Statins in homozygous familial hypercholesterolemia

@article{Marais2002StatinsIH,
  title={Statins in homozygous familial hypercholesterolemia},
  author={Adrian D Marais and Dirk Jacobus Blom and Jean Catherine Firth},
  journal={Current Atherosclerosis Reports},
  year={2002},
  volume={4},
  pages={19-25}
}
Homozygous familial hypercholesterolemia is a rare disorder resulting in severe premature atherosclerosis. Drug therapy was previously viewed as inadequate for control of the dyslipidemia, so portacaval shunting, plasmapheresis, and liver transplantation were undertaken to treat this condition. Despite these drastic measures, additional cholesterol-lowering treatment may still be required. Furthermore, there is a need for pharmacologic control until additional measures can be undertaken. The… 
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38 Citations
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38 Citations

Lipoprotein metabolism in familial hypercholesterolemia
Current Treatment of Famil ial Hypercholesterolaemia
TLDR
There is a 20-fold increase in risk of premature coronary heart disease (CHD) in untreated patients compared to control and HeFH patients usually develop CHD before age 55 and 60 respectively for men and women without treatment.
Homozygous familial hypercholesterolemia: current perspectives on diagnosis and treatment.
Current management of severe homozygous hypercholesterolaemias
TLDR
Patients with severe homozygous hypercholesterolaemia illustrate the natural history of atherosclerosis within a condensed timeframe as effective cholesterol-lowering treatment started in early childhood is essential to prevent onset of life-threatening atherosclerotic involvement of the aortic root and valve, and the coronary arteries.
Review of the scientific evolution of gene therapy for the treatment of homozygous familial hypercholesterolaemia: past, present and future perspectives
TLDR
The genetic basis of the FH disease is reviewed, paying special attention to the severe HoFH as well as the challenges in its diagnosis and clinical management, and the current therapies for this disease are discussed.
Treatment of homozygous familial hypercholesterolemia
TLDR
Familial hypercholesterolemia results from gene mutations approximately halving the number of functional LDL receptors in heterozygotes and a greater lack in homozygotes, and inhibitors of LDL synthesis, lomitapide and mipomersen, are new therapeutic options.
Reduction in Mortality in Subjects With Homozygous Familial Hypercholesterolemia Associated With Advances in Lipid-Lowering Therapy
TLDR
Lipid-lowering therapy is associated with delayed cardiovascular events and prolonged survival in patients with homozygous familial hypercholesterolemia, and this occurred despite a mean reduction in low-density lipoprotein cholesterol.
Evinacumab: a new option in the treatment of homozygous familial hypercholesterolemia
TLDR
Treatment of HoFH using a monoclonal antibody targeting ANGPTL3 with evinacumab halves LDL-C levels in HoFH patients by an LDLR-independent mechanism is indicated, indicating a promising improvement in the management of these patients.
Familial hypercholesterolemia and estimation of US patients eligible for low-density lipoprotein apheresis after maximally tolerated lipid-lowering therapy.
Nuevos fármacos para el tratamiento de la hipercolesterolemia
TLDR
Long-term studies are needed to evaluate the safety, effectiveness and impact on cardiovascular risk reduction of new lipid-lowering agents developed to reduce LDL-cholesterol in patients with severe hypercholesterolemia and statin-intolerant patients.
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References

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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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