Evidence map›Paper›PMID 35033700›Full record

ArticleThe journal of allergy and clinical immunology. In practice2022

Chronic Granulomatous Disease With Inflammatory Bowel Disease: Clinical Presentation, Treatment, and Outcomes From the USIDNET Registry.

Brenna LaBere, Maria J Gutierrez, Hannah Wright, Elizabeth Garabedian, Hans D Ochs, Ramsay L Fuleihan, Elizabeth Secord, Rebecca Marsh, Kathleen E Sullivan, Charlotte Cunningham-Rundles and 2 more

Open access · greenAbstract read
PubMed Full text at PMC Publisher
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 17 papers.

0numbers the graph read from it
0cells of the map it votes in
17citing papers in PubMed
1.7field-weighted citation impact, top 16% 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

17 citing papers in PubMed, 23 citations in OpenAlex.

  1. Article
  2. Article
  3. Article
  4. Article
  5. Article
  6. Review
  7. Review
  8. Article
  9. The Role of NADPH Oxidase 2 in Leukocytes.Antioxidants (Basel, Switzerland) · 2025
    Review
  10. Article
  11. Article
  12. Review
  13. Review
  14. Article
  15. Review
  16. Review
  17. 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

12 authors at 9 institutions in 1 country.

Brenna LaBereDepartment of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah; Division of Immunology, Boston Children's Hospital, Boston, Mass.
Maria J GutierrezDivision of Pediatric Allergy and Immunology, Johns Hopkins University, Baltimore, Md.
Hannah WrightUnited States Immunodeficiency Network, Towson, Md.
Elizabeth GarabedianNational Institutes of Health, National Human Genome Research Institute, Bethesda, Md.
Hans D OchsDivision of Immunology, Department of Pediatrics, University of Washington and Seattle Children's Research Institute, Seattle, Wash.
Ramsay L FuleihanDivision of Allergy, Immunology and Rheumatology, Department of Pediatrics, Columbia University Irving Medical Center, New York-Presbyterian and Morgan Stanley Children's Hospital, New York, NY.
Elizabeth SecordDivision of Allergy and Immunology, Wayne Pediatrics, Wayne State University School of Medicine, Detroit, Mich.
Rebecca MarshDepartment of Pediatrics, University of Cincinnati, and Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Kathleen E SullivanDivision of Allergy and Immunology, Children's Hospital of Philadelphia, Philadelphia, Pa; Department of Pediatrics, Perelman School of Medicine at University of Pennsylvania, Philadelphia, Pa.
Charlotte Cunningham-RundlesDivision of Allergy and Immunology, Department of Medicine, the Icahn School of Medicine at Mount Sinai, New York, NY.
Luigi D NotarangeloLaboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.
Karin ChenDivision of Allergy and Immunology, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah; Division of Immunology, Department of Pediatrics, University of Washington and Seattle Children's Research Institute, Seattle, Wash. Electronic address: Karin.Chen@SeattleChildrens.org.
Columbia University · USIcahn School of Medicine at Mount Sinai · USJohns Hopkins University · USNational Human Genome Research Institute · USNational Institutes of Health · USUnited States Immunodeficiency Network · USUniversity of Cincinnati · USUniversity of Washington · USWayne State University · US

Funding

Characterization of the cellular and molecular bases of inborn errors of immunityZIAAI001222 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI NOTARANGELO, LUIGI · 2017 to 2025
$15.2M
Resources to Assist Investigations in Primary Immunodeficiency Diseases (U24)U24AI086037 · NIAID · IMMUNE DEFICIENCY FOUNDATION · PI CUNNINGHAM-RUNDLES, CHARLOTTE · 2010 to 2019
$6.5M
“Role of Maternal Obesity in Epigenetic and Metabolic Programming and Lower Respiratory Infection Risk in Early Life” K23HD104933 · NICHD · JOHNS HOPKINS UNIVERSITY · PI GUTIERREZ, MARIA JIMENA · 2021 to 2025
$854k
NIAID NIH HHS U24 AI086037NICHD NIH HHS K23 HD104933
6 · The paper itself

Abstract

backgroundChronic granulomatous disease (CGD) is an inborn error of immunity caused by defects in the phagocytic nicotinamide adenine dinucleotide phosphate oxidase complex, leading to increased susceptibility to infection and inflammatory autoimmune diseases. Up to 50% of patients have gastrointestinal (GI) involvement and meet diagnostic criteria for inflammatory bowel disease (CGD-IBD).

objectiveWe analyzed patients with CGD from the US Immunodeficiency Network (USIDNET) registry to determine whether IBD changes the presentation, treatment, and outcomes of patients with CGD.

methodsA retrospective evaluation of CGD cases from the USIDNET registry was completed. CGD-IBD was defined as the presence of any major physician-reported inflammatory, noninfectious GI disease manifestation. Demographic information, conditions, infections, antimicrobial therapies, immunomodulator use, and hematopoietic stem cell transplantation data were analyzed.

resultsOf 194 patients with a diagnosis of CGD, 96 met criteria for IBD and 98 were categorized in the non-IBD group. Patients with CGD-IBD had an increased rate of infection compared with the non-IBD group (0.66 vs 0.36 infections/patient/year). Enteric organism infections were more common in patients with IBD. Immunomodulators were used at a significantly higher percentage in patients with IBD compared with patients without IBD (80% vs 56%, P < .001). Of the entire CGD cohort, 17 patients died (8.8%), with no significant difference between patients with IBD and patients without IBD (P = 1.00).

conclusionInfectious events, enteric organism infections, and use of immunomodulatory drugs were higher in patients with IBD than patients without IBD; however, mortality was not increased. Patients with CGD and concurrent IBD are at increased risk for disease complications, supporting the importance of early recognition, diagnosis, and treatment.

Indexed as

Granulomatous Disease, ChronicHematopoietic Stem Cell TransplantationInflammatory Bowel DiseasesHumansRegistriesRetrospective StudiesChronic granulomatous diseaseCrohn diseaseHematopoietic stem cell transplantImmunomodulatorInfectionInflammatory bowel disease

Identifiers

PMID35033700
PMCPMC9086117
OpenAlexW4206727883

What OpenQuestion holds

Textmetadata
LicenceTDM
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.