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Combined Liver-Kidney Transplantation for Primary Hyperoxaluria Type 1

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Pediatric Solid Organ Transplantation

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Abstract

Primary hyperoxaluria type 1 is a rare genetic condition that affects predominantly the renal and urinary system. Although it can present in patients of any age, a large proportion of affected individuals are children. This condition may be challenging to diagnose, and both medical and surgical management are often required. Transplantation of the liver and kidney is the definitive treatment for this condition; however, long-term outcomes in pediatric patients are lacking. This chapter will provide a review of PH1, with a particular focus on liver and kidney transplantation.

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References

  1. Soliman NA, Nabhan MM, Abdelrahman SM, Abdelaziz H, Helmy R, Ghanim K, et al. Clinical spectrum of primary hyperoxaluria type 1: experience of a tertiary center. Nephrol Ther. 2017;13(3):176–82.

    PubMed  PubMed Central  Google Scholar 

  2. Danpure CJ. Molecular aetiology of primary hyperoxaluria type 1. Nephron Exp Nephrol. 2004;98(2):e39–44.

    PubMed  Google Scholar 

  3. Cochat P, Hulton SA, Acquaviva C, Danpure CJ, Daudon M, De Marchi M, et al. Primary hyperoxaluria type 1: indications for screening and guidance for diagnosis and treatment. Nephrol Dial Transplant. 2012;27(5):1729–36.

    CAS  PubMed  Google Scholar 

  4. Coulter-Mackie MB, Rumsby G. Genetic heterogeneity in primary hyperoxaluria type 1: impact on diagnosis. Mol Genet Metab. 2004;83(1–2):38–46.

    CAS  PubMed  Google Scholar 

  5. Leumann E, Hoppe B. The primary hyperoxalurias. J Am Soc Nephrol. 2001;12(9):1986–93.

    PubMed  Google Scholar 

  6. Kopp N, Leumann E. Changing pattern of primary hyperoxaluria in Switzerland. Nephrol Dial Transplant. 1995;10(12):2224–7.

    CAS  PubMed  Google Scholar 

  7. Cochat P, Groothoff J. Primary hyperoxaluria type 1: practical and ethical issues. Pediatr Nephrol. 2013;28(12):2273–81.

    PubMed  Google Scholar 

  8. Cochat P, Basmaison O. Current approaches to the management of primary hyperoxaluria. Arch Dis Child. 2000;82(6):470–3.

    CAS  PubMed  PubMed Central  Google Scholar 

  9. van Woerden CS, Groothoff JW, Wanders RJ, Davin JC, Wijburg FA. Primary hyperoxaluria type 1 in the Netherlands: prevalence and outcome. Nephrol Dial Transplant. 2003;18(2):273–9.

    PubMed  Google Scholar 

  10. Strauss SB, Waltuch T, Bivin W, Kaskel F, Levin TL. Primary hyperoxaluria: spectrum of clinical and imaging findings. Pediatr Radiol. 2017;47(1):96–103.

    PubMed  Google Scholar 

  11. Ellis SR, Hulton SA, McKiernan PJ, de Ville de Goyet J, Kelly DA. Combined liver–kidney transplantation for primary hyperoxaluria type 1 in young children. Nephrol Dial Transplant. 2001;16(2):348–54.

    CAS  PubMed  Google Scholar 

  12. Cochat P, Liutkus A, Fargue S, Basmaison O, Ranchin B, Rolland MO. Primary hyperoxaluria type 1: still challenging! Pediatr Nephrol. 2006;21(8):1075–81.

    PubMed  Google Scholar 

  13. Hopp K, Cogal AG, Bergstralh EJ, Seide BM, Olson JB, Meek AM. Phenotype-genotype correlations and estimated carrier frequencies of primary hyperoxaluria. J Am Soc Nephrol. 2015;26(10):2559–70.

    CAS  PubMed  PubMed Central  Google Scholar 

  14. Harambat J, Fargue S, Acquaviva C, Gagnadoux MF, Janssen F, Liutkus A, et al. Genotype-phenotype correlation in primary hyperoxaluria type 1: the p.Gly170Arg AGXT mutation is associated with a better outcome. Kidney Int. 2010;77(5):443–9.

