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This condition is characterised by raised triglyceride and cholesterol levels
due to abnormally high concentration of IDL and chylomicrons. It is inherited
as an autosomal recessive trait.
This is a type III hyperlipidaemia according to the WHO classification.
This disease presents in early adult life and is often associated with obesity,
glucose intolerance and hyperuricaemia. Yellow palmar creases, palmer xanthomas
and tuberoeruptive xanthomas may be present. There is an increased risk of coronary
artery disease (this condition may be found in approximately 3% of survivors
of myocardial infarctions) and peripheral vascular disease.
Diagnosis:
Type III hyperlipoproteinaemia (remnant removal disease) should be suspected
in an individual with (1):
- mixed hyperlipidaemia (serum total cholesterol >8 mmol/l and serum triglyceride
>5 mmol/l) - note though that generally lipid levels are markedly raised
(cholesterol 7-12 mmol/L and triglycerides 5-20 mmol/L);often the molar
concentrations of cholesterol and triglycerides are similar in type III
hyperlipidaemia
- palmar striae and/or tuberoeruptive xanthomata, remnant (floating Beta)
lipoprotein, or defective apo E isoforms
Management:
- seek specialist advice
- statins are not generally very effective in the management of a type III
hyperlipidaemia because of the mechanism of the hyperlipidaemia being related
to excess VLDL particles (see linked item). First-line treatment is usually
with a fibrate or fish oil
Reference:
- Durrington, PN (1995). Hyperlipidaemia: diagnosis and management. 2nd edition.
Butterworth-Heinemann Limited.
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