Improved survival of patients with homozygous familial hypercholesterolaemia treated with plasma exchange.
Plasma exchange was undertaken in five patients with homozygous familial hypercholesterolaemia at intervals of two weeks for a mean of 8.4 years. These patients had survived an average of 5.5 years longer than their five respective homozygous siblings (p = 0.3), each of whom must have had a matching genetic defect but who died untreated. The 37% decrease in peak serum cholesterol concentrations maintained by plasma exchange presumably reduced progression of atherosclerosis in the treated patients and thus lessened their risk of premature death.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D005260:Female; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000401:mortality / Q000628:therapy*; D008055:Lipids / Q000097:blood; D008297:Male; D010951:Plasma Exchange; D011973:Receptors, LDL / Q000032:analysis
D008055:Lipids; D011973:Receptors, LDL