Pre-emptive liver transplantation for primary hyperoxaluria (PH-I) arrests long-term renal function deterioration

Research output: Contribution to journalArticlepeer-review

Authors

  • M Thamara P R Perera
  • Khalid Sharif
  • Carla Lloyd
  • Katharine Foster
  • Sally A Hulton
  • And 2 others
  • Darius F Mirza
  • Patrick J McKiernan

Abstract

BACKGROUND: Primary hyperoxaluria-I (PH-I) is a serious metabolic disease resulting in end-stage renal disease. Pre-emptive liver transplantation (PLT) for PH-I is an option for children with early diagnosis. There is still little information on its effect on long-term renal function in this situation.

METHODS: Long-term assessment of renal function was conducted using Schwartz's formula (estimated glomerular filtration rate-eGFR) in four children (Group A) undergoing PLT between 2002 and 2008, and a comparison was done with eight gender- and sex-matched controls (Group B) having liver transplantation for other indications.

RESULTS: All patients received a liver graft from a deceased donor. Median follow-up for the two groups was 64 and 94 months, respectively. One child in Group A underwent re-transplantation due to hepatic artery thrombosis, while acute rejection was seen in one. A significant difference was seen in eGFR at transplant (81 vs 148 mL/min/1.73 m(2)) with greater functional impairment seen in the study population. In Group A, renal function reduced by 21 and 11% compared with 37 and 35% in Group B at 12 and 24 months, respectively. At 2 years post-transplantation, there was no significant difference in eGFR between the two groups (72 vs 100 mL/min/1.73 m(2), respectively; P = 0.06).

CONCLUSIONS: Renal function remains relatively stable following pre-emptive LTx for PH-I. With early diagnosis of PH-I, isolated liver transplantation may prevent progression to end-stage renal disease and the need for renal transplantation.

Details

Original languageEnglish
Pages (from-to)354-9
Number of pages6
JournalNephrology, Dialysis, Transplantation
Volume26
Issue number1
Publication statusPublished - Jan 2011

Keywords

  • Acute Kidney Injury, Child, Child, Preschool, Female, Glomerular Filtration Rate, Graft Rejection, Humans, Hyperoxaluria, Primary, Infant, Kidney Function Tests, Liver Transplantation, Male, Survival Rate, Treatment Outcome