Evidence map›Paper›PMID 40095038›Full record

ArticlePediatric nephrology (Berlin, Germany)2025

Differences in kidney prognosis between congenital and infantile nephrotic syndrome.

Yuta Inoki, Tomoko Horinouchi, Shuhei Aoyama, Yuka Kimura, Yuta Ichikawa, Yu Tanaka, Chika Ueda, Hideaki Kitakado, Atsushi Kondo, Nana Sakakibara and 12 more

Abstract readComparative Study
PubMed Full text at PMC 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.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–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. Review
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

22 authors.

Yuta InokiDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Tomoko HorinouchiDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan. tohori@med.kobe-u.ac.jp.ORCID 0000-0003-1655-6030
Shuhei AoyamaDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Yuka KimuraDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Yuta IchikawaDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Yu TanakaDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Chika UedaDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Hideaki KitakadoDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Atsushi KondoDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Nana SakakibaraDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Koichi KameiDivision of Nephrology and Rheumatology, National Center for Child Health and Development, Setagaya-Ku, Tokyo, Japan.
Riku HamadaDepartment of Nephrology and Rheumatology, Tokyo Metropolitan Children's Medical Center, Fuchu, Tokyo, Japan.
Naoya FujitaDepartment of Pediatric Nephrology, Aichi Children's Health and Medical Center, Obu, Aichi, Japan.
Yoshimitsu GotohDepartment of Pediatric Nephrology, Japanese Red Cross Aichi Medical Center Nagoya Daini Hospital, Nagoya, Aichi, Japan.
Yoshitsugu KakuDepartment of Nephrology, Fukuoka Children's Hospital, Fukuoka, Japan.
Kei NishiyamaDepartment of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Takayuki OkamotoDepartment of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Hokkaido, Japan.
Yukiko ToyaDepartment of Pediatrics, Iwate Medical University, Morioka, Iwate, Japan.
Tomohiko YamamuraDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Shingo IshimoriDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
China NaganoDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.
Kandai NozuDepartment of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo, Kobe, Hyogo, 650-0017, Japan.

Funding

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

Abstract

backgroundMore than half of patients with congenital nephrotic syndrome (CNS) or infantile nephrotic syndrome (infantile NS) have a monogenic aetiology. This study aimed to clarify differences in the clinical course, genetic background, and genotype-phenotype correlation between CNS and infantile NS.

methodsWe enrolled patients who were diagnosed with CNS or infantile NS and referred to our hospital for genetic analysis and investigated the clinical characteristics and genetic background of patients with identified causative genes.

resultsAmong 74 patients enrolled, disease-causing genetic variants were detected in 50 patients. The median age for developing kidney failure in the genetic CNS (n = 33) and genetic infantile NS (n = 17) groups with monogenic variants was 13.2 and 19.0 months, respectively (P = 0.13). The age at developing kidney failure was significantly earlier in CNS patients with genes other than NPHS1 than in CNS patients with NPHS1 variants (1.0 vs. 31.0 months; P < 0.001). In patients with pathogenic variants other than NPHS1, there was a significant difference in the age at developing kidney failure between CNS and infantile NS patients (1.0 vs. 15.0 months; P < 0.001). Of patients with NPHS1 variants, no infants with NS had any truncating variants or developed kidney failure during follow-up.

conclusionsThe onset of CNS or infantile NS affects the kidney prognosis in patients with genetic nephrotic syndrome. Among patients with pathogenic variants in the same gene, patients with infantile NS may have a milder genotype and better prognosis than those with CNS.

Indexed as

Nephrotic SyndromeRenal InsufficiencyChild, PreschoolFemaleGenetic Association StudiesHumansInfantInfant, NewbornKidneyMaleMembrane ProteinsMutationPhenotypePrognosisRetrospective StudiesMembrane ProteinsnephrinCongenital nephrotic syndromeGenotype–phenotype correlationInfantile nephrotic syndromeKidney prognosisPathogenic variants

Identifiers

PMID40095038
PMCPMC12187875

What Socratic holds

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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 Socratic graph.