Reduction in Mortality in Subjects With Homozygous Familial Hypercholesterolemia Associated With Advances in Lipid-Lowering Therapy
| Autor: | Dirk J. Blom, Hendrick E. van Deventer, Vanessa R. Panz, Gillian J. Pilcher, A. David Marais, Frederick J. Raal, Brigitte C. Brice |
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| Rok vydání: | 2011 |
| Předmět: |
Adult
Male medicine.medical_specialty Statin Adolescent medicine.drug_class Mipomersen Familial hypercholesterolemia Cohort Studies Hyperlipoproteinemia Type II Young Adult chemistry.chemical_compound Physiology (medical) Internal medicine medicine Humans Child Retrospective Studies Proportional hazards model business.industry Cholesterol Homozygote Retrospective cohort study Cholesterol LDL Middle Aged medicine.disease Lomitapide Endocrinology chemistry Female lipids (amino acids peptides and proteins) Hydroxymethylglutaryl-CoA Reductase Inhibitors Cardiology and Cardiovascular Medicine business Cohort study |
| Zdroj: | Circulation. 124:2202-2207 |
| ISSN: | 1524-4539 0009-7322 |
| DOI: | 10.1161/circulationaha.111.042523 |
| Popis: | 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; P =0.02), and for the end point of major adverse cardiovascular events, it was 0.49 (95% confidence interval 0.22–1.07; P =0.07). This occurred despite a mean reduction in low-density lipoprotein cholesterol of only 26.4% (from 15.9±3.9 to 11.7±3.4 mmol/L; P Conclusions— Lipid-lowering therapy is associated with delayed cardiovascular events and prolonged survival in patients with homozygous familial hypercholesterolemia. |
| Databáze: | OpenAIRE |
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