What question did this study set out to answer?

This study aims to analyze the clinical features and outcomes of pediatric cases diagnosed with C3 glomerulopathy.

April 25, 2026Japanese journal of pediatric nephrology0 citationsOpen Access

Clinicopathological Features and Outcomes of Eight Pediatric Cases of C3 Glomerulopathy

TFTakafumi Fukuda AIAkane Iwasaki EKEriko Kabata

Key Points

This study aims to analyze the clinical features and outcomes of pediatric cases diagnosed with C3 glomerulopathy.
Retrospective examination of 8 pediatric cases diagnosed with C3 glomerulopathy via kidney biopsy from 2019 to 2024.
Evaluation included clinical progression, urinary findings, and pathological characteristics.
Genetic analysis was conducted for variants in pathogenic genes.
Four cases were identified due to abnormal urinalysis, and five out of seven subjects achieved remission.
Six patients received steroid or immunosuppressive therapies, while one achieved remission without treatment.
None of the patients progressed to end-stage renal failure during the median observation period of 2.4 years.

Abstract

【背景および目的】c3腎症は補体代替経路の異常活性化を病因とする稀な糸球体疾患であり，小児例の報告は少ない．【方法】2019～2024年に腎生検で診断した小児c3腎症8症例の臨床経過を後方視的に検討した．【結果】7例がc3腎炎（1例は基底膜菲薄症候群合併で別枠）で，主解析例では検尿異常での発見が4例だった．腎病理所見は，びまん性の膜性増殖性変化は認めず，巣状性変化か，メサンギウム増殖性変化や管内増殖性変化のみの症例もあった．遺伝子解析5例はいずれも既知の病原性遺伝子変異を認めなかった．7例中5例が寛解し，うち1例は無投薬で寛解した．6例にステロイド剤や免疫抑制剤を投与した．非寛解例の2例は感染時に増悪を反復したが，観察期間中央値2.4年で末期腎不全への進行はなかった．【結論】無症状の検尿異常児においてもc3腎症を鑑別に挙げ，病理に加え臨床経過と補体推移を統合して評価すべきである．

Ask AI

Helpful

Bookmark

View Full Paper

Cite This Study

Fukuda et al. (2026) studied this question.

synapsesocial.com/papers/69ec59c688ba6daa22dab713 https://doi.org/10.3165/jjpn.oa.25-028

Ask AI

Helpful

Bookmark

View Full Paper

April 25, 2026Japanese journal of pediatric nephrology0 citationsOpen Access

Clinicopathological Features and Outcomes of Eight Pediatric Cases of C3 Glomerulopathy

TFTakafumi Fukuda AIAkane Iwasaki EKEriko Kabata

Key Points

This study aims to analyze the clinical features and outcomes of pediatric cases diagnosed with C3 glomerulopathy.
Retrospective examination of 8 pediatric cases diagnosed with C3 glomerulopathy via kidney biopsy from 2019 to 2024.
Evaluation included clinical progression, urinary findings, and pathological characteristics.
Genetic analysis was conducted for variants in pathogenic genes.
Four cases were identified due to abnormal urinalysis, and five out of seven subjects achieved remission.
Six patients received steroid or immunosuppressive therapies, while one achieved remission without treatment.
None of the patients progressed to end-stage renal failure during the median observation period of 2.4 years.

Abstract

Ask AI

Helpful

Bookmark

View Full Paper

Cite This Study

Fukuda et al. (2026) studied this question.

synapsesocial.com/papers/69ec59c688ba6daa22dab713 https://doi.org/10.3165/jjpn.oa.25-028

Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

Ask AI

Helpful

Bookmark

View Full Paper