Successful treatment of homozygous familial hypercholesterolemia using cascade filtration plasmapheresis Homozigot ailevi hiperkolesterolemi'nin 'cascade filtration' plazmaferezi ile başarılı tedavisi

KARDAŞ F., ÇETİN A., Solmaz M., Büyükoǧlan R., KAYNAR L., Kendirci M., ...Daha Fazla

Turkish Journal of Hematology, cilt.29, sa.4, ss.334-341, 2012 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 29 Sayı: 4
  • Basım Tarihi: 2012
  • Doi Numarası: 10.5152/tjh.2011.20
  • Dergi Adı: Turkish Journal of Hematology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.334-341
  • Anahtar Kelimeler: Cascade filtration plasmapheresis, Familial hypercholesterolemia, Low-density lipoprotein cholesterol (LDL-C)
  • İstanbul Medipol Üniversitesi Adresli: Hayır

Özet

Objective: The aim of this study was to report the efficacy of low-density lipoprotein cholesterol (LDL-C) apheresis using a cascade filtration system in pediatric patients with homozygous familial hypercholesterolemia (FH), and to clarify the associated adverse effects and difficulties. Material and Methods: LDL-C apheresis using a cascade filtration system was performed in 3 pediatric patients with 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 clinical adverse effects were hypotension in 3 sessions (2.5%), chills (1.7%) in 2 sessions, and nausea/vomiting in 3 sessions (2.5%). Conclusion: Our experience using the cascade filtration system with 3 patients included good clinical outcomes and laboratory findings, safe usage, and minor adverse effects and technical problems.