子供におけるイディオパシー腎症候群
Damien G Noone1, Kazumoto Iijima2, Rulan Parekh3
1Department of Paediatrics, University of Toronto, Toronto, ON, Canada; Division of Nephrology, The Hospital for Sick Children, Toronto, ON, Canada.
Lancet (London, England)
|June 19, 2018
まとめ
子供のイディオパシー腎症候群 (Idiopathic nephrotic syndrome,NS) は,発生頻度が異なっており,原因は不明で,しばしば免疫問題や遺伝要因が関係しています. ステロイドに反応するNSは 予後が優れているが,ステロイド抵抗は腎疾患のリスクが高いことを示している.
科学分野:
- 小児腎臓科
- 免疫学
- 遺伝学
背景:
- Idiopathic nephrotic syndrome (NS) は10万人に1. 15~16. 9人の子供に発症し,その発生率は民族や地域によって異なります.
- 病原化は複雑で,潜在的に免疫不調,循環因子,またはポドサイト異常を含む.
- 遺伝的要因は,ステロイド耐性NSにより関与しています.
研究 の 目的:
- 子供のイディオパシー性腎症候群の現在の理解を要約します
- 病原性,治療,合併症,および予後に関する重要な側面を強調する.
主な方法:
- イディオパシー性腎症候群に関する既存の文献のレビュー
- 疫学的データ,病原性メカニズム,治療方法,予後要因の分析
主要な成果:
- 主な治療はステロイドに反応する患者に対するプレドニゾーンです.
- 頻繁に再発すると,代替免疫抑制薬が必要になる.
- 主な合併症には,感染,静脈血栓塞栓症,急性腎臓損傷があります.
結論:
- ステロイドに反応するNSは,通常,長期間,優れた腎臓の結果を示します.
- ステロイド耐性は,慢性または末期腎疾患の将来のリスクを決定する重要な要因です.
- ステロイド耐性症例の病原性と治療に関するさらなる研究が必要である.
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