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John R. Burnett - One of the best experts on this subject based on the ideXlab platform.

  • Update on the diagnosis, treatment and management of rare genetic Lipid Disorders
    Pathology, 2018
    Co-Authors: John R. Burnett, Damon A. Bell, Robert A. Hegele, Amanda J. Hooper
    Abstract:

    Summary Rare genetic Lipid Disorders affect the levels of cholesterol and/or triglyceride in the circulation and, if untreated, can often lead to severe multisystem complications. The field of rare Lipid Disorders is evolving and increasing awareness of these conditions, along with the systematic integration of recent advances or knowledge into clinical practice, is crucial to improve patient outcomes. The aim of this review is to provide an overview of selected rare genetic Lipid Disorders, focusing on the recommended diagnostic strategies and contemporary treatment and management options.

  • The role of patient registries for rare genetic Lipid Disorders.
    Current Opinion in Lipidology, 2018
    Co-Authors: David M. Ng, Matthew I. Bellgard, Amanda J. Hooper, John R. Burnett
    Abstract:

    Purpose of review We review the role, utility and current status of patient registries for rare genetic Lipid Disorders. Recent findings The creation and maintenance of rare genetic Lipid disorder patient registries is critical for disease monitoring, improving clinical best practice, facilitating research and enabling the development of novel therapeutics. An open-source disease registry platform, termed the Rare Disease Registry Framework, has been developed, optimized and deployed for homozygous familial hypercholesterolemia. A global disease-specific registry for lipoprotein lipase deficiency (LPLD), GENetherapy In the mAnagement of Lipoprotein Lipase deficiency, has been established with the aim of enrolling 20–40% of LPLD patients worldwide and will study the natural history of LPLD as well as therapeutic response to the gene therapy alipogene tiparvovec. Similarly, a registry for lysosomal acid lipase deficiency patients in Europe and the United States is studying the clinical outcomes of the enzyme-replacement therapy sebelipase alfa. Summary There are currently few disease-specific rare Lipid disorder patient registries. The very nature of rare genetic Lipid Disorders would suggest that larger national or international registries are necessary to capture clinical data on a sufficient number of patients to provide insight into the prevalence and natural history of these conditions. Furthermore, these registries can help to identify and address deficiencies in current diagnostic and management practices, and facilitate clinical trials of new therapies.

  • Screening for Lipid Disorders.
    Pathology, 2012
    Co-Authors: Damon A. Bell, Amanda J. Hooper, Bender Robert, Glenn Edwards, Frank M. Van Bockxmeer, Gerald F. Watts, John R. Burnett
    Abstract:

    Summary Lipid Disorders, also known as dysLipidaemias, are abnormalities of lipoprotein metabolism and include elevations of total cholesterol, low density lipoprotein (LDL) cholesterol, and triglyceride, and reductions in high density lipoprotein (HDL) cholesterol, and can be acquired or familial in nature. DysLipidaemia is a major risk factor for coronary heart disease and cardiovascular disease (CVD), which is the leading cause of morbidity and mortality in Australia. DysLipidaemia is defined by laboratory testing and using statistically determined criteria. Although the benefits of detecting and treating dysLipidaemia in patients with known CVD is clear, controversy remains regarding screening asymptomatic individuals who are not known to be at increased cardiovascular risk. This review examines the role of screening in the detection and treatment of individuals with Lipid Disorders.

Amanda J. Hooper - One of the best experts on this subject based on the ideXlab platform.

  • Update on the diagnosis, treatment and management of rare genetic Lipid Disorders
    Pathology, 2018
    Co-Authors: John R. Burnett, Damon A. Bell, Robert A. Hegele, Amanda J. Hooper
    Abstract:

    Summary Rare genetic Lipid Disorders affect the levels of cholesterol and/or triglyceride in the circulation and, if untreated, can often lead to severe multisystem complications. The field of rare Lipid Disorders is evolving and increasing awareness of these conditions, along with the systematic integration of recent advances or knowledge into clinical practice, is crucial to improve patient outcomes. The aim of this review is to provide an overview of selected rare genetic Lipid Disorders, focusing on the recommended diagnostic strategies and contemporary treatment and management options.

