Journal ArticleDOI
Long-term treatment with evolocumab added to conventional drug therapy, with or without apheresis, in patients with homozygous familial hypercholesterolaemia: an interim subset analysis of the open-label TAUSSIG study
Frederick J. Raal,G. Kees Hovingh,Dirk J. Blom,Raul D. Santos,Mariko Harada-Shiba,Eric Bruckert,Patrick Couture,Handrean Soran,Gerald F. Watts,Christopher E. Kurtz,Narimon Honarpour,L. Tang,Sree Kasichayanula,Scott M. Wasserman,Evan A. Stein +14 more
TLDR
The interim results suggest that evolocumab is an effective additional option to reduce LDL cholesterol in patients with homozygous familial hypercholesterolaemia or without apheresis, and does not differ from reductions achieved in patients not on Apheresis.About:
This article is published in The Lancet Diabetes & Endocrinology.The article was published on 2017-04-01. It has received 177 citations till now. The article focuses on the topics: Evolocumab & PCSK9.read more
Citations
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Journal ArticleDOI
Clinical Genetic Testing for Familial Hypercholesterolemia: JACC Scientific Expert Panel.
Amy C. Sturm,Joshua W. Knowles,Samuel S. Gidding,Zahid Ahmad,Catherine D. Ahmed,Christie M. Ballantyne,Seth J. Baum,Mafalda Bourbon,Alain Carrié,Marina Cuchel,Sarah D. de Ferranti,Joep C. Defesche,Tomáš Freiberger,Ray E. Hershberger,G. Kees Hovingh,Lala Karayan,Johannes Jacob Pieter Kastelein,Iris Kindt,Stacey R. Lane,Sarah Leigh,MacRae F. Linton,Pedro Mata,William A. Neal,Børge G. Nordestgaard,Raul D. Santos,Mariko Harada-Shiba,Eric J.G. Sijbrands,Nathan O. Stitziel,Shizuya Yamashita,Katherine Wilemon,David H. Ledbetter,Daniel J. Rader +31 more
TL;DR: The Expert Consensus Panel recommends that FH genetic testing become the standard of care for patients with definite or probable FH, as well as for their at-risk relatives, and more accurate risk stratification.
Journal ArticleDOI
The complex molecular genetics of familial hypercholesterolaemia.
TL;DR: The ‘textbook’ presentation of FH, characterized by elevated plasma LDL-cholesterol level, Mendelian inheritance, and family history of clinical features, is a subtype of a group of disorders with a complicated genetic basis.
Journal ArticleDOI
2017 Update of ESC/EAS Task Force on practical clinical guidance for proprotein convertase subtilisin/kexin type 9 inhibition in patients with atherosclerotic cardiovascular disease or in familial hypercholesterolaemia
Ulf Landmesser,M. John Chapman,Jane K Stock,Pierre Amarenco,Jill J. F. Belch,Jan Borén,Michel Farnier,Brian A. Ference,Stephan Gielen,Ian D. Graham,Diederick E. Grobbee,G. Kees Hovingh,Thomas F. Lüscher,Massimo F Piepoli,Kausik K. Ray,Erik S.G. Stroes,Olov Wiklund,Stephan Windecker,José Luis Zamorano,Fausto J. Pinto,Lale Tokgozoglu,Jeroen J. Bax,Alberico L. Catapano +22 more
TL;DR: A correction has been published: European Heart Journal, Volume 39, Issue 22, 7 June 2018, pages 2105 and 2105.
Journal ArticleDOI
PCSK9 inhibitors: clinical evidence and implementation.
TL;DR: Three cardiovascular outcome trials have now demonstrated the clinical benefit of achieving lower plasma LDL-cholesterol levels with the addition of PCSK9 inhibitors to statin therapy, and Marc Sabatine discusses the safety and efficacy data from these trials and their possible implications, such as the definition of new plasma cholesterol targets.
Journal ArticleDOI
PCSK9 Monoclonal Antibodies for the Primary and Secondary Prevention of Cardiovascular Disease
Amand F. Schmidt,Lucy S Pearce,John T. Wilkins,John P. Overington,Aroon D. Hingorani,Juan P. Casas +5 more
TL;DR: A large number of trials randomised participants to alirocumab, three trials to bococizumab, one to RG7652, and four to evolocumab found that PCSK9 inhibitors appeared to have a stronger protective effect on CVD risk, although with considerable uncertainty.
