ArticleJournal of clinical immunology2017
Gastrointestinal Manifestations of STAT3-Deficient Hyper-IgE Syndrome.
Article in Journal of clinical immunology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 1 of them a synthesis that pooled it.
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Who cites it
39 citing papers in PubMed, 1 synthesis or guideline pooled it, 72 citations in OpenAlex.
- Italian pediatric experts' consensus statement on diagnosis and management of primary atopic disorders.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2025Guideline
- Clinical features, genetics, treatment, and long-term outcomes of STAT3 hyper-IgE syndrome: Single-center cohort analysis.The Journal of allergy and clinical immunology · 2026Observational
- Eosinophilic Esophagitis: Mechanisms of Disease and Approach to Treatment.Current allergy and asthma reports · 2026Review
- STAT3-Mutated Hyper-IgE Syndrome With Retroperitoneal Abscess in Adolescence.Clinical case reports · 2026Article
- Back With Vengeance: Delayed Gastrointestinal Sequelae of Histoplasmosis in a Patient With STAT3 Hyper-IgE Syndrome.ACG case reports journal · 2025Article
- Pulmonary features and stage of disease in adult patients with hyper-IgE syndrome: a single-centre clinical study and literature review.Orphanet journal of rare diseases · 2025Article
- Hyper-IgE Syndrome: A Case Report with Insights from Bioinformatics Analysis of Key Pathways and Genes.Infection and drug resistance · 2025Article
- Elevated Immunoglobulin E Serum Levels: Possible Underlying Factors That Can Cause an Inborn Error of Immunity in the Pediatric Population with Recurrent Infections.Antibodies (Basel, Switzerland) · 2024Article
- Review article: Emerging insights into the epidemiology, pathophysiology, diagnostic and therapeutic aspects of eosinophilic oesophagitis and other eosinophilic gastrointestinal diseases.Alimentary pharmacology & therapeutics · 2024Review
- Prediction and analysis of genetic effect in idiopathic pulmonary fibrosis and gastroesophageal reflux disease.IET systems biology · 2023Article
- ERBIN and phosphoglucomutase 3 deficiency.Current opinion in immunology · 2023Review
- A Practical Update on Pediatric Eosinophilic Esophagitis.Children (Basel, Switzerland) · 2023Review
- Inherited human ZNF341 deficiency.Current opinion in immunology · 2023Review
- Food Allergy Characteristics Associated With Coexisting Eosinophilic Esophagitis in FARE Registry Participants.The journal of allergy and clinical immunology. In practice · 2023Article
- Eosinophilic Gastrointestinal Diseases in Inborn Errors of Immunity.Journal of clinical medicine · 2023Review
- Breaking down the complex pathophysiology of eosinophilic esophagitis.Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology · 2023Review
- Atopic Dermatitis-like Genodermatosis: Disease Diagnosis and Management.Diagnostics (Basel, Switzerland) · 2022Review
- Eosinophilic esophagitis an update in children.Acta bio-medica : Atenei Parmensis · 2022Article
- Case Report: Eosinophilic Esophagitis in a Patient With a Novel STAT1 Gain-of-Function Pathogenic Variant.Frontiers in immunology · 2022Article
- Eosinophilic gastrointestinal disorders in patients with inborn errors of immunity: Data from the USIDNET registry.Frontiers in immunology · 2022Article
Corrections and comments
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Authors and funding
15 authors at 5 institutions in 1 country.
Funding
Abstract
objectiveSTAT 3 deficiency (autosomal dominant hyper immunoglobulin E syndrome (AD-HIES)) is a primary immunodeficiency disorder with multi-organ involvement caused by dominant negative signal transducer and activator of transcription gene 3 (STAT3) mutations. We sought to describe the gastrointestinal (GI) manifestations of this disease.
methodsSeventy subjects aged five to 60 years with a molecular diagnosis of AD-HIES were evaluated at the National Institutes of Health (NIH). Data collection involved a GI symptom questionnaire and retrospective chart review.
resultsIn our cohort of 70 subjects, we found that 60% had GI symptoms (42/70). The most common manifestations were gastroesophageal reflux disease (GERD) observed in 41%, dysphagia in 31%, and abdominal pain in 24%. The most serious complications were food impaction in 13% and colonic perforation in 6%. Diffuse esophageal wall thickening in 74%, solid stool in the right colon in 50% (12/24), and hiatal hernia in 26% were the most prevalent radiologic findings. Esophagogastroduodenoscopy (EGD) demonstrated esophageal tortuosity in 35% (8/23), esophageal ulceration in 17% (4/23), esophageal strictures requiring dilation in 9% (2/23), and gastric ulceration in 17% (4/23). Esophageal eosinophilic infiltration was an unexpected histologic finding seen in 65% (11/17).
conclusionThe majority of AD-HIES subjects develop GI manifestations as part of their disease. Most notable are the symptoms and radiologic findings of GI dysmotility, as well as significant eosinophilic infiltration, concerning for a secondary eosinophilic esophagitis. These findings suggest that the STAT3 pathway may be implicated in a new mechanism for the pathogenesis of several GI disorders.
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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.