Evidence map›Paper›PMID 31111319›Full record

SynthesisClinical reviews in allergy & immunology2020

Clinical, Immunological, and Genetic Features in Patients with Activated PI3Kδ Syndrome (APDS): a Systematic Review.

Mahnaz Jamee, Shakiba Moniri, Majid Zaki-Dizaji, Peter Olbrich, Reza Yazdani, Farhad Jadidi-Niaragh, Fatemeh Aghamahdi, Hassan Abolhassani, Alison M Condliffe, Asghar Aghamohammadi and 1 more

Abstract readMeta-AnalysisSystematic Review
PubMed Publisher
In one paragraph

Synthesis in Clinical reviews in allergy & immunology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 100 papers, 4 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
100citing papers in PubMed, 4 pooled it
4.3field-weighted citation impact, top 5% 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

100 citing papers in PubMed, 4 syntheses or guidelines pooled it, 147 citations in OpenAlex.

  1. Pooled it
  2. Pooled it
  3. Systematic review of mortality and survival rates for APDS.Clinical and experimental medicine · 2024
    Pooled it
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  10. Review
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  12. Bridging Rare to Common Diseases: Precision Medicine and the Transforming Landscape of Pediatric Allergy and Immunology.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2026
    Review
  13. Article
  14. Review
  15. Review
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  20. Article

40 more citing papers are in PubMed but not listed here.

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 7 institutions in 4 countries.

Mahnaz JameeStudent Research Committee, Alborz University of Medical Sciences, Karaj, Iran.
Shakiba MoniriStudent Research Committee, Alborz University of Medical Sciences, Karaj, Iran.
Majid Zaki-DizajiLegal Medicine Research Center, Legal Medicine Organization, Tehran, Iran.
Peter OlbrichSección de Infectología e Inmunopatología, Unidad de Pediatría, Hospital Virgen del Rocío/Instituto de Biomedicina de Sevilla (IBiS), Seville, Spain.
Reza YazdaniResearch Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Farhad Jadidi-NiaraghImmunology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.
Fatemeh AghamahdiNon-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran.
Hassan AbolhassaniDivision of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute, Karolinska University Hospital Huddinge, Stockholm, Sweden.
Alison M CondliffeDepartment of Infection, Immunity and Cardiovascular Diseases, University of Sheffield, Sheffield, UK.
Asghar AghamohammadiResearch Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Gholamreza AziziNon-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran. azizi@abzums.ac.ir.ORCID http://orcid.org/0000-0001-5658-2511
Jahrom University of Medical Sciences · IRChildren's Medical Center · IRInstituto de Biomedicina de Sevilla · ESIranian Legal Medicine Organization · IRKarolinska University Hospital · SETabriz University of Medical Sciences · IRUniversity of Sheffield · GB

Funding

ABZUMS IR.ABZUMS.REC.1397.051.
6 · The paper itself

Abstract

Activated phosphoinositide 3-kinase delta syndrome (APDS) is a novel primary immunodeficiency (PID) caused by heterozygous gain of function mutations in PI3Kδ catalytic p110δ (PIK3CD) or regulatory p85α (PIK3R1) subunits leading to APDS1 and APDS2, respectively. Patients with APDS present a spectrum of clinical manifestations, particularly recurrent respiratory infections and lymphoproliferation. We searched PubMed, Web of Science, and Scopus databases for APDS patients and screened for eligibility criteria. A total of 243 APDS patients were identified from 55 articles. For all patients, demographic, clinical, immunologic, and molecular data were collected. Overall, 179 APDS1 and 64 APDS2 patients were identified. The most common clinical manifestations were respiratory tract infections (pneumonia (43.6%), otitis media (28.8%), and sinusitis (25.9%)), lymphoproliferation (70.4%), autoimmunity (28%), enteropathy (26.7%), failure to thrive (20.6%), and malignancy (12.8%). The predominant immunologic phenotype was hyper-IgM syndrome (48.1%). Immunologic profiling showed decreased B cells in 74.8% and CD4

Indexed as

Disease SusceptibilityPhenotypeAdolescentAdultAutoimmunityBiomarkersChildClass I Phosphatidylinositol 3-KinasesFemaleGain of Function MutationGenetic Predisposition to DiseaseHumansMalePrimary Immunodeficiency DiseasesYoung AdultBiomarkersClass I Phosphatidylinositol 3-KinasesPIK3CD protein, humanActivated phosphoinositide 3-kinase δ syndromeAPDSHyper-IgM syndromePhosphoinositide-3-kinase δ

Identifiers

PMID31111319
OpenAlexW2946662503

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

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.