  • The role of patient registries for rare genetic Lipid Disorders.
    Current Opinion in Lipidology, 2018
    Co-Authors: David M. Ng, Matthew I. Bellgard, Amanda J. Hooper, John R. Burnett
    Abstract:

    Purpose of review We review the role, utility and current status of patient registries for rare genetic Lipid Disorders. Recent findings The creation and maintenance of rare genetic Lipid disorder patient registries is critical for disease monitoring, improving clinical best practice, facilitating research and enabling the development of novel therapeutics. An open-source disease registry platform, termed the Rare Disease Registry Framework, has been developed, optimized and deployed for homozygous familial hypercholesterolemia. A global disease-specific registry for lipoprotein lipase deficiency (LPLD), GENetherapy In the mAnagement of Lipoprotein Lipase deficiency, has been established with the aim of enrolling 20–40% of LPLD patients worldwide and will study the natural history of LPLD as well as therapeutic response to the gene therapy alipogene tiparvovec. Similarly, a registry for lysosomal acid lipase deficiency patients in Europe and the United States is studying the clinical outcomes of the enzyme-replacement therapy sebelipase alfa. Summary There are currently few disease-specific rare Lipid disorder patient registries. The very nature of rare genetic Lipid Disorders would suggest that larger national or international registries are necessary to capture clinical data on a sufficient number of patients to provide insight into the prevalence and natural history of these conditions. Furthermore, these registries can help to identify and address deficiencies in current diagnostic and management practices, and facilitate clinical trials of new therapies.

  • Screening for Lipid Disorders.
    Pathology, 2012
    Co-Authors: Damon A. Bell, Amanda J. Hooper, Bender Robert, Glenn Edwards, Frank M. Van Bockxmeer, Gerald F. Watts, John R. Burnett
    Abstract:

    Summary Lipid Disorders, also known as dysLipidaemias, are abnormalities of lipoprotein metabolism and include elevations of total cholesterol, low density lipoprotein (LDL) cholesterol, and triglyceride, and reductions in high density lipoprotein (HDL) cholesterol, and can be acquired or familial in nature. DysLipidaemia is a major risk factor for coronary heart disease and cardiovascular disease (CVD), which is the leading cause of morbidity and mortality in Australia. DysLipidaemia is defined by laboratory testing and using statistically determined criteria. Although the benefits of detecting and treating dysLipidaemia in patients with known CVD is clear, controversy remains regarding screening asymptomatic individuals who are not known to be at increased cardiovascular risk. This review examines the role of screening in the detection and treatment of individuals with Lipid Disorders.

Jean-pierre Després - One of the best experts on this subject based on the ideXlab platform.

  • Treatment of Lipid Disorders in obesity
    Expert review of cardiovascular therapy, 2011
    Co-Authors: Serena Tonstad, Jean-pierre Després
    Abstract:

    Obesity is the most common cause of secondary hyperLipidemia. Atherogenic dysLipidemia refers to elevated triglycerides, low HDL-cholesterol and small dense LDL associated with visceral obesity and metabolic syndrome. Obesity may also be associated with isolated low HDL-cholesterol or high triglycerides and postprandial hyperLipidemia. While some obese patients have high LDL cholesterol concentrations, obesity has a more pronounced effect on other atherogenic Lipids and lipoproteins. Obesity may aggravate familial Lipid Disorders. Lipid Disorders in obesity are responsive to weight loss, pharmacotherapy and weight loss surgery. Statins are the Lipid-lowering drug of choice, together with lifestyle change. Hard clinical end point data to support combinations of statins with other drugs is lacking. After weight loss surgery, the absolute risk of cardiovascular disease should be reassessed, but tools to facilitate risk assessment need to be developed.

Damon A. Bell - One of the best experts on this subject based on the ideXlab platform.

