Pediatric Nephrology

, Volume 5, Issue 3, pp 332–334 | Cite as

Combined liver-kidney transplantation in a child with primary hyperoxaluria

  • Martin S. Polinsky
  • Steven Dunn
  • Bruce A. Kaiser
  • Seth L. Schulman
  • Barbara J. Wolfson
  • I. Bruce Elfenbein
  • H. J. Baluarte
Brief Report

Abstract

A 3.5-year-old boy presented with end-stage renal disease and bilateral nephrocalcinosis. Renal biopsy demonstrated marked parenchymal calcium oxalate deposition and a diagnosis of primary hyperoxaluria (PH) was made. Following 2 years of hemodialysis he received two renal allografts which were lost at 7 and 11 months, respectively, due to biopsy-proven recurrent oxalosis. Combined liver-kidney transplantation was then performed, after which renal and hepatic function initially stabilized. The patient died on the 28th postoperative day, of in fectious complications and progressive respiratory insufficiency. However, comparisons between the patterns of urinary oxalate excretion noted after the isolated renal and liver-kidney transplants indicated that, following the latter, successful biochemical correction of the enzyme defect responsible for type 1 PH had occurred.

Key words

Liver-kidney transplantation Primary hyperoxaluria 

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References

  1. 1.
    Hillman RE (1989) Primary hyperoxalurias. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic basis of inherited disease, 6th edn. McGraw Hill, New York, pp 933–944Google Scholar
  2. 2.
    David DS, Cheigh JS, Stenzel KH, Rubin AL (1983) Successful renal transplantation in a patient with primary hyperoxaluria. Transplant Proc 15:2168–2171PubMedGoogle Scholar
  3. 3.
    Scheinman JI, Najarian JS, Mauer SM (1984) Successful strategies for renal transplantation in primary hyperoxaluria. Kidney Int 25: 804–811PubMedGoogle Scholar
  4. 4.
    Katz A, Kim Y, Scheinman J, Najarian JS, Mauer SM (1989) Long term outcome of kidney transplantation in children with oxalosis. Transplant Proc 21:2033–2035PubMedGoogle Scholar
  5. 5.
    Morgan JM, Hartley MW, Miller AC, Diethelm AG (1974) Successful transplantation in hyperoxaluria. Arch Surg 109:430–433PubMedGoogle Scholar
  6. 6.
    Danpure CJ, Jennings PR (1986) Peroxisomal alanine: glyoxalate aminotransferase deficiency in primary hyperoxaluria type I. FEBS Lett 201:20–24CrossRefPubMedGoogle Scholar
  7. 7.
    Gottlieb RP, Ritter JA (1977) Flecked retina — an association with primary hyperoxaluria. J Pediatr 90:939–942PubMedGoogle Scholar
  8. 8.
    Hicks NR, Cranston DW, Charlton CAC (1983) Fifteen year follow-up of hyperoxaluria type II. N Engl J Med 309:796Google Scholar
  9. 9.
    Day DL, Scheinman JI, Mahan J (1986) Radiological aspects of primary hyperoxaluria. AJR 146:395–401PubMedGoogle Scholar
  10. 10.
    Morgan SH, Watts RWE (1989) Perspectives in the assessment and management of patients with primary hyperoxaluria. Adv Nephrol 18:95–106Google Scholar
  11. 11.
    McDonald JC, Landreneau MD, Rohr MS, DeVault GA Jr (1989) Reversal by liver transplantation of the complications of primary hyperoxaluria as well as the metabolic defect. N Engl J Med 321: 1100–1103PubMedGoogle Scholar
  12. 12.
    Cochat P, Faure JL, Divry P, Danpure CJ, Descos B, Wright C, Takvorian P, Floret D (1989) Liver transplantation in primary hyperoxaluria. Lancet I:1142–1143Google Scholar

Copyright information

© IPNA 1990

Authors and Affiliations

  • Martin S. Polinsky
    • 1
  • Steven Dunn
    • 2
  • Bruce A. Kaiser
    • 1
  • Seth L. Schulman
    • 1
  • Barbara J. Wolfson
    • 3
  • I. Bruce Elfenbein
    • 4
  • H. J. Baluarte
    • 1
  1. 1.Division of Pediatric Nephrology, St. Christopher's Hospital for ChildrenTemple University School of MedicinePhiladelphiaUSA
  2. 2.Department of Surgery, St. Christopher's Hospital for ChildrenTemple University School of MedicinePhiladelphiaUSA
  3. 3.Department of Radiology, St. Christopher's Hospital for ChildrenTemple University School of MedicinePhiladelphiaUSA
  4. 4.Department of Pathology, St. Christopher's Hospital for ChildrenTemple University School of MedicinePhiladelphiaUSA

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