Severe hypertriglyceridemia with pancreatitis: Thirteen years' treatment with lomitapide

JAMA Internal Medicine

Volumn 174, Issue 3, 2014, Pages 443-447

  Sacks, Frank M ^a,b,c   Stanesa, Maxine ^d   Hegele, Robert A ^e  

^a HARVARD SCHOOL OF PUBLIC HEALTH   (United States)

^b BRIGHAM AND WOMEN S HOSPITAL   (United States)

^c HARVARD MEDICAL SCHOOL   (United States)

^d HARVARD VANGUARD MEDICAL ASSOCIATES   (United States)

^e ROBARTS RESEARCH INSTITUTE   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

ALKALINE PHOSPHATASE; APOLIPOPROTEIN B; APOLIPOPROTEIN B100; APOLIPOPROTEIN B48; APOLIPOPROTEIN C2; ASPARTATE AMINOTRANSFERASE; CHYLOMICRON; FENOFIBRATE; GEMFIBROZIL; GENOMIC DNA; HIGH DENSITY LIPOPROTEIN CHOLESTEROL; LIPOPROTEIN LIPASE; LOMITAPIDE; LOW DENSITY LIPOPROTEIN CHOLESTEROL; NICOTINIC ACID; OMEGA 3 FATTY ACID; TRIACYLGLYCEROL; VERY LOW DENSITY LIPOPROTEIN;

ABDOMINAL PAIN; ADULT; ADULT RESPIRATORY DISTRESS SYNDROME; ALANINE AMINOTRANSFERASE BLOOD LEVEL; ALKALINE PHOSPHATASE BLOOD LEVEL; ANEMIA; ARTICLE; ASPARTATE AMINOTRANSFERASE BLOOD LEVEL; BONE MARROW; BONE MARROW BIOPSY; CASE REPORT; CHOLESTASIS; CHOLESTEROL BLOOD LEVEL; CHRONIC PAIN; CLINICAL EVALUATION; DIET RESTRICTION; DISEASE COURSE; DISEASE DURATION; DRUG DOSE REGIMEN; DRUG DOSE TITRATION; DRUG TREATMENT FAILURE; FAT CONTENT; FATTY LIVER; FEMALE; GENE SEQUENCE; GENETIC CODE; HEMATOCRIT; HEPATITIS; HEPATOMEGALY; HOMOZYGOSITY; HOSPITAL ADMISSION; HOSPITALIZATION; HUMAN; HYPERTRIGLYCERIDEMIA; HYPOTENSION; KUPFFER CELL; LEUKOCYTE COUNT; LEUKOPENIA; LIPID METABOLISM; LIPOPROTEIN BLOOD LEVEL; LIPOPROTEIN SECRETION; LIVER BIOPSY; LIVER CELL; LIVER FIBROSIS; LIVER TOXICITY; LONG TERM CARE; LOW FAT DIET; MISSENSE MUTATION; MONOTHERAPY; PANCREATITIS; PHENOTYPE; PREGNANCY; PRIORITY JOURNAL; RECURRENT DISEASE; RESPIRATORY TRACT INTUBATION; RETICULOENDOTHELIAL SYSTEM; SIDE EFFECT; SPLENOMEGALY; SPONTANEOUS ABORTION; THROMBOCYTE COUNT; THROMBOCYTOPENIA; TREATMENT DURATION; TRIACYLGLYCEROL BLOOD LEVEL;

ADULT; ANTICHOLESTEREMIC AGENTS; BENZIMIDAZOLES; FEMALE; HUMANS; HYPERTRIGLYCERIDEMIA; PANCREATITIS, CHRONIC; PREGNANCY; RECURRENCE; TREATMENT OUTCOME;

EID: 84897584807     PISSN: 21686106     EISSN: None     Source Type: Journal    
DOI: 10.1001/jamainternmed.2013.13309     Document Type: Article

References (15)

1. Brunzell JD. Clinical practice: hypertriglyceridemia. N Engl J Med. 2007;357(10):1009-1017.
2. Tsuang W, Navaneethan U, Ruiz L, Palascak JB, Gelrud A. Hypertriglyceridemic pancreatitis: presentation and management. Am J Gastroenterol. 2009;104(4):984-991.
3. Cappell MS. Acute pancreatitis: etiology, clinical presentation, diagnosis, and therapy. Med Clin North Am. 2008;92(4):889-923, ix-x. (Pubitemid 351822364)
4. Berglund L, Brunzell JD, Goldberg AC, et al Endocrine Society. Evaluation and treatment of hypertriglyceridemia: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2012;97(9):2969-2989.
5. Johansen CT, Hegele RA. Genetic bases of hypertriglyceridemic phenotypes. Curr Opin Lipidol. 2011;22(4):247-253.
6. Wetterau JR, Aggerbeck LP, Bouma ME, et al. Absence of microsomal triglyceride transfer protein in individuals with abetalipoproteinemia. Science. 1992;258(5084):999-1001.
7. Ma Y, Henderson HE, Murthy V, et al. A mutation in the human lipoprotein lipase gene as the most common cause of familial chylomicronemia in French Canadians. N Engl J Med. 1991;324(25):1761-1766.
8. Yang Y, Mu Y, Zhao Y, et al. Genetic screening of the lipoprotein lipase gene for mutations in Chinese subjects with or without hypertriglyceridemia. J Genet Genomics. 2007;34(5):381-391. (Pubitemid 46874279)
9. Zambon A, Schmidt I, Beisiegel U, Brunzell JD. Dimeric lipoprotein lipase is bound to triglyceride-rich plasma lipoproteins. J Lipid Res. 1996;37(11):2394-2404. (Pubitemid 26427453)
10. Joy TR, Hegele RA. Microsomal triglyceride transfer protein inhibition: friend or foe? Nat Clin Pract Cardiovasc Med. 2008;5(8):506-508.
11. Fraser R, Cogger VC, Dobbs B, et al. The liver sieve and atherosclerosis. Pathology. 2012;44(3):181-186.
12. Miyazaki K, Miwa S, Kodama H, et al. Hepatic and intestinal changes in rats treated with T-0126, a microsomal triglyceride transfer protein (mtp) inhibitor. J Toxicol Sci. 2007;32(2):161-177. (Pubitemid 46849773)
13. Cuchel M, Meagher EA, du Toit Theron H, et al; Phase 3 HoFH Lomitapide Study Investigators. Efficacy and safety of amicrosomal triglyceride transfer protein inhibitor in patients with homozygous familial hypercholesterolaemia: a single-arm, open-label, phase 3 study. Lancet. 2013;381(9860):40-46.
14. Samaha FF, McKenney J, Bloedon LT, Sasiela WJ, Rader DJ. Inhibition ofmicrosomal triglyceride transfer protein alone or with ezetimibe in patients with moderate hypercholesterolemia. Nat Clin Pract Cardiovasc Med. 2008;5(8):497-505.
15. Sandhu S, Al-Sarraf A, Taraboanta C, Frohlich J, Francis GA. Incidence of pancreatitis, secondary causes, and treatment of patients referred to a specialty lipid clinic with severe hypertriglyceridemia: a retrospective cohort study. Lipids Health Dis. 2011;10:157.