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European Journal of Pediatrics

, Volume 149, Issue 10, pp 716–721 | Cite as

Low-density lipoprotein plasmaphaeresis with and without lovastatin in the treatment of the homozygous form of familial hypercholesterolaemia

  • J. Thiery
  • A. K. Walli
  • G. Janning
  • D. Seidel
Metabolic Diseases

Abstract

A 7-year-old girl with homozygous familial hypercholesterolaemia and plasma low-density lipoprotein (LDL)-cholesterol levels of 820 mg/dl (21.2 mmol/l) and progressive xanthomata was treated with heparin extracorporeal low-density lipoprotein precipitation (HELP) to lower her plasma LDL. On weekly HELP treatment she maintained her pre-HELP treatment LDL-cholesterol levels at 409 mg/dl (10.6 mmol/l). The long-term HELP treatment was well tolerated and led to regression of her xanthomata. Subsequently, lovastatin [Mevacor; Merck Sharp & Dohme, Westpoint, Pa., USA (20 mg/day)] was added to the regimen, causing a further 20% decrease in her pre-HELP treatment plasma LDL-cholesterol levels. Lovastatin alone did not sufficiently lower her plasma LDL and could not replace the weekly HELP therapy. Our data show that lovastatin is an effective adjunctive therapy for lowering plasma LDL-cholesterol in a homozygous patient, once plasma LDL levels have already been lowered by regular HELP treatment.

Key words

Homozygous familial hypercholesterolaemia low-density lipoprotein plasmaphaeresis Heparin extracorporeal low-density lipoprotein precipitation Lovastatin 

Abbreviations

ACTH

adrenocorticotropic hormone

FH

familial hypercholesterolaemia

HELP

heparin extracorporeal low-density lipoprotein precipitation

HMG-CoA

hydroxymethylglutaryl-CoA

LDL

low-density lipoprotein

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Copyright information

© Springer-Verlag 1990

Authors and Affiliations

  • J. Thiery
    • 1
  • A. K. Walli
    • 1
  • G. Janning
    • 2
  • D. Seidel
    • 1
  1. 1.Institut für Klinische ChemieKlinikum Großhadern der Universität MünchenMünchen 70Federal Republic of Germany
  2. 2.Abteilung für Nephrologie, Zentrum für Innere MedizinUniversität GöttingenGöttingenFederal Republic of Germany

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