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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
medicine
Reduction in mortality in subjects with homozygous familial hypercholesterolemia associated with advances in lipid-lowering therapy
Circulation, Volume 124, No. 20, Year 2011
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Description
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<0.0001) with lipid-lowering therapy. Conclusions-: Lipid-lowering therapy is associated with delayed cardiovascular events and prolonged survival in patients with homozygous familial hypercholesterolemia. © 2011 American Heart Association. All rights reserved.
Authors & Co-Authors
Raal, Frederick Johan
South Africa, Johannesburg
School of Clinical Medicine
Pilcher, Gillian J.
South Africa, Johannesburg
School of Clinical Medicine
Panz, Vanessa R.
South Africa, Johannesburg
School of Clinical Medicine
van Deventer, Hendrick E.
South Africa, Johannesburg
University of the Witwatersrand
Brice, Brigitte C.
South Africa, Cape Town
University of Cape Town
Blom, Dirk J.
South Africa, Cape Town
University of Cape Town
Marais, Adrian David
South Africa, Cape Town
University of Cape Town
Statistics
Citations: 312
Authors: 7
Affiliations: 3
Identifiers
Doi:
10.1161/CIRCULATIONAHA.111.042523
ISSN:
00097322
e-ISSN:
15244539
Research Areas
Environmental
Noncommunicable Diseases
Study Design
Cohort Study
Study Locations
South Africa
Participants Gender
Female