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Additional file 2. PICAR statement.

Additional file 1. Search strategy.

Additional file 3. PRISMA checklist.

Additional file 4. Evidence-Base Classifications.

Background: Nephrotic syndrome is the most common kidney disease in children worldwide. Our aim was to critically appraise the quality of recent Clinical Practice Guidelines (CPGs) for idiopathic steroid-sensitive nephrotic syndrome (SSNS) in children in addition to summarize and compare their recommendations. Methods: Systematic review of CPGs. We identified clinical questions and eligibility criteria and searched and screened for CPGs using bibliographic and CPG databases. Each included CPG was assessed by four independent appraisers using the Appraisal of Guidelines for REsearch & Evaluation II (AGREE-II) instrument. We summarized recommendations in a comparison matrix. Results: Our search retrieved 282 citations, of which three CPGs were eligible and appraised: Kidney Disease: Improving Global Outcomes (KDIGO) 2012, Japan Society for Pediatric Nephrology (JSPN) 2014, and American Academy of Pediatrics (AAP) 2009. Among these, the overall assessment of two evidence-based CPGs scored > 70% (KDIGO and JSPN), which was consistent with their higher scores in the six domains of the AGREE II Instrument. In domain 3 (rigor of development), KDIGO, JSPN, and AAP scored 84%, 74%, and 41%, respectively. In domain 5 (applicability), they scored 22%, 16%, and 19%, respectively, and in domain 6 (editorial independence), they scored 94%, 65%, and 88%, respectively. Conclusions: The methodological quality of the KDIGO CPG was superior, followed by JSPN and AAP CPGs with the relevant recommendations for use in practice. Systematic review registration: The protocol was registered in the Center for Open Science (OSF) DOI: 10.17605/OSF.IO/6QTMD.

Background and objectives: The standard International Study of Kidney Disease in Children (ISKDC) regimen of prednisolone of 2 months duration for the treatment of the initial episode of Idiopathic Nephrotic Syndrome (INS) was associated with a high relapse rate. The long prednisolone protocols were introduced in order to reduce the relapse rate and steroid toxicities. The main objective of this study was to assess the efficacy and safety of a 3 months protocol of prednisolone versus a 7 months protocol for the first episode of idiopathic nephrotic syndrome. Design and setting: The study took place in the Pediatric Nephrology Department of King Saud Medical City, Riyadh which is a large referral center all over Saudi Arabia. The study was a randomized control trial using 2 groups. Group A received the 3 months protocol and Group B received the 7 months protocol. Patients and methods: All children with a confirmed diagnosis of Idiopathic Nephrotic Syndrome were included. The patients were randomized by simple randomization using sealed envelopes into two groups; group A comprised of 60 children using the daily regimen prednisolone 60 mg/m2 OD X 1 ½ months then 40mg/m2 on alternate day for 1 ½ months (total = 3 months) and group B also comprised of 60 children using the 7 months protocol, Prednisolone 60mg/m2 OD x 1 month then 40mg/m2 EOD x 2 months then 30mg/m2 EOD for 2 months then 20mg/m2 EOD for 2 months. The efficacy and safety of these two prednisolone regimens were recorded. The follow-up period was two years. Statistical analysis was done using the SPSS progress version 16 (Chicago, USA) P

Background and objectives: To assess the efficacy and safety of second line drugs used at our center in frequently relapsing and steroid dependant (FR/SD) childhood nephrotic syndrome. Patients and methods: This was a retrospective study over a period of 3 years (July 2012 to July 2015) on the use of 4 second line drugs in FR/SD nephrotic syndrome in children treated at our center. These drugs were Levamisole, Mycophenolate Mofetil (MMF), Cyclophosphamide, and Cyclosporine. We studied the relapse rate per year, cumulative dose of steroids, success, failure, and side effects of these drugs. Statistical analyses were done with the help of a statistician using the T-test and the “N-1”Chi–Square test. Results: We reviewed the charts of 60 children. All had FR/SD nephrotic syndrome and received a 3 month protocol of prednisolone. 20 received Levamisole (33%), 12 received Cyclophosphamide (20%), 20 received MMF (25%), and 13 received Cyclosporine (22%). All the four drugs significantly reduced the relapse rate and the cumulative dose of steroids (P