Kidney transplantation in children: Case

This girl has end-stage kidney disease from bilateral renal hypodysplasia, a congenital anomaly of the kidney and urinary tract and the leading cause of paediatric end-stage kidney disease leading to transplant. Her estimated glomerular filtration rate of 13 mL per minute per 1.73 square metres places her at the threshold where dialysis is imminent, so a preemptive living-donor transplant from her father is the optimal plan. This avoids dialysis entirely and offers the best long-term graft survival. ^[2]

Clinical findings

The clinical picture is that of advanced chronic kidney disease. Her growth failure, anaemia and hypertension are the systemic consequences of uraemia and reduced erythropoietin production. Renal hypodysplasia is a structural cause of kidney failure, so unlike glomerular disease there is no heavy proteinuria or nephritic syndrome to manage, and the disease will not recur in the graft because it is congenital and structural. This is a favourable feature, because it removes recurrent disease from the post-transplant differential. ^[1]

The choice of a preemptive living-related transplant is justified by two independent advantages. A living-donor kidney, especially from a parent, gives the best graft survival because the kidney is healthy and the cold ischaemia time is short. A preemptive transplant, performed before dialysis begins, avoids the growth failure, cardiovascular damage and infectious risk of dialysis and is itself associated with superior graft survival. The practical trigger is the eGFR: once it approaches 10 to 15 mL per minute per 1.73 square metres, dialysis is imminent, so the donor workup must already be complete. ^[2]