IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome
Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85–90% of patients attain complete remission of pr...
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| Published in | Pediatric nephrology (Berlin, West) Vol. 38; no. 3; pp. 877 - 919 |
|---|---|
| Main Authors | , , , , , , , , , , , , , , , , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
Berlin/Heidelberg
Springer Berlin Heidelberg
01.03.2023
Springer Springer Nature B.V Springer Verlag |
| Subjects | |
| Online Access | Get full text |
| ISSN | 0931-041X 1432-198X 1432-198X |
| DOI | 10.1007/s00467-022-05739-3 |
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| Abstract | Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85–90% of patients attain complete remission of proteinuria within 4–6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70–80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given. |
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| AbstractList | Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85–90% of patients attain complete remission of proteinuria within 4–6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70–80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given. Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85-90% of patients attain complete remission of proteinuria within 4-6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70-80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given.Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85-90% of patients attain complete remission of proteinuria within 4-6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70-80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given. Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85–90% of patients attain complete remission of proteinuria within 4–6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70–80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given. Abstract Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema. Approximately 85–90% of patients attain complete remission of proteinuria within 4–6 weeks of treatment with glucocorticoids, and therefore, have steroid-sensitive nephrotic syndrome (SSNS). Among those patients who are steroid sensitive, 70–80% will have at least one relapse during follow-up, and up to 50% of these patients will experience frequent relapses or become dependent on glucocorticoids to maintain remission. The dose and duration of steroid treatment to prolong time between relapses remains a subject of much debate, and patients continue to experience a high prevalence of steroid-related morbidity. Various steroid-sparing immunosuppressive drugs have been used in clinical practice; however, there is marked practice variation in the selection of these drugs and timing of their introduction during the course of the disease. Therefore, international evidence-based clinical practice recommendations (CPRs) are needed to guide clinical practice and reduce practice variation. The International Pediatric Nephrology Association (IPNA) convened a team of experts including pediatric nephrologists, an adult nephrologist, and a patient representative to develop comprehensive CPRs on the diagnosis and management of SSNS in children. After performing a systematic literature review on 12 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, recommendations were formulated and formally graded at several virtual consensus meetings. New definitions for treatment outcomes to help guide change of therapy and recommendations for important research questions are given. |
| Audience | Academic |
| Author | Hodson, Elisabeth Safouh, Hesham Trautmann, Agnes Boyer, Olivia Bagga, Arvind Xu, Hong Gipson, Debbie S. Wetzels, Jack Alhasan, Khalid Cano, Francisco Nakanishi, Koichi Bonilla-Felix, Melvin Bhimma, Rajendra Haffner, Dieter Cook, Wendy Banerjee, Sushmita Christian, Martin Samuel, Susan Hahn, Deirdre Kang, Hee Gyung Trachtman, Howard Vivarelli, Marina |
| Author_xml | – sequence: 1 givenname: Agnes surname: Trautmann fullname: Trautmann, Agnes organization: Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, University of Heidelberg – sequence: 2 givenname: Olivia surname: Boyer fullname: Boyer, Olivia organization: Department of Pediatric Nephrology, Reference Center for Idiopathic Nephrotic Syndrome in Children and Adults, Imagine Institute, Paris University, Necker Children’s Hospital, APHP – sequence: 3 givenname: Elisabeth surname: Hodson fullname: Hodson, Elisabeth organization: Cochrane Kidney and Transplant, Centre for Kidney Research, The Children’s Hospital at Westmead – sequence: 4 givenname: Arvind surname: Bagga fullname: Bagga, Arvind organization: Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences – sequence: 5 givenname: Debbie S. surname: Gipson fullname: Gipson, Debbie S. organization: Department of Pediatrics, Division of Nephrology, University of Michigan – sequence: 6 givenname: Susan surname: Samuel fullname: Samuel, Susan