Combined liver-kidney transplantation for primary hyperoxaluria type 1 in young children
- PMID: 11158411
- DOI: 10.1093/ndt/16.2.348
Combined liver-kidney transplantation for primary hyperoxaluria type 1 in young children
Abstract
Background: Primary hyperoxaluria type 1 (PH1) is a rare condition in which deficiency of the liver enzyme alanine:glyoxylate aminotransferase leads to renal failure and systemic oxalosis. Combined liver-kidney transplantation (LKT) is recommended for end-stage renal failure (ESRF) in adults, but management of infants and young children is controversial. We retrospectively reviewed six children who underwent LKT for PH1.
Methods: The median age at diagnosis was 1.8 years (range 3 weeks to 7 years). Two children presented with severe infantile oxalosis at 3 and 9 weeks, five patients had ESRF with nephrocalcinosis and systemic oxalosis, (median duration of dialysis 1.3 years), and one had progressive chronic renal failure. Four children underwent combined LKT, one child staged liver then kidney, and one infant had an isolated liver transplant. The median age at transplantation was 8.9 years (range 1.7-15 years).
Results: Overall patient survival was four out of six. The two infants with PH1 and severe systemic oxalosis died (2 and 3 weeks post-transplant) due to cardiovascular oxalosis and sepsis. The other four children are well at median follow-up of 10 months (range 6 months to 7.4 years). No child developed hepatic rejection and all have normal liver function. Renal rejection occurred in three patients. Despite maximal medical management, oxalate deposits recurred in all renal grafts, contributing to graft loss in one (one of the infants who died), and significant dysfunction requiring haemodialysis post-transplant for 6 months.
Conclusions: LKT is effective therapy for primary oxalosis with ESRF but has a high morbidity and mortality rate in children who present in infancy with nephrocalcinosis and systemic oxalosis. We feel that earlier LKT, or pre-emptive liver transplantation, may be a better therapeutic strategy to improve the outlook for these patients.
Comment in
-
Combined liver-kidney transplantation for primary hyperoxaluria type 1.Nephrol Dial Transplant. 2001 Oct;16(10):2113-4. doi: 10.1093/ndt/16.10.2113. Nephrol Dial Transplant. 2001. PMID: 11572916 No abstract available.
Similar articles
-
Long-term results of combined liver-kidney transplantation for primary hyperoxaluria type 1: the French experience.Liver Transpl. 2014 Dec;20(12):1475-85. doi: 10.1002/lt.24009. Liver Transpl. 2014. PMID: 25267365
-
Combined liver-kidney and isolated liver transplantations for primary hyperoxaluria type 1: the European experience. The European Study Group on Transplantation in Hyperoxaluria Type 1.Nephrol Dial Transplant. 1991;6(7):502-11. doi: 10.1093/ndt/6.7.502. Nephrol Dial Transplant. 1991. PMID: 1922912
-
Long term results of liver-kidney transplantation in children with primary hyperoxaluria.Pediatr Nephrol. 2001 Dec;16(12):946-50. doi: 10.1007/s004670100001. Pediatr Nephrol. 2001. PMID: 11793078
-
Combined liver-kidney transplantation in primary hyperoxaluria type 1.Eur J Pediatr. 1999 Dec;158 Suppl 2:S75-80. doi: 10.1007/pl00014327. Eur J Pediatr. 1999. PMID: 10603104 Review.
-
Primary Hyperoxaluria Type 1.2002 Jun 19 [updated 2022 Feb 10]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2024. 2002 Jun 19 [updated 2022 Feb 10]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2024. PMID: 20301460 Free Books & Documents. Review.
Cited by
-
Therapeutic RNA interference: A novel approach to the treatment of primary hyperoxaluria.Br J Clin Pharmacol. 2022 Jun;88(6):2525-2538. doi: 10.1111/bcp.14925. Epub 2021 Jun 11. Br J Clin Pharmacol. 2022. PMID: 34022071 Free PMC article. Review.
-
Transplantation outcomes in patients with primary hyperoxaluria: a systematic review.Pediatr Nephrol. 2021 Aug;36(8):2217-2226. doi: 10.1007/s00467-021-05043-6. Epub 2021 Apr 8. Pediatr Nephrol. 2021. PMID: 33830344 Free PMC article.
-
Bilateral native nephrectomy to reduce oxalate stores in children at the time of combined liver-kidney transplantation for primary hyperoxaluria type 1.Pediatr Nephrol. 2018 May;33(5):881-887. doi: 10.1007/s00467-017-3855-5. Epub 2017 Dec 14. Pediatr Nephrol. 2018. PMID: 29243158
-
Anaesthesia and intensive care for simultaneous liver-kidney transplantation: A single-centre experience with 12 recipients.Indian J Anaesth. 2016 Jul;60(7):476-83. doi: 10.4103/0019-5049.186025. Indian J Anaesth. 2016. PMID: 27512163 Free PMC article.
-
Combined liver and kidney transplantation in children: analysis of renal graft outcome.Pediatr Nephrol. 2016 Sep;31(9):1539-43. doi: 10.1007/s00467-016-3396-3. Epub 2016 Apr 22. Pediatr Nephrol. 2016. PMID: 27105881
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Medical