Idiopathic nephrotic syndrome in children
Copyright © 2018 Elsevier Ltd. All rights reserved..
The incidence of idiopathic nephrotic syndrome (NS) is 1·15-16·9 per 100 000 children, varying by ethnicity and region. The cause remains unknown but the pathogenesis of idiopathic NS is thought to involve immune dysregulation, systemic circulating factors, or inherited structural abnormalities of the podocyte. Genetic risk is more commonly described among children with steroid-resistant disease. The mainstay of therapy is prednisone for the vast majority of patients who are steroid responsive; however, the disease can run a frequently relapsing course, necessitating the need for alternative immunosuppressive agents. Infection and venous thromboembolism are the main complications of NS with also increased risk of acute kidney injury. Prognosis in terms of long-term kidney outcome overall is excellent for steroid-responsive disease, and steroid resistance is an important determinant of future risk of chronic or end-stage kidney disease.
Errataetall: |
ErratumIn: Lancet. 2018 Jul 28;392(10144):282. - PMID 30064651 |
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Medienart: |
E-Artikel |
Erscheinungsjahr: |
2018 |
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Erschienen: |
2018 |
Enthalten in: |
Zur Gesamtaufnahme - volume:392 |
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Enthalten in: |
Lancet (London, England) - 392(2018), 10141 vom: 07. Juli, Seite 61-74 |
Sprache: |
Englisch |
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Beteiligte Personen: |
Noone, Damien G [VerfasserIn] |
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Links: |
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Themen: |
Immunosuppressive Agents |
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Anmerkungen: |
Date Completed 25.09.2018 Date Revised 25.09.2018 published: Print-Electronic ErratumIn: Lancet. 2018 Jul 28;392(10144):282. - PMID 30064651 Citation Status MEDLINE |
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doi: |
10.1016/S0140-6736(18)30536-1 |
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funding: |
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PPN (Katalog-ID): |
NLM285542443 |
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520 | |a The incidence of idiopathic nephrotic syndrome (NS) is 1·15-16·9 per 100 000 children, varying by ethnicity and region. The cause remains unknown but the pathogenesis of idiopathic NS is thought to involve immune dysregulation, systemic circulating factors, or inherited structural abnormalities of the podocyte. Genetic risk is more commonly described among children with steroid-resistant disease. The mainstay of therapy is prednisone for the vast majority of patients who are steroid responsive; however, the disease can run a frequently relapsing course, necessitating the need for alternative immunosuppressive agents. Infection and venous thromboembolism are the main complications of NS with also increased risk of acute kidney injury. Prognosis in terms of long-term kidney outcome overall is excellent for steroid-responsive disease, and steroid resistance is an important determinant of future risk of chronic or end-stage kidney disease | ||
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