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


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          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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          The online version of this article (10.1007/s00467-020-04519-1) contains supplementary material, which is available to authorized users.

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          Most cited references216

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          Anthropometric reference data for international use: recommendations from a World Health Organization Expert Committee.

          The World Health Organization (WHO) convened an Expert Committee to reevaluate the use of anthropometry at different ages for assessing health, nutrition, and social wellbeing. The Committee's task included identifying reference data for anthropometric indexes when appropriate, and providing guidelines on how the data should be used. For fetal growth, the Committee recommended an existing sex-specific multiracial reference. In view of the significant technical drawbacks of the current National Center for Health Statistics (NCHS)/WHO reference and its inadequacy for assessing the growth of breast-fed infants, the Committee recommended the development of a new reference concerning weight and length/height for infants and children, which will be a complex and costly undertaking. Proper interpretation of midupper arm circumference for preschoolers requires age-specific reference data. To evaluate adolescent height-for-age, the Committee recommended the current NCHS/WHO reference. Use of the NCHS body mass index (BMI) data, with their upper percentile elevations and skewness, is undesirable for setting health goals; however, these data were provisionally recommended for defining obesity based on a combination of elevated BMI and high subcutaneous fat. The NCHS values were provisionally recommended as reference data for subscapular and triceps skinfold thicknesses. Guidelines were also provided for adjusting adolescent anthropometric comparisons for maturational status. Currently, there is no need for adult reference data for BMI; interpretation should be based on pragmatic BMI cutoffs. Finally, the Committee noted that few normative anthropometric data exist for the elderly, especially for those > 80 y of age. Proper definitions of health status, function, and biologic age remain to be developed for this group.
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            A Reporting Tool for Practice Guidelines in Health Care: The RIGHT Statement.

            The quality of reporting practice guidelines is often poor, and there is no widely accepted guidance or standards for such reporting in health care. The international RIGHT (Reporting Items for practice Guidelines in HealThcare) Working Group was established to address this gap. The group followed an existing framework for developing guidelines for health research reporting and the EQUATOR (Enhancing the QUAlity and Transparency Of health Research) Network approach. It developed a checklist and an explanation and elaboration statement. The RIGHT checklist includes 22 items that are considered essential for good reporting of practice guidelines: basic information (items 1 to 4), background (items 5 to 9), evidence (items 10 to 12), recommendations (items 13 to 15), review and quality assurance (items 16 and 17), funding and declaration and management of interests (items 18 and 19), and other information (items 20 to 22). The RIGHT checklist can assist developers in reporting guidelines, support journal editors and peer reviewers when considering guideline reports, and help health care practitioners understand and implement a guideline.
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              Idiopathic nephrotic syndrome in children

              The incidence of idiopathic nephrotic syndrome (NS) is 1·15-16·9 per 100 000 children, varying by ethnicity and region. The cause remains unknown but the pathogenesis of idiopathic NS is thought to involve immune dysregulation, systemic circulating factors, or inherited structural abnormalities of the podocyte. Genetic risk is more commonly described among children with steroid-resistant disease. The mainstay of therapy is prednisone for the vast majority of patients who are steroid responsive; however, the disease can run a frequently relapsing course, necessitating the need for alternative immunosuppressive agents. Infection and venous thromboembolism are the main complications of NS with also increased risk of acute kidney injury. Prognosis in terms of long-term kidney outcome overall is excellent for steroid-responsive disease, and steroid resistance is an important determinant of future risk of chronic or end-stage kidney disease.

