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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

Pediatric nephrology (Berlin, West), 2020-08, Vol.35 (8), p.1529-1561 [Periódico revisado por pares]

Berlin/Heidelberg: Springer Berlin Heidelberg

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Título:
IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome
Autor: 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
Assuntos: Adolescent ; Care and treatment ; Child ; Child, Preschool ; Children ; Clinical medicine ; Developmental stages ; Diagnosis ; Diseases ; Drug Resistance ; End-stage renal disease ; Etiology ; Female ; Glucocorticoids ; Glucocorticoids - administration & dosage ; Glucocorticoids - adverse effects ; Guidelines ; Humans ; Immunosuppressive agents ; Immunosuppressive Agents - therapeutic use ; Infant ; Infant, Newborn ; Kidney diseases ; Kidney transplantation ; Literature reviews ; Male ; Medical diagnosis ; Medical societies ; Medicine ; Medicine & Public Health ; Nephrology ; Nephrotic syndrome ; Nephrotic Syndrome - diagnosis ; Nephrotic Syndrome - drug therapy ; Patients ; Pediatric research ; Pediatrics ; Proteinuria ; Proteinuria - urine ; Remission ; Remission Induction - methods ; Urology
É parte de: Pediatric nephrology (Berlin, West), 2020-08, Vol.35 (8), p.1529-1561
Notas: ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-3
content type line 23
ObjectType-Review-1
ObjectType-Instructional Material/Guideline-4
Descrição: 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 ( P atient or P opulation covered, I ntervention, C omparator, O utcome) 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.
Editor: Berlin/Heidelberg: Springer Berlin Heidelberg
Idioma: Inglês

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

Berlin/Heidelberg: Springer Berlin Heidelberg

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