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

資料種別:
論文(リポジトリ)
責任表示:
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 ; International Pediatric Nephrology Association
言語:
英語
出版情報:
Springer, 2020-05-07
著者名:
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
International Pediatric Nephrology Association
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掲載情報:
Pediatric Nephrology
ISSN:
0931-041X  CiNii Articles  Webcat Plus  JAIRO
1432-198X  CiNii Articles  Webcat Plus  JAIRO
巻:
35
通号:
8
開始ページ:
1529
終了ページ:
1561
バージョン:
publisher
概要:
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. 続きを見る
URL:
http://hdl.handle.net/20.500.12000/46947
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