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.