Late nephrectomy in infants with congenital nephrotic syndrome of the Finnish type.

ABSTRACT

AIM: Bilateral nephrectomy is commonly performed in patients with congenital nephrotic syndrome of the Finnish type. The optimal timing of nephrectomy is unclear. METHODS: Growth, thromboembolic events, infections, transplant-related complications and ability to eat were compared between infants with early (Group 1, n = 13) and late (Group 2, n = 10) nephrectomy. 'Early' was defined as nephrectomy at 7-kg body weight followed by peritoneal dialysis and 'late' as nephrectomy at ≥10 kg followed by 3-4 weeks of haemodialysis and kidney transplantation. Patients were followed until the end of the first post-transplant year. RESULTS: Dialysis time was significantly longer in group 1 than in group 2. Late nephrectomy did not increase the risk for thromboembolic events or septicaemia but decreased tube feeding dependency (group 1 69% vs. group 2 20%, p = 0.019). Motor development at transplantation was considered normal in 80% of the infants with late nephrectomy compared to 31% in the early nephrectomy group (p = 0.019); however, the difference between the groups disappeared by the end of the follow-up. CONCLUSION: Infants with late nephrectomy have comparative outcome but less feeding tube dependency and better motor development during the first post-transplant months compared to infants with early nephrectomy.