniqKey="Mata P" first="Pedro" last="Mata">Pedro Mata</name><affiliation wicri:level="3"><nlm:affiliation>Fundación Hipercolesterolemia Familiar, Madrid, Spain.</nlm:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Fundación Hipercolesterolemia Familiar, Madrid</wicri:regionArea><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Moriarty, Patrick M" sort="Moriarty, Patrick M" uniqKey="Moriarty P" first="Patrick M" last="Moriarty">Patrick M. Moriarty</name><affiliation wicri:level="2"><nlm:affiliation>Atherosclerosis and Lipoprotein-Apheresis Center, University of Kansas Medical Center, Kansas City, KS, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Atherosclerosis and Lipoprotein-Apheresis Center, University of Kansas Medical Center, Kansas City, KS</wicri:regionArea><placeName><region type="state">Kansas</region></placeName></affiliation></author><author><name sortKey="Raal, Frederick J" sort="Raal, Frederick J" uniqKey="Raal F" first="Frederick J" last="Raal">Frederick J. Raal</name><affiliation wicri:level="4"><nlm:affiliation>Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.</nlm:affiliation><country xml:lang="fr">Afrique du Sud</country><wicri:regionArea>Faculty of Health Sciences, University of the Witwatersrand, Johannesburg</wicri:regionArea><orgName type="university">Université du Witwatersrand</orgName><placeName><settlement type="city">Johannesbourg</settlement><region type="region" nuts="2">Gauteng</region></placeName></affiliation></author><author><name sortKey="Al Rasadi, Khalid" sort="Al Rasadi, Khalid" uniqKey="Al Rasadi K" first="Khalid" last="Al-Rasadi">Khalid Al-Rasadi</name><affiliation wicri:level="1"><nlm:affiliation>Sultan Qaboos University Hospital, Muscat, Oman.</nlm:affiliation><country xml:lang="fr">Oman</country><wicri:regionArea>Sultan Qaboos University Hospital, Muscat</wicri:regionArea><wicri:noRegion>Muscat</wicri:noRegion></affiliation></author><author><name sortKey="Ray, Kausik K" sort="Ray, Kausik K" uniqKey="Ray K" first="Kausik K" last="Ray">Kausik K. Ray</name><affiliation wicri:level="3"><nlm:affiliation>School of Public Health, Imperial College London, London, UK.</nlm:affiliation><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>School of Public Health, Imperial College London, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Reiner, Zelijko" sort="Reiner, Zelijko" uniqKey="Reiner Z" first="Zelijko" last="Reiner">Zelijko Reiner</name><affiliation wicri:level="1"><nlm:affiliation>European Association for Cardiovascular Prevention and Rehabilitations, Zagreb, Croatia.</nlm:affiliation><country xml:lang="fr">Croatie</country><wicri:regionArea>European Association for Cardiovascular Prevention and Rehabilitations, Zagreb</wicri:regionArea><wicri:noRegion>Zagreb</wicri:noRegion></affiliation></author><author><name sortKey="Sijbrands, Eric J G" sort="Sijbrands, Eric J G" uniqKey="Sijbrands E" first="Eric J G" last="Sijbrands">Eric J G. Sijbrands</name><affiliation wicri:level="3"><nlm:affiliation>Department of Internal Medicine, Erasmus MC, Rotterdam, Netherlands.</nlm:affiliation><country xml:lang="fr">Pays-Bas</country><wicri:regionArea>Department of Internal Medicine, Erasmus MC, Rotterdam</wicri:regionArea><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Yamashita, Shizuya" sort="Yamashita, Shizuya" uniqKey="Yamashita S" first="Shizuya" last="Yamashita">Shizuya Yamashita</name><affiliation wicri:level="1"><nlm:affiliation>Osaka University Graduate School of Medicine, Suita, Osaka, Japan.</nlm:affiliation><country xml:lang="fr">Japon</country><wicri:regionArea>Osaka University Graduate School of Medicine, Suita, Osaka</wicri:regionArea><wicri:noRegion>Osaka</wicri:noRegion></affiliation></author></analytic><series><title level="j">The lancet. Diabetes & endocrinology</title><idno type="eISSN">2213-8595</idno><imprint><date when="2016" type="published">2016</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Familial hypercholesterolaemia is common in individuals who had a myocardial infarction at a young age. As many as one in 200 people could have heterozygous familial hypercholesterolaemia, and up to one in 300 000 individuals could be homozygous. The phenotypes of heterozygous and homozygous familial hypercholesterolaemia overlap considerably; the response to treatment is also heterogeneous. In this Review, we aim to define a phenotype for severe familial hypercholesterolaemia and identify people at highest risk for cardiovascular disease, based on the concentration of LDL cholesterol in blood and individuals' responsiveness to conventional lipid-lowering treatment. We assess the importance of molecular characterisation and define the role of other cardiovascular risk factors and advanced subclinical coronary atherosclerosis in risk stratification. Individuals with severe familial hypercholesterolaemia might benefit in particular from early and more aggressive cholesterol-lowering treatment (eg, with PCSK9 inhibitors). In addition to better tailored therapy, more precise characterisation of individuals with severe familial hypercholesterolaemia could improve resource use.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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