In a systematic review and meta-analysis of randomized controlled trials (RCT), we aimed to evaluate the benefits and harms of all interventions for children with steroid-resistant nephrotic syndrome (SRNS). Nine RCTs involving 225 children were included. Cyclosporin when compared with placebo or no treatment significantly increased the number of children who achieved complete remission [3 trials, 49 children, relative risk (RR) for persistent nephrotic syndrome 0.64, 95% confidence intervals (CI), 0.47-0.88]. There was no significant difference in the number of children who achieved complete remission between oral cyclophosphamide with prednisone and prednisone alone [2 trials, 91 children, RR 1.01, 95% CI 0.74-1.36], between intravenous cyclophosphamide and oral cyclophosphamide [1 study, 11 children, RR 0.09, 95% CI 0.01-1.39], and between azathioprine with prednisone and prednisone alone [1 trial, 31 children, RR 1.01, 95% CI 0.77-1.32]. No RCTs were identified comparing combination regimens comprising high-dose steroids, alkylating agents or cyclosporin with single agents, placebo, or no treatment. Further adequately powered and well-designed RCTs are needed to confirm the efficacy of cyclosporin and to evaluate regimens of high-dose steroids with alkylating agents or cyclosporin for SRNS.
We report a wide variation of salbutamol use in paediatric emergency departments and a high prevalence of type A adverse drug reactions when continuous-infusion intravenous salbutamol therapy was administered. More evidence is needed on the clinical significance of the adverse effects reported in this study and optimal doses for the safe use of continuous-infusion intravenous salbutamol therapy in paediatric emergency departments.
Persistence of inhibitors against factor VIII (FVIII) may be a risk factor that increases physical disability in haemophilia A (HA) patients. This study aimed to evaluate prevalence of FVIII inhibitors in previously treated children with severe HA and the impact of persistent inhibitors on knee joint status and lumbar bone mineral density (BMD). Fifty children with severe HA, FVIII <1%; aged 5-16 years were enrolled in this study; they received plasma-derived FVIII on-demand treatment for 50-250 exposure days (EDs). Inhibitors were checked at basal visit and were followed up for 1 year, using Bethesda assay. Cross-sectional clinical scoring and radiological evaluation of the knee joint (by Arnold-Hilgartner staging and Pettersson score), along with lumbar BMD by Dual Energy X-ray Absorptiometry (DEXA) were performed. Patients with persistent inhibitors for 1 to 5 years, median 2.5 years, were 10 (20%). Six had high titre and none of them had completely normal knees, seven had advanced knee arthropathy and six had low lumbar BMD in comparison to 2 and 8 of the 40 patients without inhibitors respectively (P < 0.05). Persistence of inhibitors for more than 2 years without immuno-prophylaxis was a risk factor for joint damage. Low lumbar BMD was found in 88.9% of patients with stages four and five knee arthropathy and in 66.7% of patients with positive hepatitis C. Severe HA children in this Egyptian study had a relatively low prevalence of persistent FVIII inhibitors, which, if not treated, may increase the risk of knee arthropathy and lumbar osteopenia.
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