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Background and Aims Bladder dysfunction is an important cause of chronic kidney disease (CKD) due to neurogenic causes such as a spinal lesion (SL), Hinman's syndrome, or non-neurogenic neurogenic bladder (NNNB), and valve bladder syndrome (VBS). In this study, we evaluated the outcomes of neurogenic bladder caused by different etiologies. Method Single-center retrospective study of children with neurogenic bladder (NB) over 13 years (2005-2017). Results Totally, 199 children (134 boys, 67.3%) were included and divided into three groups: Group 1 (75, NNNB), Group 2 (64, SL), and Group 3 (60, VBS). Urodynamic examination revealed impaired bladder capacity in 49 children (57.6%), atonic bladder in 1 child (1.1%), and normal bladder compliance in 35 children (41.1%). Additionally, 113 children (56.8%) experienced clean intermittent catheterization (CIC). A significant improvement in the mean estimated glomerular filtration rate and CKD stages was observed among the three studied groups, but the NNNB group showed the least improvement. Children diagnosed and treated during their first year of life had better outcomes than those who presented at a later age. CIC without the need for surgical intervention was associated with improved renal function. Pyelonephritis and hydronephrosis had significant negative impacts on kidney function Conclusion Early diagnosis and management of NB in infancy are associated with better outcome and preservation of renal function. CIC is associated with better preservation of kidney function, while vesicostomy is associated with a worse outcome. Children with NB caused by SL experienced the best kidney function preservation, while those with VBS experienced the worst.

Background and Aims Approximately 50% of children with steroid sensitive nephrotic syndrome (SSNS) will suffer from frequent relapses or steroid dependency, prompting the use of so-called steroid-sparing drugs. In this pilot study, we compare the efficacy and safety of rituximab to oral cyclophosphamide as first-line steroid-sparing medications Method A prospective open label randomized study of children with frequent relapsing or steroid-dependant SSNS. Exclusion criteria were steroid-resistant disease, prescription of immunosuppressive agents other than prednisolone or levamisole, evidence of impaired kidney function, leucopenia or active infection. The recruited children were allocated either to the oral cyclophosphamide (3mg/kg/day for 8 weeks) or intravenous rituximab treatment (two doses of 375 mg/m2/dose, 2 weeks apart) and were monitored for relapses and side effects for 12 months Results 46 subjects were included from two centers; 27 received cyclophosphamide and 19 received rituximab. One-year relapse-free survival was reached in 17 (58.6%) patients treated with cyclophosphamide compared to 16 (84.2%) with rituximab (adjusted HR: 0.36; 95% CI: 0.09 – 1.45; p=0.151). The mean interval to relapse was 6.9 months in the cyclophosphamide group (N=10) and 6.3 months in the rituximab group (N=3). Both treatments were associated with a significant (p=?) reduction in prescribed dose of oral alternate days steroid from 1.02 to 0.36mg/kg (Cyclophoshamide) and 0.86 to 0.08mg/kg (Rituximab). Importantly, a significantly (P = 0.003) higher percentage of patients achieved complete withdrawal of steroid within 3 months of commencing study treatment in the rituximab (73.7%) versus cyclophosphamide (29.6%) group. Transient leucopenia was the most frequent adverse effect observed in the cyclophosphamide group (18.5%) and one patient (3.4%) had acute hepatotoxicity beside severe leucopenia and neutropenia on the 7th week of treatment although she showed complete recovery with withdrawal of cyclophosphamide and maintained remission. A minor infusion related reaction was observed in 1 patient (5%) in the rituximab group. Conclusion Rituximab is non-inferior to cyclophosphamide and safe as a first-line steroid-sparing agent in children with SSNS. A larger multicenter study is required to assess superiority over cyclophosphamide