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Successful Treatment Of Homozygous Familial Hypercholesterolemia Using Cascade Filtration Plasmapheresis
OBJECTIVE: The aim of our study is to discuss the efficacy of low-density lipoprotein-cholesterol (LDL-C) apheresis procedure using the cascade filtration system for pediatric patients with homozygous familial hypercholesterolemia (FH), and to clarify the adverse effects and difficulties.METHODS: LDL apheresis using the cascade filtration system was performed in 3 pediatric patients with homozygous FH. In total, 120 apheresis sessions were performed for all patients. RESULTS: Cascade filtration therapy significantly reduced the mean LDL-C values from 418 ± 62 mg/dl to 145 ± 43 mg/dl (p<0.05). We determined an acute mean reduction in the plasma levels of total cholesterol (57.9%), LDL cholesterol (70.8%), and high-density lipoprotein (HDL) cholesterol (40.7%). Treatments were well tolerated. The most frequent clinical adverse effects were hypotension in 3 sessions (2.5%), chills/feeling cold (1.7%) in 2 sessions, and nausea and vomiting in 3 sessions (2.5%). CONCLUSION: Our experience with three patients using the cascade filtration system were, good clinical outcomes, laboratory findings, safety of usage, minor adverse effects and technical problems.

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Authors: Fatih Kardas, Aysun Cetin, Musa Solmaz, Ruksan Buyukoglan, Leylagul Kaynar, Mustafa Kendirci, Bulent Eser, Ali Unal

Keywords: Ailevi hiperkolesterolemi, ‘cascade filtration’ plazmaferez, düşük dansiteli lipoprotein-kolesterol (LDL-C)

  • Year: 2012
  • Number: 4
  • Referance No: 93546

  • Figure 1A: Pre-apheresis xanthomas on the right elbow of the first patient.
  • Figure 1B: Post-apheresis xanthomas on the right elbow of the first patient.
  • Figure 2: Schematic appearance of cascade filtration system,consisting of two separators.
  • Figure 3: Pre-and post-treatment LDL-C levels

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