                Author and article information

                Pediatr Nephrol
                Pediatr. Nephrol
                Pediatric Nephrology (Berlin, Germany)
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                7 May 2020
                7 May 2020
                : 35
                : 8
                : 1529-1561
                [1 ]GRID grid.7700.0, ISNI 0000 0001 2190 4373, Division of Pediatric Nephrology, , Center for Pediatrics and Adolescent Medicine, ; Heidelberg, Germany
                [2 ]GRID grid.414125.7, ISNI 0000 0001 0727 6809, Department of Pediatric Subspecialties, Division of Nephrology and Dialysis, , Bambino Gesù Pediatric Hospital and Research Center, ; Rome, Italy
                [3 ]GRID grid.22072.35, ISNI 0000 0004 1936 7697, Department of Pediatrics, Section of Pediatric Nephrology, Alberta Children’s Hospital, , University of Calgary, ; Calgary, Canada
                [4 ]GRID grid.214458.e, ISNI 0000000086837370, Division of Nephrology, , University of Michigan, ; Ann Arbor, MI USA
                [5 ]GRID grid.413618.9, ISNI 0000 0004 1767 6103, Department of Pediatrics, Division of Nephrology, , All India Institute of Medical Sciences, ; New Delhi, India
                [6 ]GRID grid.4280.e, ISNI 0000 0001 2180 6431, Department of Paediatrics, Yong Loo Lin School of Medicine, , National University of Singapore, ; Singapore, Singapore
                [7 ]GRID grid.10992.33, ISNI 0000 0001 2188 0914, Laboratory of Hereditary Kidney Diseases, Imagine Institute, INSERM U1163, , Paris Descartes University, ; Paris, France
                [8 ]GRID grid.412134.1, ISNI 0000 0004 0593 9113, Department of Pediatric Nephrology, Reference Center for Idiopathic Nephrotic Syndrome in Children and Adults, , Necker Hospital, APHP, ; 75015 Paris, France
                [9 ]GRID grid.5337.2, ISNI 0000 0004 1936 7603, Department of Pediatric Nephrology, Bristol Royal Hospital for Children, , University of Bristol, ; Bristol, UK
                [10 ]GRID grid.411249.b, ISNI 0000 0001 0514 7202, Hospital Samaritano and HRim/UNIFESP, , Federal University of São Paulo, ; São Paulo, Brazil
                [11 ]GRID grid.411668.c, ISNI 0000 0000 9935 6525, Department of Nephrology, , University Hospital Erlangen, ; Erlangen, Germany
                [12 ]GRID grid.411097.a, ISNI 0000 0000 8852 305X, Institute of Pathology, , University Hospital of Cologne, ; Cologne, Germany
                [13 ]GRID grid.443909.3, ISNI 0000 0004 0385 4466, Department of Nephrology, Luis Calvo Mackenna Children’s Hospital, , University of Chile, ; Santiago, Chile
                [14 ]GRID grid.411333.7, ISNI 0000 0004 0407 2968, Department of Nephrology, , Children’s Hospital of Fudan University, ; Shanghai, China
                [15 ]Department of Pediatrics, Prince Court Medical Centre, Kuala Lumpur, Malaysia
                [16 ]GRID grid.261331.4, ISNI 0000 0001 2285 7943, The Research Institute at Nationwide Children’s Hospital, , The Ohio State University, ; Columbus, OH USA
                [17 ]GRID grid.412738.b, Department of Paediatrics, , University of Port Harcourt Teaching Hospital, ; Port Harcourt, Rivers State Nigeria
                [18 ]GRID grid.267625.2, ISNI 0000 0001 0685 5104, Department of Child Health and Welfare (Pediatrics), Graduate School of Medicine, , University of the Ryukyus, ; Okinawa, Japan
                [19 ]GRID grid.1013.3, ISNI 0000 0004 1936 834X, 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
                [20 ]GRID grid.10423.34, ISNI 0000 0000 9529 9877, Department of Paediatric Kidney, Liver and Metabolic Diseases, , Hannover Medical School Children’s Hospital, ; Hannover, Germany
                [21 ]GRID grid.10423.34, ISNI 0000 0000 9529 9877, Department of Paediatric Kidney, Liver and Metabolic Diseases, Paediatric Research Center, , Hannover Medical School, ; Carl-Neuberg-Str. 1, 30625 Hannover, Germany
                [22 ]GRID grid.10423.34, ISNI 0000 0000 9529 9877, Center for Rare Diseases, , Hannover Medical School Children’s Hospital, ; Hannover, Germany
                © The Author(s) 2020

                Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

                : 21 December 2019
                : 7 February 2020
                : 21 February 2020
                Funded by: The International Pediatric Nephrology Association (IPNA)
                Custom metadata
                © IPNA 2020

                steroid-resistant nephrotic syndrome,children,chronic kidney disease,genetics,outcome,pediatrics,immunosuppressive treatment


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