Alan Chait, MD; Robert H. Eckel, MD
Acknowledgment: The authors thank Brandie Trotter for assistance with Figure 1.
Disclosures: Dr. Chait reports grants from the National Institutes of Health and grants and personal fees from Medimmune and personal fees from Ionis Pharmaceuticals, outside the submitted work. Dr. Eckel reports grants from the National Institute of Neurological Disorders and Stroke, National Institute on Aging, National Heart, Lung, and Blood Institute, Ionis Pharmaceuticals, uniQure Biopharma, CTSA Pilot Project, and Endece and personal fees from Cardiometabolic Health Congress, Prime, Medtelligence, Medscape, Medical Education Resources, Vox Media, Sanofi/Regeneron, Merck, Novo Nordisk, and Kowa, outside the submitted work. Authors not named here have disclosed no conflicts of interest. Disclosures can also be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=M19-0203.
Editors' Disclosures: Christine Laine, MD, MPH, Editor in Chief, reports that her spouse has stock options/holdings with Targeted Diagnostics and Therapeutics. Darren B. Taichman, MD, PhD, Executive Editor, reports that he has no financial relationships or interests to disclose. Cynthia D. Mulrow, MD, MSc, Senior Deputy Editor, reports that she has no relationships or interests to disclose. Jaya K. Rao, MD, MHS, Deputy Editor, reports that she has stock holdings/options in Eli Lilly and Pfizer. Catharine B. Stack, PhD, MS, Deputy Editor, Statistics, reports that she has stock holdings in Pfizer, Johnson & Johnson, and Colgate-Palmolive. Christina C. Wee, MD, MPH, Deputy Editor, reports employment with Beth Israel Deaconess Medical Center. Sankey V. Williams, MD, Deputy Editor, reports that he has no financial relationships or interests to disclose. Yu-Xiao Yang, MD, MSCE, Deputy Editor, reports that he has no financial relationships or interest to disclose.
Corresponding Author: Alan Chait, MD, Department of Medicine, Division of Metabolism, Endocrinology and Nutrition, University of Washington, 750 Republican Street, Mailstop 358062, Seattle, WA 98109; e-mail, email@example.com.
Current Author Addresses: Dr. Chait: Department of Medicine, Division of Metabolism, Endocrinology and Nutrition, University of Washington, 750 Republican Street, Mailstop 358062, Seattle, WA 98109.
Dr. Eckel: University of Colorado Anschutz Medical Campus, 18582 17th Avenue, Mail Stop 8106, Aurora, CO 80045.
Author Contributions: Conception and design: A. Chait, R.H. Eckel.
Analysis and interpretation of the data: R.H. Eckel.
Drafting of the article: A. Chait.
Critical revision for important intellectual content: A. Chait, R.H. Eckel.
Final approval of the article: A. Chait, R.H. Eckel.
Collection and assembly of data: A. Chait, R.H. Eckel.
The chylomicronemia syndrome occurs when triglyceride levels are severely elevated (usually >16.95 mmol/L [1500 mg/dL]) and is characterized by such clinical features as abdominal pain, acute pancreatitis, eruptive xanthomas, and lipemia retinalis. It may result from 1 of 3 conditions: the presence of secondary forms of hypertriglyceridemia concurrent with genetic causes of hypertriglyceridemia, termed multifactorial chylomicronemia syndrome (MFCS); a deficiency in the enzyme lipoprotein lipase and some associated proteins, termed familial chylomicronemia syndrome (FCS); or familial partial lipodystrophy. Most chylomicronemia syndrome cases are the result of MFCS; FCS is very rare. In all these conditions, triglyceride-rich lipoproteins accumulate because of impaired plasma clearance. This review describes the 3 major causes of the chylomicronemia syndrome; their consequences; and the approaches to treatment, which differ considerably by group.
Chait A, Eckel RH. The Chylomicronemia Syndrome Is Most Often Multifactorial: A Narrative Review of Causes and Treatment. Ann Intern Med. 2019;170:626–634. [Epub ahead of print 30 April 2019]. doi: https://doi.org/10.7326/M19-0203
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Published: Ann Intern Med. 2019;170(9):626-634.
Published at www.annals.org on 30 April 2019
Cardiology, Coronary Risk Factors, Dyslipidemia, Hospital Medicine.
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