Liver transplantation in patients with homozygotic familial hypercholesterolemia previously treated by end-to-side portocaval shunt and ileal bypass

López‐Santamaría, Manuel; Migliazza, Lucia; Gámez, M; Murcia, J; Díaz-González, Mercedes; Camarena, C.; Hierro, Loreto; Vega, A. de la; Frauca, E; Díaz, María Eugenia López-Jacoiste; Jara, Paloma; Tovar, Juan A.

doi:10.1053/jpsu.2000.0350630

Cited by 55 publications

(37 citation statements)

References 11 publications

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“…22 Liver transplantation is restricted by a lack of donor organs and the need for continuous postoperative immunosuppression. 23 LDL apheresis significantly lowers LDL-C and is considered the standard of care for patients with HoFH. 24 This procedure reduces the risk of coronary heart disease in patients with heterozygous FH.…”

Section: Discussionmentioning

confidence: 99%

Reduction in Mortality in Subjects With Homozygous Familial Hypercholesterolemia Associated With Advances in Lipid-Lowering Therapy

et al. 2011

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Background-Homozygous familial hypercholesterolemia is an inherited disorder caused by mutations in both low-density lipoprotein receptor alleles, which results in extremely elevated plasma low-density lipoprotein cholesterol concentrations and very early morbidity and mortality due to cardiovascular disease. Methods and Results-To evaluate the impact of advances in lipid-lowering (predominantly statin) therapy on cardiovascular disease morbidity and mortality in a large cohort of patients with homozygous familial hypercholesterolemia, the records of 149 patients (81 females, 68 males) from 2 specialized lipid clinics in South Africa were evaluated retrospectively. Homozygous familial hypercholesterolemia was diagnosed by confirmation of mutations in genes affecting low-density lipoprotein cholesterol or by clinical criteria. A Cox proportional hazard model with time-varying exposure was used to estimate the risk of death and major adverse cardiovascular events among statin-treated patients compared with statin-naive patients. The hazard ratio for benefit from lipid therapy, calculated with the Cox proportional hazards model for the end point of death, was 0.34 (95% confidence interval 0.14 -0.86; Pϭ0.02), and for the end point of major adverse cardiovascular events, it was 0.49 (95% confidence interval 0. Clinical Perspective on p 2207The frequency of FH throughout the world has been estimated at 1 in 500 people in the less severe heterozygous form and at 1 per 1 million people in the more severe homozygous form. 1 In South African white Afrikaners, there is a much higher prevalence of heterozygous and homozygous FH, estimated at 1:100 and 1:30 000, respectively. 3 This high prevalence is due to a founder effect that occurred when a limited number of LDL receptor mutations were introduced by Dutch families who settled in the Cape Province during the second half of the seventeenth century. 4 Until the 1980s, treatment of FH was limited to a low-fat diet and minimally effective lipid-modifying agents. Lipidlowering drug therapy changed radically in the 1990s with the introduction of the 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase inhibitors, or statins, a drug class that is remarkably effective in lowering LDL-C. 5 Multiple ranContinuing medical education (CME) credit is available for this article. Go to http://cme.ahajournals.org to take the quiz. The aims of the present study were to assess the impact of modern lipid-lowering therapy, predominantly statin therapy, on all-cause and cardiovascular mortality in a large cohort of HoFH patients who have been followed up for up to 40 years at 2 specialized lipid treatment centers in South Africa. Methods PatientsThis study was a retrospective cohort design that reviewed data from July 1972, the time of inception of the first specialized lipid clinics in South Africa, to March 2009. Medical records of HoFH subjects were reviewed to establish genetic and phenotypic data, anthropometric measures, and data on cardiovascular events and lipidlowering drug...

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Section: Discussionmentioning

confidence: 99%

Reduction in Mortality in Subjects With Homozygous Familial Hypercholesterolemia Associated With Advances in Lipid-Lowering Therapy

et al. 2011

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“…Thus, the liver transplantation can offer an efficient treatment for homozygous familial hypercholesterolemia [63][64][65].…”

Section: Liver Transplantationmentioning

confidence: 99%

Microsomal Transfer Protein Inhibitors, New Approach for Treatment of Familial Hypercholesterolemia, Review of the Literature, Original Findings, and Clinical Significance

Kolovou

Vasiliadis

Gontoras

et al. 2015

Cardiovascular Therapeutics

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SUMMARYThe genetic causes of cholesterol metabolism disorders usually lead to premature atherosclerosis. The most well recognized genetically caused hypercholesterolemia is familial hypercholesterolemia. Although the disease is well known, as the discovery of low-density lipoprotein receptor, the classical treatment with lipid-lowering drugs (statins, fibrates, ezetimibe, colesevelam) is still not adequate and new options are seeking. This review is an attempt to analyze the microsomal transfer protein (MTP) inhibitors as a new approach for treatment of familial hypercholesterolemia, to reviews the literature according to MTP inhibitors and finally to provide original findings.

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“…Other aggressive therapeutic options include portacaval shunting and liver transplantation. 10,12 Ezetimibe is the first of a new class of cholesterol absorption inhibitors that potently inhibits dietary and biliary cholesterol absorption at the brush border of the intestine without affecting the absorption of triglycerides or fat-soluble vitamins. [13][14][15] Ezetimibe is absorbed rapidly, extensively conjugated to glucuronide in the intestine, and circulated enterohepatically.…”

mentioning

confidence: 99%

Efficacy and Safety of Ezetimibe Coadministered With Atorvastatin or Simvastatin in Patients With Homozygous Familial Hypercholesterolemia

2002

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Background-Patients with homozygous familial hypercholesterolemia (HoFH) have a high incidence of cardiovascular morbidity and mortality from premature atherosclerosis, and the efficacy of pharmacological therapy has been limited. We evaluated the efficacy, safety, and tolerability of ezetimibe, a novel cholesterol absorption inhibitor, in a multicenter, double-blind, randomized trial of HoFH patients receiving atorvastatin or simvastatin.

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Liver transplantation in patients with homozygotic familial hypercholesterolemia previously treated by end-to-side portocaval shunt and ileal bypass

Cited by 55 publications

References 11 publications

Reduction in Mortality in Subjects With Homozygous Familial Hypercholesterolemia Associated With Advances in Lipid-Lowering Therapy

Reduction in Mortality in Subjects With Homozygous Familial Hypercholesterolemia Associated With Advances in Lipid-Lowering Therapy

Microsomal Transfer Protein Inhibitors, New Approach for Treatment of Familial Hypercholesterolemia, Review of the Literature, Original Findings, and Clinical Significance

Efficacy and Safety of Ezetimibe Coadministered With Atorvastatin or Simvastatin in Patients With Homozygous Familial Hypercholesterolemia

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