title: Pre-emptive liver transplantation for primary hyperoxaluria (PH-I) arrests long-term renal function deterioration.
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.
authors.affiliation: Liver Unit, Birmingham Children’s Hospital NHS Foundation Trust, Birmingham B4 6NH, UK. tam-per@hotmail.com
authors.forename: M Thamara P R
authors.initials: MT
authors.lastname: Perera
authors.forename: Khalid
authors.initials: K
authors.lastname: Sharif
authors.forename: Carla
authors.initials: C
authors.lastname: Lloyd
authors.forename: Katharine
authors.initials: K
authors.lastname: Foster
authors.forename: Sally A
authors.initials: SA
authors.lastname: Hulton
authors.forename: Darius F
authors.initials: DF
authors.lastname: Mirza
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: McKiernan
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology / Q000517:prevention & control*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005919:Glomerular Filtration Rate; D006084:Graft Rejection / Q000517:prevention & control*; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000150:complications / Q000601:surgery*; D007223:Infant; D007677:Kidney Function Tests; D016031:Liver Transplantation; D008297:Male; D015996:Survival Rate; D016896:Treatment Outcome