Combined liver-kidney transplantation in a child with primary hyperoxaluria
- 18 Downloads
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 wordsLiver-kidney transplantation Primary hyperoxaluria
Unable to display preview. Download preview PDF.
- 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
- 8.Hicks NR, Cranston DW, Charlton CAC (1983) Fifteen year follow-up of hyperoxaluria type II. N Engl J Med 309:796Google Scholar
- 10.Morgan SH, Watts RWE (1989) Perspectives in the assessment and management of patients with primary hyperoxaluria. Adv Nephrol 18:95–106Google Scholar
- 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