Liver transplantation for homozygote familial hypercholesterolemia: the only curative treatment

Alim A., Tokat Y., Erdogan Y., Gokkaya Z., DAYANGAÇ M., Yuzer Y., ...Daha Fazla

Pediatric Transplantation, cilt.20, sa.8, ss.1060-1064, 2016 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 20 Sayı: 8
  • Basım Tarihi: 2016
  • Doi Numarası: 10.1111/petr.12763
  • Dergi Adı: Pediatric Transplantation
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.1060-1064
  • Anahtar Kelimeler: familial hypercholesterolemia, LDL receptor mutation, living donor liver transplantation, pediatric transplantation, premature cardiovascular diseases
  • İstanbul Medipol Üniversitesi Adresli: Evet

Özet

FH is an autosomal dominant genetic disorder characterized by increased TC and LDL level, which leads to xanthomas, atherosclerosis, and cardiac complications even in childhood. The treatment options are diet, medical treatment, lipid apheresis, and LT. The aim of our study was to analyze our data of patients with FH. Between 2004 and 2015, there were 51 patients who underwent pediatric LT at our center. All patients with FH were identified, and the data were retrospectively analyzed. There were eight patients with homozygous FH in the median age of 10 years (IQR 6–12) who underwent LT. The median pre-operative TC and LDL levels were 611 mg/dL (IQR: 460–844) and 574 mg/dL (IQR: 398–728) and decreased to normal levels 1 week after LT (TC: 193 mg/dL and LDL: 141 mg/dL). Two patients died two and 18 months after LT due to sudden cardiac arrest. Both patients were diagnosed with cardiovascular disease pre-operatively. The LT is the only curative treatment for this disease. To achieve an excellent outcome, it should be performed before the development of cardiovascular disease, because the regression of severe cardiovascular disease after transplantation is limited.