IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome.
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 neph...
| Publicado en: | Pediatric Nephrology Vol. 35; no. 8; pp. 1529 - 1562 |
|---|---|
| Autores principales: | , , , , , , , , , , , , , , , , , , , |
| Formato: | algorithm practice guidelines tables/charts Journal Article |
| Publicado: |
Springer Nature
Aug2020
|
| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=144237394&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 144237394 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 0931041X EF1 jtl: Pediatric Nephrology issn: 0931041X maglogo: N pubinfo: dt: Aug2020 vid: 35 iid: 8 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 144237394 144144617 144237394 144237394 10.1007/s00467-020-04519-1 144237394 ppf: 1529 ppct: 33 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome. aug: au: 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 affil: Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany sug: subj: Nephrotic Syndrome Diagnosis Nephrotic Syndrome Drug Therapy Glucocorticoids Therapeutic Use Drug Resistance Nephrology Organizations International Agencies Practice Guidelines Child Nephrotic Syndrome Familial and Genetic Mutation Nephrotic Syndrome Immunology Immunosuppressive Agents Therapeutic Use Immunosuppression Nephrotic Syndrome Complications Kidney Failure, Chronic Child: 6-12 years ab: 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. pubtype: Academic Journal doctype: algorithm practice guidelines tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
|---|