    CAS  PubMed  Google Scholar 

  15. Cochat P, Deloraine A, Rotily M, Olive F, Liponski I, Deries N. Epidemiology of primary hyperoxaluria type 1. Société de Néphrologie and the Société de Néphrologie Pédiatrique. Nephrol Dial Transplant. 1995;10(Suppl 8):3–7.

    PubMed  Google Scholar 

  16. van der Hoeven SM, van Woerden CS, Groothoff JW. Primary hyperoxaluria type 1, a too often missed diagnosis and potentially treatable cause of end-stage renal disease in adults: results of the Dutch cohort. Nephrol Dial Transplant. 2012;27(10):3855–62.

    PubMed  Google Scholar 

  17. Kogiso T, Tokushige K, Hashimoto E, Miyakata C, Taniai M, Torii N, et al. Primary hyperoxaluria complicated with liver cirrhosis: a case report. Hepatol Res. 2015;45(12):1251–5.

    PubMed  Google Scholar 

  18. Tanriover B, Mejia A, Foster SV, Mubarak A. Primary hyperoxaluria involving the liver and hepatic artery: images of an aggressive disease. Kidney Int. 2010;77(7):651.

    PubMed  Google Scholar 

  19. El Hage S, Ghanem I, Baradhi A, Mourani C, Mallat S, Dagher F, et al. Skeletal features of primary hyperoxaluria type 1, revisited. J Child Orthop. 2008;2(3):205–10.

    PubMed  PubMed Central  Google Scholar 

  20. Mookadam F, Smith T, Jiamsripong P, Moustafa SE, Monico CG, Lieske JC, et al. Cardiac abnormalities in primary hyperoxaluria. Circ J. 2010;74(11):2403–9.

    PubMed  PubMed Central  Google Scholar 

  21. Jacob DE, Grohe B, Geßner M, Beck BB, Hoppe B. Kidney stones in primary hyperoxaluria: new lessons learnt. PLoS One. 2013;8(8):e70617.

    CAS  PubMed  PubMed Central  Google Scholar 

  22. Birtel J, Herrmann P, Garrelfs SF, Dulz S, Atiskova Y, Diederen RM, et al. The ocular phenotype in primary hyperoxaluria type 1. Am J Ophthalmol. 2019;206:184–91.

    PubMed  Google Scholar 

  23. Beck BB, Hoyer-Kuhn H, Göbel H, Habbig S, Hoppe B. Hyperoxaluria and systemic oxalosis: an update on current therapy and future directions. Expert Opin Investig Drugs. 2013;22(1):117–29.

    CAS  PubMed  Google Scholar 

  24. Fargue S, Harambat J, Gagnadoux MF, Tsimaratos M, Janssen F, Llanas B, et al. Effect of conservative treatment on the renal outcome of children with primary hyperoxaluria type 1. Kidney Int. 2009;76(7):767–73.

    PubMed  Google Scholar 

  25. Leumann E, Hoppe B, Neuhaus T. Management of primary hyperoxaluria: efficacy of oral citrate administration. Pediatr Nephrol. 1993;7(2):207–11.

    CAS  PubMed  Google Scholar 

  26. Khorsandi SE, Samyn M, Hassan A, Vilca-Melendez H, Waller S, Shroff R, et al. An institutional experience of pre-emptive liver transplantation for pediatric primary hyperoxaluria type 1. Pediatr Transplant. 2016;20(4):523–9.

    PubMed  Google Scholar 

  27. Perera MT, Sharif K, Lloyd C, Foster K, Hulton SA, Mirza DF, et al. Pre-emptive liver transplantation for primary hyperoxaluria (PH-I) arrests long-term renal function deterioration. Nephrol Dial Transplant. 2011;26(1):354–9.

    PubMed  Google Scholar 

  28. Coulthard MG, Lodge JP. Liver transplantation before advanced renal failure in primary hyperoxaluria type 1. Pediatr Nephrol. 1993;7(6):774.

    CAS  PubMed  Google Scholar 

  29. Cochat P, Schärer K. Should liver transplantation be performed before advanced renal insufficiency in primary hyperoxaluria type 1? Pediatr Nephrol. 1993;7(2):212–8.

