Evidence map›Paper›PMID 40473980›Full record

ArticlePediatric nephrology (Berlin, Germany)2025

Relationship between clinical and pathologic findings and the presence of genetic variants in patients with steroid-resistant nephrotic syndrome.

Koichi Kamei, Kandai Nozu, Tomoko Horinouchi, Nana Sakakibara, Kentaro Nishi, Naoya Fujita, Shuichiro Fujinaga, Hiroshi Kaito, Aya Inaba, Riku Hamada and 11 more

Abstract readMulticenter Study
PubMed Publisher
In one paragraph

Article in Pediatric nephrology (Berlin, Germany), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed, 1 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

21 authors.

Koichi KameiDivision of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1Setagaya-Ku, OkuraTokyo, 157-8535, Japan. kamei-k@ncchd.go.jp.ORCID 0000-0003-3528-6961
Kandai NozuDepartment of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, Japan.
Tomoko HorinouchiDepartment of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, Japan.
Nana SakakibaraDepartment of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, Japan.
Kentaro NishiDivision of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1Setagaya-Ku, OkuraTokyo, 157-8535, Japan.
Naoya FujitaDepartment of Nephrology, Aichi Children's Health and Medical Center, Aichi, Japan.
Shuichiro FujinagaDivision of Nephrology, Saitama Children's Medical Center, Saitama, Japan.
Hiroshi KaitoDepartment of Nephrology, Hyogo Prefectural Kobe Children's Hospital, Hyogo, Japan.
Aya InabaDepartment of Pediatrics, Yokohama City University Medical Center, Kanagawa, Japan.
Riku HamadaDivision of Nephrology and Rheumatology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Yuko ShimaDepartment of Pediatrics, Wakayama Medical University, Wakayama, Japan.
Takayuki OkamotoDepartment of Pediatrics, Hokkaido University Graduate School of Medicine, Hokkaido, Japan.
Junya HashimotoDepartment of Nephrology, Faculty of Medicine, Toho University, Tokyo, Japan.
Masaki YamamotoDepartment of Pediatrics, Seirei Hamamatsu General Hospital, Shizuoka, Japan.
Yoshimitsu GotohDepartment of Pediatric Nephrology, Japanese Red Cross Aichi Medical Center Nagoya Daini Hospital, Aichi, Japan.
Yusuke OkudaDepartment of Pediatrics, Kitasato University School of Medicine, Kanagawa, Japan.
Hirotsugu KitayamaDepartment of Nephrology, Shizuoka Children's Hospital, Shizuoka, Japan.
Junya FujimuraDepartment of Pediatrics, Kakogawa Central City Hospital, Hyogo, Japan.
Shingo IshimoriDepartment of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, Japan.
Naohiro KamiyoshiDepartment of Pediatrics, Japanese Red Cross Society Himeji Hospital, Hyogo, Japan.
Norishige YoshikawaClinical Research Center, Takatsuki General Hospital, Osaka, Japan.

Funding

Japan Agency for Medical Research and Development (AMED)
6 · The paper itself

Abstract

backgroundGenetic analysis, crucial in determining treatment strategies for steroid-resistant nephrotic syndrome (SRNS), can be performed in limited facilities and requires a long time. Predicting the presence or absence of genetic variants by clinical and pathologic features is preferable.

methodsIn this multicenter, retrospective study, we compared the clinical or pathologic features between the patients with and without genetic variants in children with SRNS and evaluated the efficacy of immunosuppressive treatment and long-term kidney outcomes.

resultsFifty-three patients in 17 institutes were included, and 11 patients (21%) showed genetic variants. Two patients with a family history of nephrotic syndrome harbored genetic variants. Serum albumin level at onset was significantly lower in patients without genetic variants (p = 0.001). The receiver operating characteristic curve analysis showed that a cutoff value of serum albumin level of 2.3 g/dL at onset had a sensitivity and specificity of 82% and 90%, respectively, in predicting genetic variants. Patients with asymptomatic proteinuria at onset were more likely to harbor genetic variants (p = 0.05). None of the pathologic features was significantly different between the two groups. Mesangial proliferation and diffuse foot process effacement were observed more in patients without genetic variants, although statistically insignificant. Immunosuppressive treatment was less effective, and the 5-year kidney survival was poorer (31% and 78%, p = 0.03) in patients with genetic variants than in those without genetic variants.

conclusionsHigher serum albumin levels at onset can predict the presence of genetic variants. Pathologic features might have limited utility in predicting them.

Indexed as

Nephrotic SyndromeAdolescentChildChild, PreschoolDrug ResistanceFemaleGenetic VariationHumansImmunosuppressive AgentsInfantKidneyMaleRetrospective StudiesSerum AlbuminTreatment OutcomeImmunosuppressive AgentsSerum AlbuminAcute kidney injuryColumbia classificationFoot process effacementGenetic variantSerum albuminSteroid-resistant nephrotic syndrome (SRNS)

Identifiers

PMID40473980

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.