  • Update on the diagnosis, treatment and management of rare genetic Lipid Disorders
    Pathology, 2018
    Co-Authors: John R. Burnett, Damon A. Bell, Robert A. Hegele, Amanda J. Hooper
    Abstract:

    Summary Rare genetic Lipid Disorders affect the levels of cholesterol and/or triglyceride in the circulation and, if untreated, can often lead to severe multisystem complications. The field of rare Lipid Disorders is evolving and increasing awareness of these conditions, along with the systematic integration of recent advances or knowledge into clinical practice, is crucial to improve patient outcomes. The aim of this review is to provide an overview of selected rare genetic Lipid Disorders, focusing on the recommended diagnostic strategies and contemporary treatment and management options.

  • Screening for Lipid Disorders.
    Pathology, 2012
    Co-Authors: Damon A. Bell, Amanda J. Hooper, Bender Robert, Glenn Edwards, Frank M. Van Bockxmeer, Gerald F. Watts, John R. Burnett
    Abstract:

    Summary Lipid Disorders, also known as dysLipidaemias, are abnormalities of lipoprotein metabolism and include elevations of total cholesterol, low density lipoprotein (LDL) cholesterol, and triglyceride, and reductions in high density lipoprotein (HDL) cholesterol, and can be acquired or familial in nature. DysLipidaemia is a major risk factor for coronary heart disease and cardiovascular disease (CVD), which is the leading cause of morbidity and mortality in Australia. DysLipidaemia is defined by laboratory testing and using statistically determined criteria. Although the benefits of detecting and treating dysLipidaemia in patients with known CVD is clear, controversy remains regarding screening asymptomatic individuals who are not known to be at increased cardiovascular risk. This review examines the role of screening in the detection and treatment of individuals with Lipid Disorders.

Lucy N. Marion - One of the best experts on this subject based on the ideXlab platform.

  • Screening for Lipid Disorders in Children: US Preventive Services Task Force Recommendation Statement
    Pediatrics, 2007
    Co-Authors: Ned Calonge, Diana B. Petitti, Thomas G. Dewitt, Leon Gordis, Kimberly D. Gregory, Russell Harris, Kenneth W. Kizer, Michael L. Lefevre, Carol Loveland-cherry, Lucy N. Marion
    Abstract:

    The US Preventive Services Task Force (USPSTF) concludes that the evidence is insufficient to recommend for or against routine screening for Lipid Disorders in infants, children, adolescents, or young adults (up to age 20) (I recommendation). ### Importance There is good evidence that children with Lipid Disorders (dysLipidemia) are at risk for becoming adults with Lipid Disorders. ### Detection For children with familial dysLipidemia, the group most likely to benefit from screening, use of family history in screening may be inaccurate because of variability of definitions and unreliability of information. Serum Lipid levels are accurate screening tests for childhood dysLipidemia, although many children with multifactorial types of dysLipidemia would have normal Lipid levels in adulthood. Fifty percent of children and adolescents with dysLipidemia will have dysLipidemia as adults. ### Benefits of Detection and Early Treatment* Trials of statin drugs in children with monogenic dysLipidemia (defined below in “Clinical Considerations”) indicate improved total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), and high-density lipoprotein cholesterol (HDL-C) measures. For children with multifactorial types of dysLipidemia, there is no evidence that diet or exercise interventions in childhood lead to improved Lipid profiles or better health outcomes in adulthood. ### Harms of Detection and Early Treatment Potential harms of screening may include labeling of children whose dysLipidemia would not persist into adulthood or cause health problems, although evidence is lacking. Adverse effects from Lipid-lowering medications and low-fat diets, including potential long-term harms, have been inadequately evaluated in children. ### USPSTF Assessment The USPSTF was unable to determine the balance between potential benefits and harms for routinely screening children and adolescents for dysLipidemia. Address correspondence to Ned Calonge, MD, MPH, Colorado Department of Public Health and Environment, 4300 Cherry Creek Dr S, Denver, CO 80246. E-mail: ned.calonge{at}state.co.us