    CAS  PubMed  Google Scholar 

  30. Harambat J, van Stralen KJ, Espinosa L, Groothoff JW, Hulton SA, Cerkauskiene R, et al. Characteristics and outcomes of children with primary oxalosis requiring renal replacement therapy. Clin J Am Soc Nephrol. 2012;7(3):458–65.

    PubMed  PubMed Central  Google Scholar 

  31. Jamieson NV, European PHI Transplantation Study Group. A 20-year experience of combined liver/kidney transplantation for primary hyperoxaluria (PH1): the European PH1 transplant registry experience 1984-2004. Am J Nephrol. 2005;25(3):282–9.

    PubMed  Google Scholar 

  32. Cibrik DM, Kaplan B, Arndorfer JA, Meier-Kriesche HU. Renal allograft survival in patients with oxalosis. Transplantation. 2002;74(5):707–10.

    CAS  PubMed  Google Scholar 

  33. Gagnadoux MF, Lacaille F, Niaudet P, Revillon Y, Jouvet P, Jan D, et al. Long term results of liver-kidney transplantation in children with primary hyperoxaluria. Pediatr Nephrol. 2001;16(12):946–50.

    CAS  PubMed  Google Scholar 

  34. Nair P, Al-Otaibi T, Nampoory N, Al-Qabandi W, Said T, Halim MA. Combined liver and kidney transplantation in primary hyperoxaluria: a report of three cases and review of the literature. Saudi J Kidney Dis Transpl. 2013;24(5):969–75.

    PubMed  Google Scholar 

  35. Kemper MJ. Concurrent or sequential liver and kidney transplantation in children with primary hyperoxaluria type 1? Pediatr Transplant. 2005;9(6):693–6.

    PubMed  Google Scholar 

  36. Rasmussen A, Davies HF, Jamieson NV, Evans DB, Calne RY. Combined transplantation of liver and kidney from the same donor protects the kidney from rejection and improves kidney graft survival. Transplantation. 1995;59(6):919–21.

    CAS  PubMed  Google Scholar 

  37. Astarcioglu I, Karademir S, Gülay H, Bora S, Astarcioglu H, Kavukcu S, et al. Primary hyperoxaluria: simultaneous combined liver and kidney transplantation from a living related donor. Liver Transpl. 2003;9(4):433–6.

    PubMed  Google Scholar 

  38. de la Cerda F, Jimenez WA, Gjertson DW, Venick R, Tsai E, Ettenger R. Renal graft outcome after combined liver and kidney transplantation in children: UCLA and UNOS experience. Pediatr Transplant. 2010;14(4):459–64.

    PubMed  Google Scholar 

  39. Siegal D, Su WS, DaBreo D, Puglia M, Gregor L, Gangji AS. Liver-kidney transplantation in primary hyperoxaluria Type-1: case report and literature review. Int J Organ Transplant Med. 2011;2(3):126–32.

    CAS  PubMed  PubMed Central  Google Scholar 

  40. Narasimhan G, Govil S, Rajalingam R, Venkataraman C, Shanmugam NP, Rela M. Preserving double equipoise in living donor liver-kidney transplantation for primary hyperoxaluria type 1. Liver Transpl. 2015;21(10):1324–6.

    PubMed  Google Scholar 

  41. Sasaki K, Sakamoto S, Uchida H, Shigeta T, Matsunami M, Kanazawa H, et al. Two-step transplantation for primary hyperoxaluria: a winning strategy to prevent progression of systemic oxalosis in early onset renal insufficiency cases. Pediatr Transplant. 2015;19(1):E1–6.

    PubMed  Google Scholar 

  42. Mor E, Nesher E, Ben-Ari Z, Weissman I, Shaharabani E, Eizner S, et al. Sequential liver and kidney transplantation from a single living donor in two young adults with primary hyperoxaluria type 1. Liver Transpl. 2013;19(6):646–8.

    PubMed  Google Scholar 

  43. Unek T, Egeli T, Özbilgin M, Çelik A, Atilla K, Ağalar C, et al. Long-term results of living donors in simultaneous kidney and liver transplantations. Transplant Proc. 2017;49(3):403–6.

