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

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

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26 Citations (Scopus)


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.

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


  • 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


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