Report of 3 Patients With Urea Cycle Defects Treated With Related Living-Donor Liver Transplant

Ozcay F., Baris Z., Moray G., Haberal N., Torgay A., Haberal M.

EXPERIMENTAL AND CLINICAL TRANSPLANTATION, cilt.13, ss.126-130, 2015 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 13
  • Basım Tarihi: 2015
  • Doi Numarası: 10.6002/ect.tdtd2015.p69
  • Dergi Adı: EXPERIMENTAL AND CLINICAL TRANSPLANTATION
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.126-130
  • Eskişehir Osmangazi Üniversitesi Adresli: Hayır

Özet

Urea cycle defects are a group of metabolic disorders caused by enzymatic disruption of the urea cycle pathway, transforming nitrogen to urea for excretion from the body. Severe cases present in early infancy with life-threatening metabolic decompensation, and these episodes of hyperammonemia can be fatal or result in permanent neurologic damage. Despite the progress in pharmacologic treatment, long-term survival is poor especially for severe cases. Liver trans plant is an alternative treatment option, providing sufficient enzymatic activity and decreasing the risk of metabolic decompensation.