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

      review-article
      1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , , on behalf of the International Pediatric Nephrology Association
      Pediatric Nephrology (Berlin, Germany)
      Springer Berlin Heidelberg
      Steroid-sensitive nephrotic syndrome, SSNS, Children, Frequently relapsing nephrotic syndrome, Steroid-dependent nephrotic syndrome, Steroid toxicity, Pediatrics, Immunosuppressive treatment

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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.

          Supplementary Information

          The online version contains supplementary material available at 10.1007/s00467-022-05739-3.

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          A Novel Coronavirus from Patients with Pneumonia in China, 2019

          Summary In December 2019, a cluster of patients with pneumonia of unknown cause was linked to a seafood wholesale market in Wuhan, China. A previously unknown betacoronavirus was discovered through the use of unbiased sequencing in samples from patients with pneumonia. Human airway epithelial cells were used to isolate a novel coronavirus, named 2019-nCoV, which formed a clade within the subgenus sarbecovirus, Orthocoronavirinae subfamily. Different from both MERS-CoV and SARS-CoV, 2019-nCoV is the seventh member of the family of coronaviruses that infect humans. Enhanced surveillance and further investigation are ongoing. (Funded by the National Key Research and Development Program of China and the National Major Project for Control and Prevention of Infectious Disease in China.)
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            Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents

            These pediatric hypertension guidelines are an update to the 2004 "Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents." Significant changes in these guidelines include (1) the replacement of the term "prehypertension" with the term "elevated blood pressure," (2) new normative pediatric blood pressure (BP) tables based on normal-weight children, (3) a simplified screening table for identifying BPs needing further evaluation, (4) a simplified BP classification in adolescents ≥13 years of age that aligns with the forthcoming American Heart Association and American College of Cardiology adult BP guidelines, (5) a more limited recommendation to perform screening BP measurements only at preventive care visits, (6) streamlined recommendations on the initial evaluation and management of abnormal BPs, (7) an expanded role for ambulatory BP monitoring in the diagnosis and management of pediatric hypertension, and (8) revised recommendations on when to perform echocardiography in the evaluation of newly diagnosed hypertensive pediatric patients (generally only before medication initiation), along with a revised definition of left ventricular hypertrophy. These guidelines include 30 Key Action Statements and 27 additional recommendations derived from a comprehensive review of almost 15 000 published articles between January 2004 and July 2016. Each Key Action Statement includes level of evidence, benefit-harm relationship, and strength of recommendation. This clinical practice guideline, endorsed by the American Heart Association, is intended to foster a patient- and family-centered approach to care, reduce unnecessary and costly medical interventions, improve patient diagnoses and outcomes, support implementation, and provide direction for future research.
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              Extended international (IOTF) body mass index cut-offs for thinness, overweight and obesity : Extended international BMI cut-offs

              The international (International Obesity Task Force; IOTF) body mass index (BMI) cut-offs are widely used to assess the prevalence of child overweight, obesity and thinness. Based on data from six countries fitted by the LMS method, they link BMI values at 18 years (16, 17, 18.5, 25 and 30 kg m(-2)) to child centiles, which are averaged across the countries. Unlike other BMI references, e.g. the World Health Organization (WHO) standard, these cut-offs cannot be expressed as centiles (e.g. 85th). To address this, we averaged the previously unpublished L, M and S curves for the six countries, and used them to derive new cut-offs defined in terms of the centiles at 18 years corresponding to each BMI value. These new cut-offs were compared with the originals, and with the WHO standard and reference, by measuring their prevalence rates based on US and Chinese data. The new cut-offs were virtually identical to the originals, giving prevalence rates differing by < 0.2% on average. The discrepancies were smaller for overweight and obesity than for thinness. The international and WHO prevalences were systematically different before/after age 5. Defining the international cut-offs in terms of the underlying LMS curves has several benefits. New cut-offs are easy to derive (e.g. BMI 35 for morbid obesity), and they can be expressed as BMI centiles (e.g. boys obesity = 98.9th centile), allowing them to be compared with other BMI references. For WHO, median BMI is relatively low in early life and high at older ages, probably due to its method of construction. © 2012 The Authors. Pediatric Obesity © 2012 International Association for the Study of Obesity.
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                Author and article information

