ARPKD with
renal insufficiency during the first months of life is a clinical challenge. We report on two children with
ARPKD with massively enlarged kidneys requiring
renal replacement therapy in early infancy. Patient 1 developed pulmonary insufficiency due to massively enlarged kidneys. At the age of six months the girl was listed for KT as "high urgency" on the Eurotransplant waiting list. A kidney from a deceased donor was pre-emptively transplanted and simultaneous
nephrectomy performed. No postoperative complications were observed, and the patient was discharged from in-patient care 42 days after
transplantation. Unexpectedly, she died at the age of one yr due to cerebral vascular
spasms of unknown origin. Patient 2 was transferred at the age of three months to our clinic with life-threatening pulmonary insufficiency. Pre-emptive KT was not possible; therefore, bilateral
nephrectomy was performed and PD begun. The boy is still doing well on PD one yr later. Pre-emptive KT and bilateral
nephrectomy followed by PD are two options for infants with
ARPKD and excessive kidney enlargement. PD could be complicated and in some cases become impossible by peritoneal damage during
nephrectomy. On the other hand, KT covers a high risk of
infections caused by immunosuppression. The decision, which method to choose, should be driven by the individual situation of the patient and the expertise of the center.