References
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Journal ArticleDOI
Efficacy and safety of more intensive lowering of LDL cholesterol: a meta-analysis of data from 170,000 participants in 26 randomised trials.
Colin Baigent,L Blackwell,Jonathan Emberson,Lisa Holland,Christina Reith,Neeraj Bhala,Richard Peto,Elizabeth H Barnes,Anthony C Keech,John Simes,Rory Collins +10 more
TL;DR: Further reductions in LDL cholesterol safely produce definite further reductions in the incidence of heart attack, of revascularisation, and of ischaemic stroke, with each 1·0 mmol/L reduction reducing the annual rate of these major vascular events by just over a fifth.
Journal ArticleDOI
Molecular genetics of the LDL receptor gene in familial hypercholesterolemia
TL;DR: 79 additional mutations are described and the insights that all 150 mutations have provided into the structure/function relationship of the receptor protein and the clinical manifestations of FH are reviewed.
Journal ArticleDOI
Mipomersen, an apolipoprotein B synthesis inhibitor, for lowering of LDL cholesterol concentrations in patients with homozygous familial hypercholesterolaemia: a randomised, double-blind, placebo-controlled trial.
Frederick J. Raal,Raul D. Santos,Dirk J. Blom,A. David Marais,Min-Ji Charng,William C. Cromwell,Robin H. Lachmann,Daniel Gaudet,Ju L. Tan,Scott Chasan-Taber,Diane Tribble,JoAnn Flaim,Stanley T. Crooke +12 more
TL;DR: Inhibition of apolipoprotein B synthesis by mipomersen represents a novel, effective therapy to reduce LDL cholesterol concentrations in patients with homozygous familial hypercholesterolaemia who are already receiving lipid-lowering drugs, including high-dose statins.
Journal ArticleDOI
Homozygous familial hypercholesterolaemia: new insights and guidance for clinicians to improve detection and clinical management. A position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society
Marina Cuchel,Eric Bruckert,Henry N. Ginsberg,Frederick J. Raal,Raul D. Santos,Robert A. Hegele,Jan Albert Kuivenhoven,Børge G. Nordestgaard,Olivier S. Descamps,Elisabeth Steinhagen-Thiessen,Anne Tybjærg-Hansen,Gerald F. Watts,Maurizio Averna,Catherine Boileau,Jan Borén,A.L. Catapano,Joep C. Defesche,G. Kees Hovingh,Steve E. Humphries,Petri T. Kovanen,Luis Masana,Päivi Pajukanta,Klaus G. Parhofer,Kausik K. Ray,Anton F. H. Stalenhoef,Erik S.G. Stroes,Marja-Riitta Taskinen,Albert Wiegman,Olov Wiklund,M. John Chapman +29 more
TL;DR: In this article, the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society (EAS) critically reviewed available data with the aim of providing clinical guidance for the recognition and management of HoFH.
Journal ArticleDOI
Inhibition of PCSK9 With Evolocumab in Homozygous Familial Hypercholesterolaemia (TESLA Part B): A Randomised, Double-Blind, Placebo-Controlled Trial
Frederick J. Raal,Narimon Honarpour,Dirk J. Blom,G. Kees Hovingh,Feng Xu,Rob Scott,Scott M. Wasserman,Evan A. Stein +7 more
TL;DR: In patients with homozygous familial hypercholesterolaemia receiving stable background lipid-lowering treatment and not on apheresis, evolocumab 420 mg administered every 4 weeks was well tolerated and significantly reduced LDL cholesterol compared with placebo.
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Mutations in PCSK9 cause autosomal dominant hypercholesterolemia.
Marianne Abifadel,Mathilde Varret,Jean-Pierre Rabès,Delphine Allard,Khadija Ouguerram,Martine Devillers,Corinne Cruaud,Suzanne Benjannet,Louise Wickham,D. Erlich,Aurélie Derré,Ludovic Villéger,Michel Farnier,Isabel Beucler,Eric Bruckert,Jean Chambaz,Bernard Chanu,Jean-Michel Lecerf,Gérald Luc,Philippe Moulin,Jean Weissenbach,Annick Prat,Michel Krempf,Claudine Junien,Nabil G. Seidah,Catherine Boileau +25 more