Evidence map›Paper›PMID 38634985›Full record

ArticleJournal of clinical immunology2024

Phenotypic and Immunological Characterization of Patients with Activated PI3Kδ Syndrome 1 Presenting with Autoimmunity.

Qifan Li, Wenjie Wang, Qi Wu, Qinhua Zhou, Wenjing Ying, Xiaoying Hui, Bijun Sun, Jia Hou, Feng Qian, Xiaochuan Wang and 1 more

Open access · hybridAbstract read
PubMed Full text at PMC Publisher
In one paragraph

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 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
0.7field-weighted citation impact, top 31% of its field
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

4 citing papers in PubMed, 3 citations in OpenAlex.

  1. Article
  2. T follicular helper cells in primary immune regulatory disorders.The Journal of allergy and clinical immunology · 2025
    Review
  3. Article
  4. 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

11 authors at 2 institutions in 1 country.

Qifan Li *Department of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Wenjie Wang *Department of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Qi WuDepartment of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Qinhua ZhouDepartment of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Wenjing YingDepartment of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Xiaoying HuiDepartment of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Bijun SunDepartment of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Jia HouDepartment of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Feng QianMinistry of Education Key Laboratory of Contemporary Anthropology, Human Phenome Institute, School of Life Sciences, Fudan University, Shanghai, 200438, China.
Xiaochuan WangDepartment of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China. xchwang@shmu.edu.cn.
Jinqiao SunDepartment of Clinical Immunology, National Children Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China. jinqiaosun@fudan.edu.cn.
Children's Hospital of Fudan University · CNFudan University · CN

Funding

National Key Research and Development Program of China 2022YFC2704905Shanghai Municipal Health Commission Talent Program 2022XD024Shanghai Municipal Science and Technology Major Project 2023SHZDZX02
6 · The paper itself

Abstract

purposeAutoimmunity is a significant feature of APDS1 patients. We aimed to explore the pathogenic immune phenotype and possible mechanisms of autoimmunity in APDS1 patients.

methodsThe clinical records and laboratory data of 42 APDS1 patients were reviewed. Immunophenotypes were evaluated by multiparametric flow cytometry. Autoantibodies were detected via antigen microarray analysis.

resultsA total of 42 children with PIK3CD gene mutations were enrolled. Immunological tests revealed increased proportions of effector memory cells (86%) and central memory cells (59%) among CD4+ T cells; increased proportions of effector memory cells (83%) and terminally differentiated effector memory T cells (38%) among CD8+ T cells. Fewer CD3+ T cells and B cells and higher IgG levels were reported in patients with autoimmunity. The proportion of Tregs was decreased, and the proportions of Th9, Tfh, and Tfr cells were increased in APDS1 patients. Among APDS1 patients, higher proportion of Th2 and Tfr cells were found in those with autoimmunity. The proportions of CD11c+ B and CD21lo B cells in patients with autoimmunity were significantly increased. Antigen microarray analysis revealed a wide range of IgG/IgM autoantibodies in patients with APDS1. In patients with autoimmunity, the proportion of Tfr might be positively correlated with autoantibodies.

conclusionsThe pathogenic immune phenotype of APDS1 patients included (1) deceased CD3+ T-cell and B-cell counts and increased IgG levels in patients with autoimmunity, (2) an imbalanced T helper cell subset, (3) increased proportions of autoreactive B cells, and (4) distinct autoantibody reactivities in patients with autoimmunity.

Indexed as

AutoantibodiesAutoimmunityB-LymphocytesChildHumansImmunoglobulin GPhenotypeSyndromeAutoantibodiesImmunoglobulin GActivated PI3Kδ Syndrome 1Autoantigen MicroarrayImmune PhenotypesPIK3CD

Identifiers

PMID38634985
PMCPMC11026262
OpenAlexW4394920533

What OpenQuestion holds

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LicenceCC BY
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Registered trials

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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.