※一部利用できない機能があります
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:
- 掲載情報:
- Pediatric Nephrology
- ISSN:
- 0931-041X
1432-198X - 巻:
- 35
- 通号:
- 8
- 開始ページ:
- 1529
- 終了ページ:
- 1561
- バージョン:
- VoR
- 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.
論文 続きを見る - URL:
- http://hdl.handle.net/20.500.12000/46947