Two-step transplantation for primary hyperoxaluria: A winning strategy to prevent progression of systemic oxalosis in early onset renal insufficiency cases.

[primary hyperoxaluria type 1]

Several transplant strategies for PH 1 have been proposed , and LT is performed to correct the metabolic defects . The patients with PH 1 often suffer from ESRD and require simultaneous LKT , which leads to a long wait due to the shortage of suitable organ donors . Five patients with PH 1 underwent LDLT at our institute . Three of the five patients were under dialysis before LDLT , while the other two patients were categorized as CKD stage 3 . An isolated LDLT was successfully performed in all but our first case , who had complicated postoperative courses and consequently died due to sepsis after retransplantation . The renal function of the patients with CKD stage 3 was preserved after LDLT . On the other hand , our second case with ESRD underwent successful LDKT six months after LDLT , and our infant case is waiting for the subsequent KT without any post-LDLT complications after the early establishment of PD . In conclusion , a two -step transplant strategy may be needed as a life-saving option for patients with PH 1 and may be possible even in small infants with systemic oxalosis . While waiting for a subsequent KT , an early resumption of PD should be considered from the perspective of the long -term requirement of RRT .