ReviewJournal of research in medical sciences : the official journal of Isfahan University of Medical Sciences2017
Hyperimmunoglobulin E syndrome: Genetics, immunopathogenesis, clinical findings, and treatment modalities.
Review in Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers, 1 of them a synthesis that pooled it.
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Who cites it
30 citing papers in PubMed, 1 synthesis or guideline pooled it, 43 citations in OpenAlex.
- Audiovestibular Dysfunction in Hyper-IgE Syndrome: A Systematic Review of Characteristics, Pathophysiology, Diagnosis, and Management.International journal of molecular sciences · 2025Pooled it
- Autoimmune Hemolytic Anemia in a Child with DOCK8-Deficient Hyper-IgE Syndrome.Indian journal of pediatrics · 2026Article
- Case Report: A case series of using whole exome sequencing to detect novel variants in Vietnamese patients with inborn errors of immunity.Frontiers in genetics · 2026Article
- Diagnosing Hyper IgE Syndrome in a Resource-Limited Setting: A Case Report Highlighting the Utility of Clinical Scoring and Multidisciplinary Management.Case reports in immunology · 2026Article
- Efficacy of omalizumab in the treatment of STAT3 loss-of-function mutations associated with autosomal dominant hyperimmunoglobulin E syndrome-a case report.Translational pediatrics · 2025Article
- Clinical analysis of hyperimmunoglobulin E syndrome in children withTranslational pediatrics · 2025Article
- [Clinical features and immunotherapy for children with loss-of-function/gain-of-function mutations in theZhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2025Article
- Serum IgE and IgA Levels in Pediatric Henoch-Schönlein Purpura: Clinical Characteristics and Immunological Correlations in the Context of Infectious Diseases-A Five-Year Retrospective Analysis.International journal of molecular sciences · 2025Article
- Hyper IgE Syndromes: Understanding, Management, and Future Perspectives: A Narrative Review.Health science reports · 2025Article
- Intracranial infections ofFrontiers in immunology · 2025Review
- STAT3 c.1915C > T variant-associated Hyper-IgE syndrome in a child: a case report.Frontiers in pediatrics · 2025Article
- Neutrophil-avid nanocarrier uptake by STAT3 dominant-negative hyper-IgE syndrome patient neutrophils.Life science alliance · 2024Article
- 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
- Allergic bronchopulmonary aspergillosis with marked peripheral blood eosinophilia and pulmonary eosinophilia: A case report.World journal of clinical cases · 2023Article
- DisseminatedOpen forum infectious diseases · 2023Article
- Antibiotic susceptibility and clonal distribution ofFrontiers in cellular and infection microbiology · 2023Article
- An unusual cause of facial wound in a child: Hyper IgE syndrome-associated Noma neonatorum.International wound journal · 2022Article
- AAV-monoclonal antibody expression protects mice from Ebola virus without impeding the endogenous antibody response to heterologous challenge.Molecular therapy. Methods & clinical development · 2022Article
- An Unusual Pattern of Premature Cervical Spine Degeneration in STAT3-LOF.Journal of clinical immunology · 2021Article
- The other side of the coin: IgE deficiency, a susceptibility factor for malignancy occurrence.The World Allergy Organization journal · 2021Review
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Authors and funding
6 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The hyperimmunoglobulin E syndromes (HIESs) are very rare immunodeficiency syndromes with multisystem involvement, including immune system, skeleton, connective tissue, and dentition. HIES are characterized by the classic triad of high serum levels of immunoglobulin E (IgE), recurrent staphylococcal cold skin abscess, and recurrent pneumonia with pneumatocele formation. Most cases of HIES are sporadic although can be inherited as autosomal dominant and autosomal recessive traits. A fundamental immunologic defect in HIES is not clearly elucidated but abnormal neutrophil chemotaxis due to decreased production or secretion of interferon γ has main role in the immunopathogenesis of syndrome, also distorted Th1/Th2 cytokine profile toward a Th2 bias contributes to the impaired cellular immunity and a specific pattern of infection susceptibility as well as atopic-allergic constitution of syndrome. The ophthalmic manifestations of this disorder include conjunctivitis, keratitis, spontaneous corneal perforation, recurrent giant chalazia, extensive xanthelasma, tumors of the eyelid, strabismus, and bilateral keratoconus. The diagnosis of HIES is inconclusive, dependent on the evolution of a constellation of complex multisystemic symptoms and signs which develop over the years. Until time, no treatment modality is curative for basic defect in HIES, in terms of cytokines/chemokines derangement. Of note, bone marrow transplant and a monoclonal anti-IgE (omalizumab) are hoped to be successful treatment in future.
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