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Turkish Journal of Haematology 2012-Dec

Successful treatment of homozygous familial hypercholesterolemia using cascade filtration plasmapheresis.

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Fatih Kardaş
Aysun Cetin
Musa Solmaz
Rüksan Büyükoğlan
Leylagül Kaynar
Mustafa Kendirci
Bülent Eser
Ali Unal

关键词

抽象

OBJECTIVE

The aim of this study was to report the efficacy of low-density lipoprotein cholesterol (LDL-C) apheresisusing a cascade filtration system in pediatric patients with homozygous familial hypercholesterolemia (FH), and toclarify the associated adverse effects and difficulties.

METHODS

LDL-C apheresis using a cascade filtration system was performed in 3 pediatric patientswith homozygous FH; in total, 120 apheresis sessions were performed.

RESULTS

Cascade filtration therapy significantly reduced the mean LDL-C values from 418 ± 62 mg/dL to 145 ± 43 mg/dL (p= 0.011). We observed an acute mean reduction in the plasma level of total cholesterol (57.9%), LDL-C (70.8%),and high-density lipoprotein cholesterol (HDL-C) (40.7%). Treatments were well tolerated. The most frequent clinicaladverse effects were hypotension in 3 sessions (2.5%), chills (1.7%) in 2 sessions, and nausea/vomiting in 3 sessions(2.5%).

CONCLUSIONS

Our experience using the cascade filtration system with 3 patients included good clinical outcomes andlaboratory findings, safe usage, and minor adverse effects and technical problems.

BACKGROUND

None declared.

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