Abstract
It is controversial if patients with advanced PH should be treated by isolated liver (L) TX or by combined kidney (K) TX + LTX. We observed 2 PH children with deficiency of hepatic alanine: glyoxylate transferase who were managed differently. No. 1 presented at 6 weeks with a serum creatinine (SCR) up to 3.8 mg/dl. Despite transient improvement by vitamin 86 the disease progressed. At age 1.8 yrs isolated LTX was performed. General condition improved but somatic development remained retarded requiring continuous tube feeding. Renal function stabilized: 1.8yrs post-TX SCR 2.5 mg/dl, creatinine clearance 15 ml/min/1.73 m2 vs 10 before TX. Serum oxalate (OX) decreased slowly but remained slightly elevated. Urinary OX and glycolate excretion dropped, interrupted by transient peaks. Patient No. 2 needed dialysis treatment at 4 yrs and underwent combined LTX + KTX 1 year later. GFR and OX in serum and urine rapidly normalized; 9 months post TX the condition is excellent.
Our experience demonstrates that isolated LTX is able to maintain K function in severe PH, even if performed in advanced renal failure, but enzyme replacement seems to be insufficient to compensate fully, in contrast to combined LTX + KTX.
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Schärer, K., Ruder, H., Otto, G. et al. 176 LIVER TRANSPLANTATION (TX) STABILIZES KIDNEY FUNCTION IN ADVANCED RENAL FAILURE OF PRIMARY HYPEROXALURIA (PH). Pediatr Res 28, 306 (1990). https://doi.org/10.1203/00006450-199009000-00200
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DOI: https://doi.org/10.1203/00006450-199009000-00200