Browsing by Author "Hölttä, Tuula"

Background The only effective treatment for severe forms of congenital nephrotic syndrome is nephrectomy and kidney transplantation (KT). Optimal timing of nephrectomy is unclear. Methods The influence of early (Group 1, n = 13) versus delayed (Group 2, n = 10) nephrectomy on patient outcome was evaluated. The key laboratory results, growth, number of thromboembolic events and infections, KT-related complications, and eating ability after KT were compared between the two groups. Results Patients in group 1 were significantly younger at nephrectomy compared to group 2 (278 vs. 408 days, p = 0.007) and the dialysis time was significantly (p < 0.001) longer, 261 vs. 36 days, respectively. The occurrence of thromboembolic events or septicemia before KT did not differ between the groups. In group 1, eating problems and need for nasogastric tube (NGT) feeding were more common than in group 2. NGT dependency at discharge, 3 months, and 6 months post-KT were 100%, 92%, and 69% in group 1 and 90% (p = 0.244), 50% (p = 0.022), and 20% (p = 0.019) in group 2, respectively. Neurocognitive development was considered normal before KT and 12 months after the KT in 30.8% and 84.6% of the patients in group 1 and in 80% (p = 0.019) and 90% (p = 0.704) in group 2, respectively. Conclusions Infants with delayed nephrectomy seem to have comparative outcome but less infantile eating disorders and better neurocognitive development during the first post-KT months compared to infants with early nephrectomy.