SynthesisClinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association2022
A Systematic Review of Monogenic Inflammatory Bowel Disease.
Synthesis in Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 86 papers, 4 of them syntheses that pooled 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.
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.
Who cites it
86 citing papers in PubMed, 4 syntheses or guidelines pooled it, 135 citations in OpenAlex.
- A therapeutic atlas of monogenic inflammatory bowel disease.Journal of Crohn's & colitis · 2026Pooled it
- Comprehensive evaluation of clinical phenotypes and pathogenic features in late-onset monogenic inflammatory bowel disease: a comparative study with infantile-onset cases.BMC gastroenterology · 2025Pooled it
- Hemophagocytic lymphohistiocytosis in patients with inflammatory bowel diseases: a systematic review.Frontiers in immunology · 2025Pooled it
- Crohn's-like Enteritis in X-Linked Agammaglobulinemia: A Case Series and Systematic Review.The journal of allergy and clinical immunology. In practice · 2021Pooled it
- Clinical characteristics and prognosis of infant onset inflammatory bowel disease in Chinese children: a 10-year single-center experience.Translational pediatrics · 2026Article
- Bioinformatics analysis reveals shared gene signatures and molecular mechanisms between periodontitis and rheumatoid arthritis in the context of aging.Acta odontologica Scandinavica · 2026Article
- Article
- Clinical and genomic features of Iranian patients with very early onset IBD.Pediatric research · 2026Article
- FCGR2A promoter variant reveals shared genetic susceptibility between IBD and stroke.Molecular and cellular biochemistry · 2026Article
- The genetic landscape of congenital diarrheas and very early onset inflammatory bowel disease in the Middle East.JPGN reports · 2026Article
- Translating IBD Genomics into Clinical Practice.Digestive diseases and sciences · 2026Review
- Treat-to-target in Behçet syndrome.Nature reviews. Rheumatology · 2026Review
- Orofacial granulomatosis in pediatric Crohn's disease: clinical outcomes and genetic background in the era of biologics: a retrospective study in Japan.Intestinal research · 2026Article
- Intersection Between Systemic Autoimmune Diseases, Primary Immunodeficiency and Cancer: a Field in its Infancy.Journal of clinical immunology · 2026Review
- BIRC3 (Encoding cIAP2) Variants Result in Dysregulated RIPK1 Signaling Leading to Increased Epithelial Cell Death and Are Associated With Monogenic Crohn's Disease.Gastroenterology · 2026Article
- Clinical Phenotypes and Genetic Findings in Very-Early-Onset Inflammatory Bowel Disease: A Vietnamese Pediatric Cohort Study.Children (Basel, Switzerland) · 2026Article
- Functional changes in the gut microbiota are associated with the intestinal phenotype in A20 haploinsufficiency.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2026Article
- Real-world outcomes of ustekinumab, vedolizumab, and tumor necrosis factor inhibitors in very-early-onset inflammatory bowel disease: a multi-center cohort study.Journal of gastroenterology · 2026Article
- Monogenic forms of inflammatory bowel disease: Genetic mechanisms, models, and clinical implications.Molecular medicine (Cambridge, Mass.) · 2026Review
- Diagnosis of Pediatric Inflammatory Bowel Disease (PIBD)-Implications for the Asia-Pacific Region: A Position Paper from the Asian Pan-Pacific Society for Pediatric Gastroenterology, Hepatology, and Nutrition PIBD Working Group.Pediatric gastroenterology, hepatology & nutrition · 2026Review
26 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
11 authors at 7 institutions in 4 countries.
Funding
Abstract
BACKGROUND &
aimsAdvances in genomic technologies have led to increasing reports of monogenic inflammatory bowel disease (IBD). Here, we systematically review the literature to determine the clinical features, genetic profile, and previously used treatment strategies in monogenic IBD.
methodsA systematic review of MEDLINE articles published between January 2000 and December 2020 was conducted. A total of 750 individual monogenic IBD cases were identified from 303 eligible articles.
resultsThe most frequently reported monogenic IBD genes were IL10RA/B, XIAP, CYBB, LRBA, and TTC7A. In total, 63.4% of patients developed IBD before 6 years of age, 17.4% developed IBD between ages 10 and 17.9 years, and 10.9% developed IBD after age 18. There was a substantial difference between these age groups and the underlying monogenic disorders. Only 31.7% had any history of extraintestinal comorbidity (EIC) before IBD onset, but 76.0% developed at least 1 EIC during their clinical course. The most common EICs were atypical infection (44.7%), dermatologic abnormality (38.4%), and autoimmunity (21.9%). Bowel surgery, biologic therapy, and hematopoietic stem cell transplantation were performed in 27.1%, 32.9%, and 23.1% of patients, respectively.
conclusionsMonogenic IBD cases, although rare, have varied extraintestinal comorbidities and limited treatment options including surgery and transplant. Early identification and improved understanding of the characteristics of the genes and underlying disease processes in monogenic IBD is important for effective management.
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Registered trials
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.