Evidence map›Paper›PMID 40971131›Full record

ArticleClinical and experimental nephrology2026

Investigation of clinical and genetic characteristics of Alport syndrome using a national registry in Japan (JP-ALPS).

Yusuke Okuda, Naoaki Mikami, Riku Hamada, Hiroshi Hataya, Kazuki Tanaka, Chikako Terano, Naoya Fujita, Kenichiro Miura, Kiyonobu Ishizuka, Yoko Shirai and 18 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Clinical and experimental nephrology, 2026. 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. Article
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

28 authors.

Yusuke OkudaDepartment of Pediatrics, Kitasato University School of Medicine, 1-15-1 Kitazato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan. okuda@kitasato-u.ac.jp.ORCID http://orcid.org/0000-0003-3707-695X
Naoaki MikamiDepartment of Nephrology and Rheumatology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Riku HamadaDepartment of Nephrology and Rheumatology, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Hiroshi HatayaDepartment of General Pediatrics, Tokyo Metropolitan Children's Medical Center, Tokyo, Japan.
Kazuki TanakaDepartment of Pediatric Nephrology, Aichi Children's Health and Medical Center, Aichi, Japan.
Chikako TeranoDepartment of Pediatric Nephrology, Aichi Children's Health and Medical Center, Aichi, Japan.
Naoya FujitaDepartment of Pediatric Nephrology, Aichi Children's Health and Medical Center, Aichi, Japan.
Kenichiro MiuraDepartment of Pediatric Nephrology, Tokyo Women's Medical University, Tokyo, Japan.
Kiyonobu IshizukaDepartment of Pediatric Nephrology, Tokyo Women's Medical University, Tokyo, Japan.
Yoko ShiraiDepartment of Pediatric Nephrology, Tokyo Women's Medical University, Tokyo, Japan.
Koichi KameiDivision of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.
Masao OguraDivision of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.
Takayuki OkamotoDepartment of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Ryota SuzukiDepartment of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Shunsuke ShinozukaDepartment of Pediatrics, Matsudo City General Hospital, Matsudo, Japan.
Yuko ShimaDepartment of Pediatrics, Wakayama Medical University, Wakayama, Japan.
Masafumi OkaDepartment of Pediatrics, Faculty of Medicine, Saga University, Saga, Japan.
Wataru ShimabukuroDepartment of Child Health and Welfare (Pediatrics), Graduate School of Medicine, University of the Ryukyus, Ginowan, Japan.
Hiroyasu TsukaguchiSecond Department of Internal Medicine, Clinical Genetics Center, Kansai Medical University, Hirakata, Japan.
Tetsuji InagakiDepartment of Nephrology, Miyagi Children's Hospital, Sendai, Japan.
Kei NishiyamaDepartments of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Taeko HashimotoDepartment of Pediatric Nephrology, Tokyo Women's Medical University, Tokyo, Japan.
Naoko ItoDepartment of Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.
Tomohiko YamamuraDepartment of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.
Tomoko HorinouchiDepartment of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.
Kenji IshikuraDepartment of Pediatrics, Kitasato University School of Medicine, 1-15-1 Kitazato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan.
Koichi NakanishiDepartment of Child Health and Welfare (Pediatrics), Graduate School of Medicine, University of the Ryukyus, Ginowan, Japan.
Kandai NozuDepartment of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundComprehensive epidemiological information regarding Alport syndrome, particularly from national cohorts, is limited.

methodsUtilizing a national Alport syndrome cohort in Japan established in October 2022, we analyzed clinical characteristics according to genotype. Only baseline data collected retrospectively at enrollment were used. We present longitudinal trends in estimated glomerular filtration rate (eGFR) and urine protein-to-creatinine ratio.

resultsOf the 121 patients included, 105 (86.8%) underwent genetic testing and 82 (67.8%) had a kidney biopsy. Among those with genetic testing, 77 (73.3%) had X-linked Alport syndrome. Kidney function was normal at disease onset, with a median eGFR of 112.9 (interquartile range, 99.3-131.1) mL/min/1.73 m

conclusionsIn our cohort, which consisted mainly of patients who did not require kidney replacement therapy in childhood and adolescence, kidney function was preserved throughout this period except for some male patients with X-linked Alport syndrome. RAS inhibitor use may be associated with a reduced rate of eGFR decline.

Indexed as

Nephritis, HereditaryAdolescentAdultChildChild, PreschoolCollagen Type IVCreatinineDisease ProgressionFemaleGenetic TestingGlomerular Filtration RateHumansJapanKidneyKidney Failure, ChronicMaleCollagen Type IVCreatinineAlport syndromeeGFR slopeRegistryRenin-angiotensin system inhibitors

Identifiers

PMID40971131
PMCPMC12811307

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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.