Direct adsorption of lipoproteins from whole blood by direct adsorption of lipoprotein apheresis: first experience in two hypercholesterolemic children.
Low density lipoprotein (LDL) cholesterol apheresis, combined with lipid lowering drugs, provides a safe and effective means of improving the prognosis of patients with homozygote familial hypercholesterolemia, especially if started before the age of seven. The direct adsorption of lipoprotein (DALI) is the first extracorporeal low density lipoprotein removing system compatible with whole blood. The purpose of the present study was to clarify the efficacy and safety of DALI in children with homozygous familial hypercholesterolemia. Two boys, aged 9 and 15 years, with familial hypercholesterolemia, who were highly resistant to dietary regimes and to drug therapy, were treated with the low density lipoprotein adsorber DALI apheresis once every 2 weeks for 24 weeks. The treated blood volumes for each procedure were 2911 mL (493 +/- SD) and 5982 mL (1129 +/- SD), respectively. In our patients, the acute mean LDL cholesterol reductions were 44.7 +/- 8.9% and 58.8 +/- 4.5%. The corresponding reductions were 42.5 +/- 7.2% and 56 +/- 4.3% for total cholesterol, and 46.5 +/- 17.1% and 55 +/- 7.5% for very low density lipoprotein cholesterol (VLDL-C). There were insignificant losses of high density lipoprotein (12.2 +/- 5.7%, 8.3 +/- 5.5%). Treatment was well tolerated in general, and neither patient suffered from irreversible or long-lasting adverse effects. Our experience with DALI apheresis is encouraging. The present report is the first on the use of DALI in children. Based on this short-term evaluation we think that DALI might be safe and effective in children with homozygote familial hypercholesterolemia, however further evaluation of long-term effects is needed.
Bayrakci
Umut Selda
US
Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, Ankara, Turkey. ukavak@yahoo.com
Besbas
Nesrin
N
Ozcebe
Osman
O
Coskun
Turgay
T
Akgul
Ergan
E
Kutluk
Tezer
T
Bakkaloglu
Aysin
A
D000293:Adolescent; D000327:Adsorption; D001781:Blood Component Removal / Q000009:adverse effects / Q000379:methods*; D002648:Child; D002784:Cholesterol / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D015243:Cholesterol, VLDL / Q000097:blood; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000628:therapy*; D008074:Lipoproteins / Q000493:pharmacokinetics*; D008297:Male; D016896:Treatment Outcome
D008078:Cholesterol, LDL; D015243:Cholesterol, VLDL; D008074:Lipoproteins; D002784:Cholesterol