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IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome.
Trautmann, Agnes; Vivarelli, Marina; Samuel, Susan; Gipson, Debbie; Sinha, Aditi; Schaefer, Franz; Hui, Ng Kar; Boyer, Olivia; Saleem, Moin A; Feltran, Luciana; Müller-Deile, Janina; Becker, Jan Ulrich; Cano, Francisco; Xu, Hong; Lim, Yam Ngo; Smoyer, William; Anochie, Ifeoma; Nakanishi, Koichi; Hodson, Elisabeth; Haffner, Dieter.
Afiliación
  • Trautmann A; Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany.
  • Vivarelli M; Department of Pediatric Subspecialties, Division of Nephrology and Dialysis, Bambino Gesù Pediatric Hospital and Research Center, Rome, Italy.
  • Samuel S; Department of Pediatrics, Section of Pediatric Nephrology, Alberta Children's Hospital, University of Calgary, Calgary, Canada.
  • Gipson D; Division of Nephrology, University of Michigan, Ann Arbor, MI, USA.
  • Sinha A; Department of Pediatrics, Division of Nephrology, All India Institute of Medical Sciences, New Delhi, India.
  • Schaefer F; Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany.
  • Hui NK; Department of Paediatrics, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.
  • Boyer O; Laboratory of Hereditary Kidney Diseases, Imagine Institute, INSERM U1163, Paris Descartes University, Paris, France.
  • Saleem MA; Department of Pediatric Nephrology, Reference Center for Idiopathic Nephrotic Syndrome in Children and Adults, Necker Hospital, APHP, 75015, Paris, France.
  • Feltran L; Department of Pediatric Nephrology, Bristol Royal Hospital for Children, University of Bristol, Bristol, UK.
  • Müller-Deile J; Hospital Samaritano and HRim/UNIFESP, Federal University of São Paulo, São Paulo, Brazil.
  • Becker JU; Department of Nephrology, University Hospital Erlangen, Erlangen, Germany.
  • Cano F; Institute of Pathology, University Hospital of Cologne, Cologne, Germany.
  • Xu H; Department of Nephrology, Luis Calvo Mackenna Children's Hospital, University of Chile, Santiago, Chile.
  • Lim YN; Department of Nephrology, Children's Hospital of Fudan University, Shanghai, China.
  • Smoyer W; Department of Pediatrics, Prince Court Medical Centre, Kuala Lumpur, Malaysia.
  • Anochie I; The Research Institute at Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA.
  • Nakanishi K; Department of Paediatrics, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State, Nigeria.
  • Hodson E; Department of Child Health and Welfare (Pediatrics), Graduate School of Medicine, University of the Ryukyus, Okinawa, Japan.
  • Haffner D; 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.
Pediatr Nephrol ; 35(8): 1529-1561, 2020 08.
Article en En | MEDLINE | ID: mdl-32382828
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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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Glucocorticoides / Inmunosupresores / Síndrome Nefrótico Tipo de estudio: Diagnostic_studies / Guideline Límite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Revista: Pediatr Nephrol Asunto de la revista: NEFROLOGIA / PEDIATRIA Año: 2020 Tipo del documento: Article País de afiliación: Alemania Pais de publicación: Alemania
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Glucocorticoides / Inmunosupresores / Síndrome Nefrótico Tipo de estudio: Diagnostic_studies / Guideline Límite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Revista: Pediatr Nephrol Asunto de la revista: NEFROLOGIA / PEDIATRIA Año: 2020 Tipo del documento: Article País de afiliación: Alemania Pais de publicación: Alemania