organization: Section of Pediatric Nephrology, Department of Pediatrics, Alberta Children’s Hospital Research Institute, University of Calgary – sequence: 7 givenname: Jack surname: Wetzels fullname: Wetzels, Jack organization: Department of Nephrology, Radboud University Medical Center – sequence: 8 givenname: Khalid surname: Alhasan fullname: Alhasan, Khalid organization: Pediatric Department, College of Medicine, King Saud University – sequence: 9 givenname: Sushmita surname: Banerjee fullname: Banerjee, Sushmita organization: Department of Pediatric Nephrology, Institute of Child Health – sequence: 10 givenname: Rajendra surname: Bhimma fullname: Bhimma, Rajendra organization: University of KwaZulu-Natal – sequence: 11 givenname: Melvin surname: Bonilla-Felix fullname: Bonilla-Felix, Melvin organization: Department of Pediatrics, University of Puerto Rico-Medical Sciences Campus – sequence: 12 givenname: Francisco surname: Cano fullname: Cano, Francisco organization: Department of Pediatric Nephrology, Luis Calvo Mackenna Children’s Hospital, University of Chile – sequence: 13 givenname: Martin surname: Christian fullname: Christian, Martin organization: Children’s Kidney Unit, Nottingham Children’s Hospital – sequence: 14 givenname: Deirdre surname: Hahn fullname: Hahn, Deirdre organization: Division of Pediatric Nephrology, Department of Paediatrics, The Children’s Hospital at Westmead – sequence: 15 givenname: Hee Gyung surname: Kang fullname: Kang, Hee Gyung organization: Division of Pediatric Nephrology, Department of Pediatrics, Seoul National University Children’s Hospital & Seoul National University College of Medicine – sequence: 16 givenname: Koichi surname: Nakanishi fullname: Nakanishi, Koichi organization: Department of Child Health and Welfare (Pediatrics), Graduate School of Medicine, University of the Ryukyus – sequence: 17 givenname: Hesham surname: Safouh fullname: Safouh, Hesham organization: Pediatric Nephrology Unit, Faculty of Medicine, Cairo University – sequence: 18 givenname: Howard surname: Trachtman fullname: Trachtman, Howard organization: Department of Pediatrics, Division of Nephrology, University of Michigan – sequence: 19 givenname: Hong surname: Xu fullname: Xu, Hong organization: Department of Nephrology, Children’s Hospital of Fudan University – sequence: 20 givenname: Wendy surname: Cook fullname: Cook, Wendy organization: Nephrotic Syndrome Trust (NeST) – sequence: 21 givenname: Marina surname: Vivarelli fullname: Vivarelli, Marina organization: Division of Nephrology and Dialysis, Department of Pediatric Subspecialties, Bambino Gesù Pediatric Hospital IRCCS – sequence: 22 givenname: Dieter orcidid: 0000-0002-9601-7813 surname: Haffner fullname: Haffner, Dieter email: Haffner.Dieter@mh-hannover.de organization: Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children’s Hospital, Hannover and Center for Rare Diseases, Hannover Medical School |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/36269406$$D View this record in MEDLINE/PubMed https://hal.science/hal-03927257$$DView record in HAL |
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| Copyright | The Author(s) 2022 2022. The Author(s). COPYRIGHT 2023 Springer The Author(s) 2022. This work is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License. Distributed under a Creative Commons Attribution 4.0 International License |
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| Keywords | Steroid toxicity Pediatrics Steroid-dependent nephrotic syndrome Steroid-sensitive nephrotic syndrome SSNS Immunosuppressive treatment Children Frequently relapsing nephrotic syndrome |
| Language | English |
| License | 2022. The Author(s). Distributed under a Creative Commons Attribution 4.0 International License: http://creativecommons.org/licenses/by/4.0 Open AccessThis 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/. |
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| Snippet | Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is... Abstract Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally.... |
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| SubjectTerms | Care and treatment Child Children Clinical medicine Diagnosis Edema Glucocorticoids Glucocorticoids - therapeutic use Guidelines Human health and pathology Humans Immunosuppressive agents Immunosuppressive Agents - adverse effects Kidney diseases Life Sciences Literature reviews Medical diagnosis Medicine Medicine & Public Health Methods Morbidity Nephrologists Nephrology Nephrotic syndrome Nephrotic Syndrome - diagnosis Nephrotic Syndrome - drug therapy Nephrotic Syndrome - epidemiology Patients Pediatrics Practice Practice guidelines (Medicine) Proteinuria Proteinuria - drug therapy Recurrence Remission Remission (Medicine) Services Steroids Steroids - adverse effects Transitional care Urology |
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| Title | IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome |
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