Item type |
デフォルトアイテムタイプ(フル)(1) |
公開日 |
2020-10-12 |
タイトル |
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タイトル |
IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome |
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言語 |
en |
作成者 |
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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アクセス権 |
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アクセス権 |
open access |
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アクセス権URI |
http://purl.org/coar/access_right/c_abf2 |
主題 |
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言語 |
en |
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主題Scheme |
Other |
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主題 |
Steroid-resistant nephrotic syndrome |
主題 |
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言語 |
en |
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主題Scheme |
Other |
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主題 |
Children |
主題 |
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言語 |
en |
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主題Scheme |
Other |
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主題 |
Chronic kidney disease |
主題 |
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言語 |
en |
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主題Scheme |
Other |
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主題 |
Genetics |
主題 |
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言語 |
en |
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主題Scheme |
Other |
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主題 |
Outcome |
主題 |
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言語 |
en |
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主題Scheme |
Other |
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主題 |
Pediatrics |
主題 |
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言語 |
en |
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主題Scheme |
Other |
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主題 |
Immunosuppressive treatment |
内容記述 |
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内容記述タイプ |
Other |
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内容記述 |
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. |
内容記述 |
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内容記述タイプ |
Other |
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内容記述 |
論文 |
出版者 |
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出版者 |
Springer |
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言語 |
en |
言語 |
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言語 |
eng |
資源タイプ |
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資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
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資源タイプ |
journal article |
出版タイプ |
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出版タイプ |
VoR |
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出版タイプResource |
http://purl.org/coar/version/c_970fb48d4fbd8a85 |
識別子 |
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識別子 |
http://hdl.handle.net/20.500.12000/46947 |
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識別子タイプ |
HDL |
関連情報 |
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識別子タイプ |
DOI |
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関連識別子 |
https://doi.org/10.1007/s00467-020-04519-1 |
関連情報 |
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識別子タイプ |
DOI |
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関連識別子 |
https://doi.org/10.1007/s00467-020-04519-1 |
収録物識別子 |
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収録物識別子タイプ |
ISSN |
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収録物識別子 |
0931-041X |
収録物識別子 |
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収録物識別子タイプ |
ISSN |
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収録物識別子 |
1432-198X |
収録物名 |
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収録物名 |
Pediatric Nephrology |
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言語 |
en |
書誌情報 |
巻 35,
号 8,
p. 1529-1561,
発行日 2020-05-07
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