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IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome.

Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany. Department of Pediatric Subspecialties, Division of Nephrology and Dialysis, Bambino Gesù Pediatric Hospital and Research Center, Rome, Italy. Department of Pediatrics, Section of Pediatric Nephrology, Alberta Children's Hospital, University of Calgary, Calgary, Canada. Division of Nephrology, University of Michigan, Ann Arbor, MI, USA. Department of Pediatrics, Division of Nephrology, All India Institute of Medical Sciences, New Delhi, India. Department of Paediatrics, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore. Laboratory of Hereditary Kidney Diseases, Imagine Institute, INSERM U1163, Paris Descartes University, Paris, France. Department of Pediatric Nephrology, Reference Center for Idiopathic Nephrotic Syndrome in Children and Adults, Necker Hospital, APHP, 75015, Paris, France. Department of Pediatric Nephrology, Bristol Royal Hospital for Children, University of Bristol, Bristol, UK. Hospital Samaritano and HRim/UNIFESP, Federal University of São Paulo, São Paulo, Brazil. Department of Nephrology, University Hospital Erlangen, Erlangen, Germany. Institute of Pathology, University Hospital of Cologne, Cologne, Germany. Department of Nephrology, Luis Calvo Mackenna Children's Hospital, University of Chile, Santiago, Chile. Department of Nephrology, Children's Hospital of Fudan University, Shanghai, China. Department of Pediatrics, Prince Court Medical Centre, Kuala Lumpur, Malaysia. The Research Institute at Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA. Department of Paediatrics, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State, Nigeria. Department of Child Health and Welfare (Pediatrics), Graduate School of Medicine, University of the Ryukyus, Okinawa, Japan. Cochrane Kidney and Transplant, Centre for Kidney Research, The Children's Hospital at Westmead and the Sydney School of Public Health, University of Sydney, Sydney, Australia. Department of Paediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children's Hospital, Hannover, Germany. Haffner.Dieter@mh-hannover.de. Department of Paediatric Kidney, Liver and Metabolic Diseases, Paediatric Research Center, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany. Haffner.Dieter@mh-hannover.de. Center for Rare Diseases, Hannover Medical School Children's Hospital, Hannover, Germany. Haffner.Dieter@mh-hannover.de.

Pediatric nephrology (Berlin, Germany). 2020;(8):1529-1561
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Abstract

Idiopathic nephrotic syndrome newly affects 1-3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4-6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given.

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Publication Type : Review

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