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A cross-national investigation of cardiovascular survival in homozygous familial hypercholesterolemia: The Sino-Roman Study

Stefanutti, Claudia

Journal of clinical lipidology. Volume 13:Number 4 (2019, July 20th); pp 608-617 -- Elsevier

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  • Title:
    A cross-national investigation of cardiovascular survival in homozygous familial hypercholesterolemia: The Sino-Roman Study
  • Author: Stefanutti, Claudia;
    Pang, Jing;
    Di Giacomo, Serafina;
    Wu, Xue;
    Wang, Xumin;
    Morozzi, Claudia;
    Watts, Gerald F.;
    Lin, Jie
  • Found In: Journal of clinical lipidology. Volume 13:Number 4 (2019, July 20th); pp 608-617
  • Journal Title: Journal of clinical lipidology
  • Subjects: Diabète--Périodiques; Hypertension artérielle--Périodiques; Lipides--Métabolisme, Troubles du--Périodiques; Obésité--Périodiques; Lipoproteins--drug effects--Periodicals; Lipids--Metabolism--Disorders--Periodicals; Lipids--Periodicals; Lipids; Lipids--Metabolism--Disorders; Periodicals; Familial hypercholesterolemia--Homozygous--China--Italy--Lipoprotein apheresis--Novel therapies; Electronic journals; Dewey: 616.3997005
  • Rights: Licensed
  • Publication Details: Elsevier
  • Abstract: Background Homozygous familial hypercholesterolemia (hoFH) is a rare inherited disorder characterized by extreme elevation of low-density lipoprotein (LDL) cholesterol, accelerated coronary artery disease, and premature death. Aggressive LDL-lowering therapies are important for survival, but these are not available worldwide. Objective The aim of the study was to compare and contrast cardiovascular outcomes and mortality of hoFH patients in 2 countries with disparate use of lipoprotein apheresis (LA) and modern therapies for lowering LDL cholesterol. Methods A retrospective study was undertaken comparing cardiovascular disease (CVD)-free survival and mortality in 44 hoFH patients who were treated with statins but not LA, from a center in Beijing, China, and 18 hoFH patients who were treated with LA and novel therapies from an early age, from a center in Rome, Italy. Results CVD-free survival and survival were significantly reduced in Chinese patients compared with the Italian patients after 30 years of follow-up (log-rank P  < .01). In a pooled analysis, cardiovascular survival was significantly increased with earlier age at treatment, longer duration of treatment, and lower on-treatment LDL cholesterol concentrations ( P  < .05). In addition, the probability of a CVD event and death were increased in patients that carried a null mutation in the LDLR or had elevated lipoprotein(a). Conclusions We show that coronary artery disease outcomes in patients with hoFH can be significantly improved with earlier and potent LDL cholesterol lowering with pharmacotherapies and LA. This has major implications for countries, such as China, where the models of care for hoFH remains underdeveloped. Highlights We identified important gaps in the care of homozygous familial hypercholesterolemia patients in China and Italy. Chinese patients had significantly reduced survival compared with Italian patients. Efficacious therapies (eg, drugs and lipoprotein apheresis) are crucial to improve outcomes in homozygous familial hypercholesterolemia.
  • Identifier: System Number: ETOCvdc_100092054676.0x000001; Journal ISSN: 1933-2874; 10.1016/j.jacl.2019.05.002
  • Publication Date: 2019
  • Physical Description: Electronic
  • Shelfmark(s): 4958.545500
  • UIN: ETOCvdc_100092054676.0x000001

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