Severe hypertriglyceridemia with pancreatitis: thirteen years' treatment with lomitapide

JAMA Internal Medicine
Frank M SacksRobert A Hegele

Abstract

Recurrent pancreatitis is a potentially fatal complication of severe hypertriglyceridemia. Genetic defects and lifestyle risk factors may render this condition unresponsive to current treatments. We report this first case of long-term management of intractable near-fatal recurrent pancreatitis secondary to severe hypertriglyceridemia by a novel use of lomitapide, an inhibitor of microsomal triglyceride transfer protein, recently approved for treatment of familial homozygous hypercholesterolemia. The patient had been hospitalized many times for pancreatitis since age 15 years. Her serum triglyceride level averaged 3900 mg/dL while she received therapy with approved lipid drugs. She is homozygous for a coding mutation (P234L) in lipoprotein lipase, leaving her unable to metabolize triglycerides in chylomicrons and very low density lipoproteins (VLDL). Lomitapide reduces the secretion of chylomicrons and VLDL. Lomitapide, which was started when she was 44 years old after near-fatal pancreatitis, lowered her fasting triglyceride level from greater than 3000 mg/dL to a mean (SD) of 903 (870) mg/dL while she received 30 mg/d and to 524 (265) mg/dL while she received 40 mg/d; eliminated chronic abdominal pain; and prevented pancreatit...Continue Reading

Citations

Oct 2, 2014·European Journal of Internal Medicine·Pedro ValdivielsoNils Ewald
Nov 10, 2015·Clinical Therapeutics·Damon A Bell, Gerald F Watts
Dec 17, 2014·Journal of the American College of Cardiology·Robert S RosensonDaniel Gaudet
Mar 20, 2016·Current Atherosclerosis Reports·Jeffrey E AlfonsiSteven E Gryn
Mar 5, 2016·Expert Review of Clinical Pharmacology·Daniela NeefIoanna Gouni-Berthold
Mar 26, 2016·The Lancet. Diabetes & Endocrinology·Ezim Ajufo, Daniel J Rader
Mar 26, 2016·Korean Circulation Journal·Seung Hwan HanKwang Kon Koh
Dec 17, 2014·Journal of Clinical Lipidology·Nikroo HashemiDavid E Cohen
Jan 19, 2016·Current Opinion in Lipidology·Steven E Gryn, Robert A Hegele
Mar 4, 2015·Nature Reviews. Endocrinology·Amanda J Brahm, Robert A Hegele
May 6, 2015·Atherosclerosis. Supplements·Claudia Stefanutti, Ulrich Julius
Aug 2, 2014·Progress in Lipid Research·Amirhossein SahebkarGerald F Watts
Sep 14, 2016·Current Treatment Options in Cardiovascular Medicine·Justin ParizoJoshua W Knowles
Oct 4, 2016·Critical Reviews in Clinical Laboratory Sciences·Meghan T Walsh, M Mahmood Hussain
Oct 28, 2016·Expert Opinion on Investigational Drugs·Amanda J Brahm, Robert A Hegele
Nov 20, 2016·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Elizabeth P NewberryNicholas O Davidson
Mar 4, 2017·American Journal of Cardiovascular Drugs : Drugs, Devices, and Other Interventions·Xin LiuGeorge Liu
Apr 30, 2015·Journal of Lipid Research·Saho MakishimaSadahiko Iwamoto
Jun 10, 2017·Expert Opinion on Pharmacotherapy·Amanda J Berberich, Robert A Hegele
Apr 17, 2018·The Journal of Clinical Endocrinology and Metabolism·Nivedita PatniAbhimanyu Garg
May 31, 2018·Expert Review of Clinical Pharmacology·Rabia ChaudhryAnthony S Wierzbicki
Sep 6, 2018·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·James M Falko
Oct 30, 2015·Circulation·Samuel S GiddingUNKNOWN American Heart Association Atherosclerosis, Hypertension, and Obesity in the Young Committee of the Council on Cardiovascula
Aug 30, 2014·Circulation Research·Murray W HuffRobert A Hegele
Sep 18, 2015·Arteriosclerosis, Thrombosis, and Vascular Biology·Robert A HegeleFrancine K Welty
Oct 22, 2015·Circulation. Cardiovascular Genetics·John R Burnett, Robert A Hegele
Feb 15, 2019·Drug Safety : an International Journal of Medical Toxicology and Drug Experience·Lauren PavlikNaga P Chalasani
Jul 18, 2019·The New England Journal of Medicine·Jay H Hoofnagle, Einar S Björnsson
Apr 5, 2014·Clinical Pharmacology and Therapeutics·R HajhosseinyA S Wierzbicki
Dec 4, 2014·Clinical Medicine : Journal of the Royal College of Physicians of London·Anthony S WierzbickiMfon Ewang-Emukowhate
Nov 23, 2019·Expert Opinion on Investigational Drugs·David RhaindsJean-Claude Tardif
Sep 29, 2017·Current Opinion in Lipidology·Anthony C Keech, Alicia J Jenkins
Aug 28, 2019·Journal of Internal Medicine·Mats Eriksson, Bo Angelin
Apr 2, 2020·Frontiers in Endocrinology·Priska StahelGary F Lewis
Feb 8, 2018·Annual Review of Medicine·Ezim Ajufo, Daniel J Rader
Apr 27, 2017·Annals of Pediatric Endocrinology & Metabolism·Mo Kyung JungHo-Seong Kim
Oct 17, 2018·Current Diabetes Reports·Om Ganda
May 27, 2020·Current Opinion in Cardiology·Ahmed HandhleAnthony S Wierzbicki
Nov 17, 2020·Frontiers in Endocrinology·Ronald B Goldberg, Alan Chait

❮ Previous
Next ❯

Related Concepts

Related Feeds

ApoE, Lipids & Cholesterol

Serum cholesterol, triglycerides, apolipoprotein B (APOB)-containing lipoproteins (very low-density lipoprotein (VLDL), immediate-density lipoprotein (IDL), and low-density lipoprotein (LDL), lipoprotein A (LPA)) and the total cholesterol/high-density lipoprotein (HDL) cholesterol ratio are all connected in diseases. Here is the latest research.

Anthelmintics

Anthelmintics or antihelminthics are a group of antiparasitic drugs that expel parasitic worms (helminths) and other internal parasites from the body by either stunning or killing them and without causing significant damage to the host. Discover the latest research on anthelmintics here.

Related Papers

International Journal of Cardiology
Genovefa D KolovouKatherine K Anagnostopoulou
Therapeutic Apheresis and Dialysis : Official Peer-reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy
C StefanuttiC Morozzi
© 2021 Meta ULC. All rights reserved