Primary Hyperoxaluria

Combined liver-kidney transplantation shows promise in treating oxalate bone disease in pediatric patients

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Combined liver-kidney transplantation (CLKT) and kidney after liver transplantation (KALT) show promise in treating oxalate bone disease in pediatric patients with primary hyperoxaluria type 1 (PH1), according to a study.

The study highlights significant improvements in bone health, including the cessation of pathological fractures and moderate catch-up growth, following successful transplantation.

The retrospective cohort study focused on 10 pediatric patients with PH1 who underwent transplantation. The cohort was followed up for a median duration of 8.1 years.

Among the patients, 7 were diagnosed with infantile oxalosis. Prior to transplantation, 9 patients underwent intensified dialysis regimes, underscoring the advanced disease state. Despite challenges, successful outcomes were achieved in most cases, with stable kidney and liver function observed post-transplantation.

Before the procedure, 7 patients experienced severe skeletal pain, while 3 suffered from pathological fractures. Notably, patients who underwent CLKT or KALT showed cessation of pathological fractures post-transplantation. Plasma oxalate levels exhibited a significant decline within 6 months following transplantation, indicating the efficacy of the procedure in reducing oxalate burden.

Determinants of calcium-phosphorus metabolism did not significantly differ from other pediatric patients on hemodialysis. Importantly, 7 out of 10 children exhibited restricted growth pre-transplantation, with a moderate catch-up growth observed at the last follow-up, highlighting the potential for improved bone health and growth post-transplantation.

Büscher R, Pape L, Büscher AK. Bone health in children with primary hyperoxaluria type 1 following liver and kidney transplantation. Front Pediatr. 2024;12:1353880. doi: 10.3389/fped.2024.1353880. PMID: 38455394; PMCID: PMC10917879.