Evidence map›Paper›PMID 38231436›Full record

ArticleJournal of clinical immunology2024

Clinical and Treatment History of Patients with Partial DiGeorge Syndrome and Autoimmune Cytopenia at Multiple Centers.

Priya K Patel, Michell Lozano Chinga, Melis Yilmaz, Sonia Joychan, Boglarka Ujhazi, Maryssa Ellison, Sumai Gordon, Daime Nieves, Krisztian Csomos, Don Eslin and 14 more

Erratum issuedAbstract read
PubMed 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. An erratum has been issued. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
1.1field-weighted citation impact, top 25% 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

3 citing papers in PubMed, 2 citations in OpenAlex.

  1. Review
  2. Article
  3. Review
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

24 authors at 8 institutions in 4 countries.

Priya K PatelJohns Hopkins All Children's Hospital, 601 5Th Street South, Outpatient Care Clinic, 3Rd Floor, St. Petersburg, FL, 33701, USA. patelp15@usf.edu.ORCID 0000-0001-5400-3782
Michell Lozano ChingaDivision of Pediatric Hematology Oncology, University of Utah Primary Children's Hospital, Salt Lake City, UT, USA.
Melis YilmazDepartment of Pediatrics, Division of Allergy and Immunology, University of South Florida Morsani College of Medicine, St Petersburg, FL, USA.
Sonia JoychanDepartment of Pediatrics, Division of Allergy and Immunology, University of South Florida Morsani College of Medicine, St Petersburg, FL, USA.
Boglarka UjhaziDepartment of Pediatrics, Division of Allergy and Immunology, University of South Florida Morsani College of Medicine, St Petersburg, FL, USA.
Maryssa EllisonDepartment of Pediatrics, Division of Allergy and Immunology, University of South Florida Morsani College of Medicine, St Petersburg, FL, USA.
Sumai GordonDepartment of Pediatrics, Division of Allergy and Immunology, University of South Florida Morsani College of Medicine, St Petersburg, FL, USA.
Daime NievesJohns Hopkins All Children's Hospital, 601 5Th Street South, Outpatient Care Clinic, 3Rd Floor, St. Petersburg, FL, 33701, USA.
Krisztian CsomosDepartment of Pediatrics, Division of Allergy and Immunology, University of South Florida Morsani College of Medicine, St Petersburg, FL, USA.
Don EslinBayCare Medical Group, St. Joseph's Hospital, Pediatric Hematology Oncology, St. Petersburg, FL, USA.
Zeinab A AfifyDivision of Pediatric Hematology Oncology, University of Utah Primary Children's Hospital, Salt Lake City, UT, USA.
Jessica MeznarichDivision of Pediatric Hematology Oncology, University of Utah Primary Children's Hospital, Salt Lake City, UT, USA.
John BohnsackDivision of Pediatric Hematology Oncology, University of Utah Primary Children's Hospital, Salt Lake City, UT, USA.
Kelly WalkovichBayCare Medical Group, St. Joseph's Hospital, Pediatric Hematology Oncology, St. Petersburg, FL, USA.
Markus G SeidelClinical Department of Pediatric Hematology Oncology, Medical University of Graz, Graz, Austria.
Svetlana SharapovaBelarusian Research Center for Pediatric Oncology, Hematology, and Immunology, Minsk, Belarus.
Oksana BoyarchykImmunopathology Department, National Research Center Institute of Immunology FMBA, Moscow, Russia.
Elena LatyshevaBelarusian Research Center for Pediatric Oncology, Hematology, and Immunology, Minsk, Belarus.
Irina TuzankinaBelarusian Research Center for Pediatric Oncology, Hematology, and Immunology, Minsk, Belarus.
Ahmad B ShakerBayCare Medical Group, St. Anthony's Hospital, St. Petersburg, FL, USA.
Irmel AyalaJohns Hopkins All Children's Hospital, 601 5Th Street South, Outpatient Care Clinic, 3Rd Floor, St. Petersburg, FL, 33701, USA.
Panida SriaroonJohns Hopkins All Children's Hospital, 601 5Th Street South, Outpatient Care Clinic, 3Rd Floor, St. Petersburg, FL, 33701, USA.
Emma Westermann-ClarkDepartment of Pediatrics, Division of Allergy and Immunology, University of South Florida Morsani College of Medicine, St Petersburg, FL, USA.
Jolan E WalterJohns Hopkins All Children's Hospital, 601 5Th Street South, Outpatient Care Clinic, 3Rd Floor, St. Petersburg, FL, 33701, USA.
University of South Florida St. Petersburg · USJohns Hopkins University · USPrimary Children's Hospital · USBelarusian Research Center For Pediatric Oncology and Hematology · BYSt. Joseph's Hospital · USInstitute of Immunology and Physiology · RUMedical University of Graz · ATSt. Anthony’s Hospital · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundPatients with partial DiGeorge syndrome (pDGS) can present with immune dysregulation, the most common being autoimmune cytopenia (AIC). There is a lack of consensus on the approach to type, combination, and timing of therapies for AIC in pDGS. Recognition of immune dysregulation early in pDGS clinical course may help individualize treatment and prevent adverse outcomes from chronic immune dysregulation.

objectivesObjectives of this study were to characterize the natural history, immune phenotype, and biomarkers in pDGS with AIC.

methodsData on clinical presentation, disease severity, immunological phenotype, treatment selection, and response for patients with pDGS with AIC were collected via retrospective chart review. Flow cytometric analysis was done to assess T and B cell subsets, including biomarkers of immune dysregulation.

resultsTwenty-nine patients with the diagnosis of pDGS and AIC were identified from 5 international institutions. Nineteen (62%) patients developed Evan's syndrome (ES) during their clinical course and twenty (69%) had antibody deficiency syndrome. These patients demonstrated expansion in T follicular helper cells, CD19

conclusionsAIC in pDGS is often refractory to conventional AIC treatment paradigms. Biomarkers may have utility for correlation with disease state and potentially even response to therapy. Immunomodulating therapies could be initiated early based on early immune phenotyping and biomarkers before the disease develops or significantly worsens.

Indexed as

CytopeniaDiGeorge SyndromeAntigens, CD19Disease ProgressionHumansRetrospective StudiesAntigens, CD19Autoimmune cytopeniaImmune dysregulationPartial DiGeorge syndrome

Identifiers

PMID38231436
OpenAlexW4390949819

What OpenQuestion holds

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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.