ReviewCurrent opinion in pediatrics2011
Hyperimmunoglobulin E syndromes in pediatrics.
Review in Current opinion in pediatrics, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
What it found
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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
12 citing papers in PubMed, 42 citations in OpenAlex.
- Multi-Omics Profiling in PGM3 and STAT3 Deficiencies: A Tale of Two Patients.International journal of molecular sciences · 2023Article
- Hyper IgE Syndrome in an Isolated Population in Israel.Frontiers in immunology · 2022Article
- Update on Atopic Dermatitis: Diagnosis, Severity Assessment, and Treatment Selection.The journal of allergy and clinical immunology. In practice · 2020Review
- Metabolomics Distinguishes DOCK8 Deficiency from Atopic Dermatitis: Towards a Biomarker Discovery.Metabolites · 2019Article
- Human hyper-IgE syndrome: singular or plural?Mammalian genome : official journal of the International Mammalian Genome Society · 2018Review
- Progressive multifocal leukoencephalopathy in a patient with lymphoma and presumptive hyper IgE syndrome.Journal of neurovirology · 2017Article
- New genetic discoveries and primary immune deficiencies.Clinical reviews in allergy & immunology · 2014Review
- Recurrent Skin and Lung Infections in Autosomal Dominant Hyper IgE Syndrome with Transactivation Domain STAT3 Mutation.Case reports in immunology · 2014Article
- Neurobehavioral profiles in individuals with hyperimmunoglobulin E Syndrome (HIES) and brain white matter hyperintensities.Journal of clinical immunology · 2013Article
- The involvement of the JAK-STAT signaling pathway in chronic inflammatory skin disease atopic dermatitis.JAK-STAT · 2013Review
- Defective actin accumulation impairs human natural killer cell function in patients with dedicator of cytokinesis 8 deficiency.The Journal of allergy and clinical immunology · 2013Article
- Additional diverse findings expand the clinical presentation of DOCK8 deficiency.Journal of clinical immunology · 2012Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 2 institutions in 1 country.
Funding
Abstract
purpose of reviewThe hyper-IgE syndromes (HIES) are primary immunodeficiencies characterized by eczema, sinopulmonary infections, and elevated serum IgE. This review discusses the clinical similarities and differences between the autosomal dominant HIES (AD-HIES) and autosomal recessive HIES (AR-HIES) forms, as well as their causative genetic and pathophysiological mechanisms. RECENT
findingsOver the past 4 years, three genetic defects have been identified in HIES. Mutations in STAT3 are associated with AD-HIES, whereas mutations in DOCK8, or rarely TYK2, are associated with AR-HIES. Recent work has confirmed that measuring T helper 17 cell numbers can help predict STAT3 mutations. In AR-HIES, loss of DOCK8 expression was found to impair T cell expansion and durable-specific antibody production by B cells. These factors probably contribute to the viral skin and other infectious susceptibilities, severe allergies, and high risk of malignancies that define this disorder. SUMMARY: Establishing the molecular diagnosis of HIES is important for optimal patient management. Infections in AD-HIES are usually well controlled by antibiotics. By contrast, the viral infections in AR-HIES are difficult to manage. Their higher mortality and progressive course emphasizes the need to identify AR-HIES patients early, for consideration of potentially curative hematopoietic cell transplantation.
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Identifiers
What OpenQuestion holds
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.