Evidence map›Paper›PMID 39052129›Full record

ArticleJournal of clinical immunology2024

Discordant Phenotypes of Nephritis in Patients with X-linked Agammaglobulinemia.

Toru Kanamori, Tomohiro Udagawa, Takayuki Fujii, Hiroyoshi Matsukura, Yuka Iwaya, Motoshi Sonoda, Keisuke Sugimoto, Masahiro Takeguchi, Atsunori Yoshino, I-Feng Wang and 6 more

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Article in Journal of clinical immunology, 2024. 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. Revisiting X-linked agammaglobulinemia.Journal of human immunity · 2025
    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

16 authors.

Toru KanamoriDepartment of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-3-45 Yushima, Bunkyo-ku, 113-8519, Tokyo, Japan.
Tomohiro UdagawaDepartment of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-3-45 Yushima, Bunkyo-ku, 113-8519, Tokyo, Japan. tudaped@tmd.ac.jp.
Takayuki FujiiDepartment of Nephrology, Seirei Sakura Citizen Hospital, Sakura, Japan.
Hiroyoshi MatsukuraDepartment of Pediatrics, Saiseikai Takaoka Hospital, Takaoka, Japan.
Yuka IwayaDepartment of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Motoshi SonodaDepartment of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Keisuke SugimotoDepartment of Pediatrics, Kinki University Faculty of Medicine, Osaka-Sayama, Japan.
Masahiro TakeguchiDepartment of Pediatrics, Oita University Faculty of Medicine, Oita, Japan.
Atsunori YoshinoDepartment of Pediatrics, Dokkyo Medical University Saitama Medical Center, Saitama, Japan.
I-Feng WangDivision of Nephrology, National Institute of Cancer Research, National Health Research Institutes, Kaohsiung Medical University, Kaohsiung, Taiwan.
Daw-Yang HwangDivision of Nephrology, National Institute of Cancer Research, National Health Research Institutes, Kaohsiung Medical University, Kaohsiung, Taiwan.
Harry W SchroederDivision of Clinical Immunology and Rheumatology, University of Alabama at Birmingham, Birmingham, AL, USA.
Masaki ShimizuDepartment of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-3-45 Yushima, Bunkyo-ku, 113-8519, Tokyo, Japan.
Hans D OchsDepartment of Pediatrics, University of Washington, Seattle Children's Research Institute, Seattle, WA, USA.
Tomohiro MorioDepartment of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-3-45 Yushima, Bunkyo-ku, 113-8519, Tokyo, Japan.
Hirokazu KaneganeDepartment of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-3-45 Yushima, Bunkyo-ku, 113-8519, Tokyo, Japan. hkanegane.ped@tmd.ac.jp.

Funding

MEXT/JSPS KAKENHI 22K07887
6 · The paper itself

Abstract

purposeTo define the clinical and histological characteristics of nephritis in patients with X-linked agammaglobulinemia (XLA) and their immunological profiles.

methodsThe clinical, immunological, and histological findings of nine patients with XLA and nephritis were retrospectively analyzed.

resultsBased on kidney histological findings, patients with XLA and nephritis could be divided into two groups, viz., chronic glomerulonephritis (CGN) and tubulointerstitial nephritis (TIN). The two groups showed different immunological profiles. Patients in the CGN group exhibited an atypical immunological profile of XLA, with pathogenic leaky B cells producing immunoglobulins that may play a role in forming immune complexes and causing immune-mediated glomerulonephritis. In contrast, patients in the TIN group exhibited a typical immunological profile of XLA, suggesting that antibody-independent/other BTK-dependent mechanisms, or immunoglobulin replacement therapy (IgRT)-related immune/nonimmune-mediated nephrotoxicity causes TIN.

conclusionNephritis occurring in patients with XLA could have links between their renal pathology and immunological status. Careful observation is recommended to detect kidney pathology in patients with XLA on IgRT.

Indexed as

AgammaglobulinemiaGenetic Diseases, X-LinkedPhenotypeAdolescentAdultAgammaglobulinaemia Tyrosine KinaseB-LymphocytesChildChild, PreschoolFemaleGlomerulonephritisHumansKidneyMaleNephritisNephritis, InterstitialAgammaglobulinaemia Tyrosine KinaseChronic glomerulonephritisImmunoglobulin replacement therapyLeaky B cellsTubulointerstitial nephritisX-linked agammaglobulinemia

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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.