Faculty Collaboration Database

Renal transplantation in a child with primary oxalosis. Child Nephrol Urol 1990;10(2):103-6

Date

01/01/1990

Pubmed ID

2253246

Scopus ID

2-s2.0-0025080818 (requires institutional sign-in at Scopus site)   3 Citations

Abstract

Primary hyperoxaluria (oxalosis) is an autosomal recessive disorder due to an inherited deficiency of the peroxisomal alanine:glyoxylate aminotransferase characterized by increased production and urinary excretion of oxalate and glycolate resulting in renal failure due to oxalate deposition. Because of the risk of continuing oxalate deposition in the transplanted kidney, oxalosis had been considered a contraindication for transplantation. A 5-year-old boy with oxalosis, maintained on peritoneal dialysis, received a haploidentical qiving-related transplant. The preoperative management included donor-specific transfusions and daily hemodialysis to remove a maximum amount of oxalate. The immunosuppression consisted of azathioprine and prednisone. Aggressive fluid management including noncalciuric diuretics (hydrochlorothiazide) kept urine output high. Pyridoxine, magnesium, neutral phosphate and sodium benzoate were used to prevent deposition of oxalate in the transplanted kidney. Two acute rejection episodes responded to steroid boluses. A kidney biopsy during the second rejection episode confirmed the diagnosis but also revealed oxalate deposits in the transplanted kidney. More than 4 years after transplantation, the patient has catch-up growth and his serum creatinine is 1.4 mg/dl. In conclusion, oxalosis is not an absolute contraindication to renal transplantation. Transplantation can be performed successfully utilizing living-related donor kidneys and aggressive medical management. The risks of deterioration of function and oxalate deposition in the transplant kidney are offset by improvement in quality of life.

Author List

Casale AJ, Leichter HE, Sheth KJ, Segura AD, Lawson RK

Author

Annette D. Segura MD Adjunct Assistant Professor in the Pathology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Azathioprine
Child, Preschool
Graft Rejection
Humans
Hyperoxaluria, Primary
Kidney Transplantation
Male
Prednisone
Preoperative Care