                Contributors
                Haffner.Dieter@mh-hannover.de
                Journal
                Pediatr Nephrol
                Pediatr Nephrol
                Pediatric Nephrology (Berlin, Germany)
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                0931-041X
                1432-198X
                21 October 2022
                21 October 2022
                : 1-43
                Affiliations
                [1 ]GRID grid.7700.0, ISNI 0000 0001 2190 4373, Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, , University of Heidelberg, ; Heidelberg, Germany
                [2 ]GRID grid.508487.6, ISNI 0000 0004 7885 7602, Department of Pediatric Nephrology, Reference Center for Idiopathic Nephrotic Syndrome in Children and Adults, Imagine Institute, , Paris University, Necker Children’s Hospital, APHP, ; Paris, France
                [3 ]GRID grid.413973.b, ISNI 0000 0000 9690 854X, Cochrane Kidney and Transplant, Centre for Kidney Research, , The Children’s Hospital at Westmead, ; Sydney, Australia
                [4 ]GRID grid.413618.9, ISNI 0000 0004 1767 6103, Division of Nephrology, Department of Pediatrics, , All India Institute of Medical Sciences, ; New Delhi, India
                [5 ]GRID grid.214458.e, ISNI 0000000086837370, Department of Pediatrics, Division of Nephrology, , University of Michigan, ; Ann Arbor, MI USA
                [6 ]GRID grid.22072.35, ISNI 0000 0004 1936 7697, Section of Pediatric Nephrology, Department of Pediatrics, Alberta Children’s Hospital Research Institute, , University of Calgary, ; Calgary, Canada
                [7 ]GRID grid.10417.33, ISNI 0000 0004 0444 9382, Department of Nephrology, , Radboud University Medical Center, ; Nijmegen, The Netherlands
                [8 ]GRID grid.56302.32, ISNI 0000 0004 1773 5396, Pediatric Department, College of Medicine, , King Saud University, ; Riyadh, Saudi Arabia
                [9 ]GRID grid.414710.7, ISNI 0000 0004 1801 0469, Department of Pediatric Nephrology, , Institute of Child Health, ; Kolkata, India
                [10 ]GRID grid.16463.36, ISNI 0000 0001 0723 4123, University of KwaZulu-Natal, ; Durban, South Africa
                [11 ]GRID grid.267034.4, ISNI 0000 0001 0153 191X, Department of Pediatrics, , University of Puerto Rico-Medical Sciences Campus, ; San Juan, Puerto Rico
                [12 ]GRID grid.443909.3, ISNI 0000 0004 0385 4466, Department of Pediatric Nephrology, , Luis Calvo Mackenna Children’s Hospital, University of Chile, ; Santiago, Chile
                [13 ]Children’s Kidney Unit, Nottingham Children’s Hospital, Nottingham, UK
                [14 ]GRID grid.413973.b, ISNI 0000 0000 9690 854X, Division of Pediatric Nephrology, Department of Paediatrics, , The Children’s Hospital at Westmead, ; Sydney, Australia
                [15 ]GRID grid.31501.36, ISNI 0000 0004 0470 5905, Division of Pediatric Nephrology, Department of Pediatrics, , Seoul National University Children’s Hospital & Seoul National University College of Medicine, ; Seoul, Korea
                [16 ]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
                [17 ]GRID grid.7776.1, ISNI 0000 0004 0639 9286, Pediatric Nephrology Unit, Faculty of Medicine, , Cairo University, ; Cairo, Egypt
                [18 ]GRID grid.214458.e, ISNI 0000000086837370, Department of Pediatrics, Division of Nephrology, , University of Michigan, ; Ann Arbor, MI USA
                [19 ]GRID grid.411333.7, ISNI 0000 0004 0407 2968, Department of Nephrology, , Children’s Hospital of Fudan University, ; Shanghai, China
                [20 ]Nephrotic Syndrome Trust (NeST), Somerset, UK
                [21 ]GRID grid.414125.7, ISNI 0000 0001 0727 6809, Division of Nephrology and Dialysis, Department of Pediatric Subspecialties, , Bambino Gesù Pediatric Hospital IRCCS, ; Rome, Italy
                [22 ]GRID grid.10423.34, ISNI 0000 0000 9529 9877, Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children’s Hospital, Hannover and Center for Rare Diseases, , Hannover Medical School, ; Hannover, Germany
                Author information
                http://orcid.org/0000-0002-9601-7813
                Article
                5739
                10.1007/s00467-022-05739-3
                9589698
                36269406
                eee04fde-0844-4279-bcb2-c3cbabcf6f19
                © The Author(s) 2022

                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/.

                History
                : 15 June 2022
                : 3 August 2022
                : 22 August 2022
                Funding
                Funded by: International Society of Pediatric Nephrology (IPNA)
                Funded by: Project Deal
                Award ID: for Open Access
                Funded by: Medizinische Hochschule Hannover (MHH) (3118)
                Categories
                Guidelines

                Nephrology
                steroid-sensitive nephrotic syndrome,ssns,children,frequently relapsing nephrotic syndrome,steroid-dependent nephrotic syndrome,steroid toxicity,pediatrics,immunosuppressive treatment

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