title: Liver and kidney transplant in primary hyperoxaluria: a single center experience.
abstract: OBJECTIVE
  Primary hyperoxaluria, especially type 1, is a severe disease with multisystem morbidity and high mortality. We present 3 primary hyperoxaluria type 1 patients who underwent liver transplant, including living-donor liver transplant or combined liver and kidney transplant in our institution.
  
  
  METHODS
  Patients who underwent liver transplant or combined liver/kidney transplant at our institution were evaluated, retrospectively. Between January 2002 and 2013, there were 3 patients who underwent transplant for primary hyperoxaluria. All 3 patients had disease onset in childhood, and the definitive diagnosis was established at age < 1, 6, and 8 years. Although early diagnosis was made, primary hyperoxaluria resulted in end-stage renal disease in 2 patients, and hemodialysis was introduced before liver transplant. All 3 patients underwent living-donor liver transplant. Case 1 was a 10-year-old girl who had an uneventful course after living-donor liver transplant, and she received a living-donor kidney transplant from the same donor 4 months after living-donor liver transplant. Case 2 was a 7-yearold boy who was the younger brother of the first patient; he did not have end-stage renal disease or any renal disorder after successful living-donor liver transplant. Case 3 was a 3-year-old boy who was diagnosed at age 2 months with renal disorders; although he was discharged from the hospital after living-donor liver transplant, he was readmitted because of unconsciousness that developed 1 day after discharge, and he died because of intracranial hemorrhage 2 months after liver transplant, unable to receive a kidney transplant.
  
  
  CONCLUSIONS
  Primary hyperoxaluria is a rare disorder that is difficult to diagnose until end-organ damage is severe. Outcomes may be improved with early and accurate diagnosis, aggressive supportive treatment, and correction of the enzyme defect by liver transplant before systemic oxalosis develops. However, kidney transplant or combined liver and kidney transplant is required in many primary hyperoxaluria type 1 patients because of the delayed diagnosis or long organ waiting time.
authors.affiliation: From the Department of General Surgery, Baskent University, School of Medicine, Ankara, Turkey.
authors.forename: Gökhan
authors.initials: G
authors.lastname: Moray
authors.forename: Tugan
authors.initials: T
authors.lastname: Tezcaner
authors.forename: Figen
authors.initials: F
authors.lastname: Özçay
authors.forename: Esra
authors.initials: E
authors.lastname: Baskın
authors.forename: Aydıncan
authors.initials: A
authors.lastname: Akdur
authors.forename: Mahir
authors.initials: M
authors.lastname: Kırnap
authors.forename: Sedat
authors.initials: S
authors.lastname: Yıldırım
authors.forename: Gülnaz
authors.initials: G
authors.lastname: Arslan
authors.forename: Mehmet
authors.initials: M
authors.lastname: Haberal
mesh_terms: D002648:Child; D002675:Child, Preschool; D058625:End Stage Liver Disease / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000150:complications* / Q000175:diagnosis; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects; D016031:Liver Transplantation / Q000009:adverse effects; D019520:Living Donors; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014421:Turkey