    CAS  PubMed  Google Scholar 

  44. Angelico R, Guzzo I, Pellicciaro M, Saffioti MC, Grimaldi C, Mourani C. Same donor laparoscopic liver and kidney procurement for sequential living donor liver-kidney transplantation in primary hyperoxaluria type I. J Laparoendosc Adv Surg Tech A. 2019;29(12):1616–22.

    PubMed  Google Scholar 

  45. Malla I, Lysy PA, Godefroid N, Smets F, Malaise J, Reding R, et al. Two-step transplantation for primary hyperoxaluria: cadaveric liver followed by living donor related kidney transplantation. Pediatr Transplant. 2009;13(6):782–4.

    PubMed  Google Scholar 

  46. Popescu I, Dima SO. Domino liver transplantation: how far can we push the paradigm? Liver Transpl. 2012;18(1):22–8.

    PubMed  Google Scholar 

  47. Franchello A, Paraluppi G, Romagnoli R, Petrarulo M, Vitale C, Pacitti A, et al. Severe course of primary hyperoxaluria and renal failure after domino hepatic transplantation. Am J Transplant. 2005;5(9):2324–7.

    PubMed  Google Scholar 

  48. Saner FH, Treckmann J, Pratschke J, Arbogast H, Rahmel A, Vester U, et al. Early renal failure after domino liver transplantation using organs from donors with primary hyperoxaluria type 1. Transplantation. 2010;90(7):782–5.

    PubMed  Google Scholar 

  49. Cochat P, Fargue S, Harambat J. Primary hyperoxaluria type 1: strategy for organ transplantation. Curr Opin Organ Transplant. 2010;15(5):590–3.

    PubMed  Google Scholar 

  50. Cha D, Concepcion K, Gallo A, Concepcion W. Combined liver kidney transplantation in pediatrics: indications, special considerations, and outcomes. Clin Surg. 2017;2:1352.

    Google Scholar 

  51. Grenda R, Kaliciński P. Combined and sequential liver-kidney transplantation in children. Pediatr Nephrol. 2018;33(12):2227–37.

    PubMed  PubMed Central  Google Scholar 

  52. Millan MT, Berquist WE, So SK, Sarwal MM, Wayman KI, Cox KL, et al. One hundred percent patient and kidney allograft survival with simultaneous liver and kidney transplantation in infants with primary hyperoxaluria: a single-center experience. Transplantation. 2003;76(10):1458–63.

    PubMed  Google Scholar 

  53. Nissel R, Latta K, Gagnadoux MF, Kelly D, Hulton S, Kemper MJ, et al. Body growth after combined liver-kidney transplantation in children with primary hyperoxaluria type 1. Transplantation. 2006;82(1):48–54.

    PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Cleveland Clinic, Cleveland, OH, USA

    Charles B. Chen & Kadakkal Radhakrishnan

  2. Department of General Surgery, Digestive Disease & Surgery Institute, Cleveland Clinic, Cleveland, OH, USA

    Koji Hashimoto

Authors
  1. Charles B. Chen
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  2. Kadakkal Radhakrishnan
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  3. Koji Hashimoto
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Corresponding author

Correspondence to Koji Hashimoto .

Editor information

Editors and Affiliations

  1. Recanati/Miller Transplantation Institute, Mount Sinai Hospital, NY, USA

    Ron Shapiro

  2. Division of Transplant Surgery, University of California, San Francisco, CA, USA

    Minnie M. Sarwal

  3. Department of Nephrology, Akron General Medical Center at Cleveland Clinic Foundation and Akron Children’s Hospital, OH, USA

    Rupesh Raina

  4. Kidney and Urology Institute, Medanta, The Medicity, Gurugram, Haryana, India

    Sidharth Kumar Sethi

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Chen, C.B., Radhakrishnan, K., Hashimoto, K. (2023). Combined Liver-Kidney Transplantation for Primary Hyperoxaluria Type 1. In: Shapiro, R., Sarwal, M.M., Raina, R., Sethi, S.K. (eds) Pediatric Solid Organ Transplantation. Springer, Singapore. https://doi.org/10.1007/978-981-19-6909-6_32

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  • DOI: https://doi.org/10.1007/978-981-19-6909-6_32

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  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-19-6908-9

  • Online ISBN: 978-981-19-6909-6

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