Evidence map›Paper›PMID 35172220›Full record

ArticleThe journal of allergy and clinical immunology. In practice2022

Morbidity, Mortality, and Therapeutics in Combined Immunodeficiency: Data From the USIDNET Registry.

Jessica Durkee-Shock, Anqing Zhang, Hua Liang, Hannah Wright, Julieann Magnusson, Elizabeth Garabedian, Rebecca A Marsh, Kathleen E Sullivan, Michael D Keller, USIDNET Consortium

Abstract read
In one paragraph

Article in The journal of allergy and clinical immunology. In practice, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.

0numbers the graph read from it
0cells of the map it votes in
10citing papers in PubMed
0.8field-weighted citation impact, top 33% 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

10 citing papers in PubMed, 10 citations in OpenAlex.

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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

10 authors at 3 institutions in 1 country.

Jessica Durkee-ShockNational Institute of Allergy and Infectious Diseases, Bethesda, Md; Children's National Medical Center, Washington, DC.
Anqing ZhangGeorge Washington University Department of Biostatistics, Washington, DC.
Hua LiangGeorge Washington University Department of Biostatistics, Washington, DC.
Hannah WrightUSIDNET, Towson, Md.
Julieann MagnussonUSIDNET, Towson, Md.
Elizabeth GarabedianNational Human Genome Research Institute, Bethesda, Md.
Rebecca A MarshDivision of Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Kathleen E SullivanDivision of Allergy and Immunology, Children's Hospital of Philadelphia, Philadelphia, Pa.
Michael D KellerDivision of Allergy and Immunology, Children's National Hospital, Washington, DC; Department of Pediatrics, George Washington University, Washington, DC. Electronic address: mkeller@childrensnational.org.
USIDNET Consortium
Children's Hospital of Philadelphia · USCincinnati Children's Hospital Medical Center · USNational Human Genome Research Institute · US

Funding

USIDNET: A resource for clinical immunologistsR24AI171055 · NIAID · CHILDREN'S HOSP OF PHILADELPHIA · PI KATHLEEN E SULLIVAN · 2022 to 2026
$7.0M
Resources to Assist Investigations in Primary Immunodeficiency Diseases (U24)U24AI086037 · NIAID · IMMUNE DEFICIENCY FOUNDATION · PI CUNNINGHAM-RUNDLES, CHARLOTTE · 2010 to 2019
$6.5M
NIAID NIH HHS R24 AI171055NIAID NIH HHS U24 AI086037
6 · The paper itself

Abstract

backgroundOptimal management of patients with combined immunodeficiency, especially pertaining to hematopoietic stem cell transplantation (HSCT), remains unclear.

objectiveTo identify factors influencing HSCT and mortality in the population with combined immunodeficiency in North America.

methodsWe identified 337 participants in the United States Immunodeficiency Network database with diverse forms of combined immunodeficiency and their characteristics, including demographic characteristics, laboratory values, infectious history, comorbidities, and treatment strategies. Univariate analysis was performed using logistic regression, whereas multivariate analysis was performed using multiple Cox proportional hazards.

resultsOn univariate analysis, disseminated invasive viral infections and variants in STAT3, GATA2, and, DOCK8 were associated with increased odds of HSCT. Mucocutaneous fungal infections and variants in STAT3 were associated with increased odds of survival, whereas disseminated/invasive fungal infections, disseminated/invasive viral infections, and parasitic infections were associated with decreased odds of survival. On multiple variable Cox proportional hazards analysis, variants in ZAP70, nonspecific bacterial, and disseminated/invasive viral infections were associated with increased hazards of transplantation, whereas variants in multiple genes (RMRP, NEMO, DOCK8, CD40L, and CARD9), disseminated/invasive viral infections, autoimmune disease, and higher absolute lymphocyte count were associated with increased hazards of death. Importantly, demographic characteristics, basic lymphocyte subset counts, and absence of genetic diagnosis were not associated with HSCT or mortality.

conclusionsWe determined that specific genetic diagnoses and infection burden impacts the decision to undergo HSCT in this cohort. In addition, certain genetic diagnoses and invasive viral infections carry an increased risk of mortality.

Indexed as

Hematopoietic Stem Cell TransplantationPrimary Immunodeficiency DiseasesGuanine Nucleotide Exchange FactorsHumansLymphocyte CountMorbidityRegistriesDOCK8 protein, humanGuanine Nucleotide Exchange FactorsCombined immunodeficiencyHematopoietic stem cell transplantationMortalityUSIDNET

Identifiers

PMID35172220
PMCPMC13586977
OpenAlexW4212824175

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.