Long-term prevention of premature coronary atherosclerosis in homozygous familial hypercholesterolemia

Beate Roxane Jaeger, Theoharis Tsobanelis, Frank Bengel, Markus Schwaiger, Dietrich Seidel

Research output: Contribution to journalArticlepeer-review

Abstract

Homozygous familial hypercholesterolemia (FHH) leads to severe premature atherosclerosis. A 22-year-old woman with FHH has been treated with a combination of H.E.L.P. apheresis (heparin-mediated extracorporeal LDL precipitation) and statins for 15 years. The combined treatment maintained a plasma LDL-cholesterol reduction from baseline of 840 to 122 mg/dL (85% reduction). In addition, H.E.L.P. apheresis reduced the elevated lipoprotein(a) and fibrinogen levels by 60% to 70%. All xanthomata disappeared. There is no evidence of premature atherosclerosis studied by means of electron beam computed tomography and 13N-ammonia positron emission tomography: The entire coronary vasculature is free of calcifications. Her myocardial blood flow at rest (87 mL/100 g/min) and during stress (308 mL/100 g/min) and the coronary flow reserve (3.5) are normal after H.E.L.P. treatment. This case demonstrates the efficacy and safety of the combined treatment of H.E.L.P. apheresis and statins even in serious cases of FHH.

Original languageEnglish (US)
Pages (from-to)125-128
Number of pages4
JournalJournal of Pediatrics
Volume141
Issue number1
DOIs
StatePublished - 2002
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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