Pediatrics 1992 Feb;89(2):251-5

Rapid improvement in the renal tubular dysfunction associated with tyrosinemia following hepatic replacement

Shoemaker LR, Strife CF, Balistreri WF, Ryckman FC
Division of Nephrology, Children's Hospital Medical Center, Cincinnati, OH
45229-2899

Abstract
The postoperative management of patients with hereditary tyrosinemia type I
(McKusick 27670) following liver transplantation is often complicated by the
renal tubular dysfunction associated with this disease. To characterize better
the temporal course of the improvement in renal excretory activity following
hepatic replacement, renal tubular function and metabolite excretion were
studied in a 4-year-old girl with hereditary tyrosinemia during the immediate
post-transplantation course. Tubular reabsorption of bicarbonate and phosphate
were normal 5 days following transplantation, in contrast to glucosuria,
hyperaminoaciduria, and tyrosyluria, which persisted for approximately 3 weeks.
After hepatic replacement, serum amino acid concentrations returned to normal
and succinylacetone was no longer detected in the urine. This is the third
tyrosinemia patient reported to achieve complete resolution of urinary
abnormalities following transplantation, and the only patient in whom renal
tubular function was formally assessed within the first postoperative week