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… 
Lipoprotein metabolism in familial hypercholesterolemia
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TLDR
Probucol appears to be the most satisfactory treatment for homozygous familial hypercholesterolaemia currently available and was well tolerated by child and adult patients.
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TLDR
The prevalences of homozygotes and heterozygotes with familial hypercholesterolaemia in Transvaal Afrikaners, calculated from this group of patients, were 1 in 30,000 and 1 in 100 respectively, which are the highest ever reported and may help to explain why South African whites have the highest death rate from coronary heart disease in the Western world.
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TLDR
Portacaval shunt has been effective therapy for patients with FH who were refractory or intolerant to medical treatment; it should be performed before the development of irreversible cardiovascular damage.
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TLDR
The results indicate that mevinolin provides a safe and highly effective means of reducing LDL levels in patients with heterozygous familial hypercholesterolaemia refractory to conventional treatment but is less useful in homozygotes.
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TLDR
Atorvastatin is a powerful and safe lipid-modifying agent for LDL cholesterol; it also modifies HDL cholesterol and triglyceride concentrations, and may suffice as a single agent for many subjects with heterozygous FH.
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