TY - JOUR
T1 - IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome
AU - Trautmann, Agnes
AU - Boyer, Olivia
AU - Hodson, Elisabeth
AU - Bagga, Arvind
AU - Gipson, Debbie S.
AU - Samuel, Susan
AU - Wetzels, Jack
AU - Alhasan, Khalid
AU - Banerjee, Sushmita
AU - Bhimma, Rajendra
AU - Bonilla-Felix, Melvin
AU - Cano, Francisco
AU - Christian, Martin
AU - Hahn, Deirdre
AU - Kang, Hee Gyung
AU - Nakanishi, Koichi
AU - Safouh, Hesham
AU - Trachtman, Howard
AU - Xu, Hong
AU - Cook, Wendy
AU - Vivarelli, Marina
AU - Haffner, Dieter
AU - on behalf of the International Pediatric Nephrology Association
AU - Bouts, Antonia
AU - Dossier, Claire
AU - Emma, Francesco
AU - Kemper, Markus
AU - Topaloglu, Rezan
AU - Waters, Aoife
AU - Thorsten Weber, Lutz
AU - Zurowska, Alexandra
AU - Gibson, Keisha L.
AU - Greenbaum, Larry
AU - Massengill, Susan
AU - Selewski, David
AU - Srivastava, Tarak
AU - Wang, Chia-shi
AU - Wenderfer, Scott
AU - Johnstone, Lilian
AU - Larkins, Nicholas
AU - Wong, William
AU - Alba, Agnes A.
AU - Ha, T. S.
AU - Mokham, Masoumeh
AU - Zhong, Xuhui
AU - Hamada, Riku
AU - Iijima, Kazumoto
AU - Ishikura, Kenji
AU - Nozu, Kandai
AU - Bresolin, Nilzete
AU - de Jesus Gonzalez, Nilka
N1 - Funding Information: The authors acknowledge the valuable contributions of the members of the voting panel representing the IPNA regional societies, i.e., ESPN: Antonia Bouts (Amsterdam, Netherlands), Claire Dossier (Paris, France), Francesco Emma (Rome, Italy), Markus Kemper (Hamburg, Germany), Rezan Topaloglu (Ankara, Turkey), Aoife Waters (London, United Kingdom), Lutz Thorsten Weber (Cologne, Germany), Alexandra Zurowska (Gdansk, Poland); ASPN: Keisha L. Gibson (Chapel Hill, United States), Larry Greenbaum (Atlanta, United States), Susan Massengill (Charlotte, United States), David Selewski (Charleston, United States), Tarak Srivastava (Kansas City, United States), Chia-shi Wang (Atlanta, United States), Scott Wenderfer (Vancouver, Canada); ANZPNA: Lilian Johnstone (Clayton, Australia), Nicholas Larkins (Perth, Australia), William Wong (Auckland, New Zealand); AsPNA: Agnes A. Alba (Quezon City, Philippines), TS Ha (Cheongju, Korea), Masoumeh Mokham (Tehran, Iran), Xuhui Zhong (Bejing, China); JSPN: Riku Hamada (Tokyo, Japan), Kazumoto Iijima (Kobe, Japan), Kenji Ishikura, (Sagamihara, Japan), Kandai Nozu (Kobe, Japan); ALANEPE: Nilzete Bresolin (Florianópolis, Brazil), Nilka De Jesus Gonzalez (San Juan, Puerto Rico), Jaime Restrepo (Cali, Colombia); AFPNA: Ifeoma Anochie (Port Harcourt, Nigeria), Mignon McCulloch (Cape Town, South Africa). The authors would also like to thank the following acting as external experts; pediatric endocrinologists: Agnes Linglart (Paris, France), Dirk Schnabel (Berlin, Germany); general pediatricians: Adamu Sambo (Gloucestershire, United Kingdom); experts in transition: Marjo van Helden (Nijmegen, Netherlands), Ben Sprangers (Leuven, Belgium); dieticians: Stefanie Steinmann (Hannover, Germany), Sheridan Collins (Sydney, Australia), Katie Byrne (Ann Arbor, United States); and patient representatives: Clemens and Juliane Brauner (Hannover, Germany), Chandana Guha (Sydney, Australia), Stephane Serre (Toulouse, France). Publisher Copyright: © 2022, The Author(s).
PY - 2023/3
Y1 - 2023/3
N2 - 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.
AB - 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.
KW - Children
KW - Frequently relapsing nephrotic syndrome
KW - Immunosuppressive treatment
KW - Pediatrics
KW - SSNS
KW - Steroid toxicity
KW - Steroid-dependent nephrotic syndrome
KW - Steroid-sensitive nephrotic syndrome
UR - http://www.scopus.com/inward/record.url?scp=85140356200&partnerID=8YFLogxK
U2 - https://doi.org/10.1007/s00467-022-05739-3
DO - https://doi.org/10.1007/s00467-022-05739-3
M3 - Article
C2 - 36269406
SN - 0931-041X
VL - 38
SP - 877
EP - 919
JO - Pediatric nephrology (Berlin, Germany)
JF - Pediatric nephrology (Berlin, Germany)
IS - 3
ER -