Articles with "hyper ige" as a keyword



A New Patient with Inherited TYK2 Deficiency

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Published in 2019 at "Journal of Clinical Immunology"

DOI: 10.1007/s10875-019-00713-5

Abstract: Complete TYK2 deficiency (IMMUNODEFICIENCY 35 OMIM (611521)) is a rare disorder, inherited as an autosomal recessive (AR) trait, which has been previously described in nine patients from seven unrelated kindreds [1–3]. It was first reported… read more here.

Keywords: complete tyk2; hyper ige; deficiency; tyk2 deficiency ... See more keywords
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Clinical Manifestation of Hyper IgE Syndrome Including Otitis Media

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Published in 2018 at "Current Allergy and Asthma Reports"

DOI: 10.1007/s11882-018-0806-6

Abstract: Purpose of ReviewThe hyper IgE syndromes (HIES) comprise a group of rare primary immunodeficiency disorders (PIDDs), which are characterized by extremely high serum IgE levels, eczema, recurrent skin and pulmonary infections. Both autosomal dominant (AD)… read more here.

Keywords: otitis media; hyper ige; clinical manifestation; clinical manifestations ... See more keywords

Phenotyping and long-term follow up of patients with hyper IgE syndrome.

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Published in 2019 at "Allergologia et immunopathologia"

DOI: 10.1016/j.aller.2018.07.009

Abstract: INTRODUCTION AND OBJECTIVES Long-term follow up of patients with hyper IgE syndrome (HIES), as a primary immunodeficiency disorder, has been poorly investigated. This study describes common clinical and immunological features of patients with HIES in… read more here.

Keywords: hyper ige; term follow; long term; patients hyper ... See more keywords
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A set of clinical and laboratory markers differentiates hyper-IgE syndrome from severe atopic dermatitis.

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Published in 2020 at "Clinical immunology"

DOI: 10.1016/j.clim.2020.108645

Abstract: Hyper-IgE syndrome (HIES) patients may share many features observed in severe atopic dermatitis (SAD), making a diagnostic dilemma for physicians. Determining clinical and laboratory markers that distinguish both disorders could provide early diagnosis and treatment.… read more here.

Keywords: laboratory markers; hyper ige; severe atopic; ige syndrome ... See more keywords

PA03 Should patients with atopic dermatitis be screened for genes associated with hyper-IgE syndrome?

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Published in 2024 at "British Journal of Dermatology"

DOI: 10.1093/bjd/ljae090.258

Abstract: Individuals with severe atopic dermatitis (AD) may present with very high serum IgE levels (> 10 000 IU mL−1). Signal transducer and activator of transcription 3 hyper-IgE syndrome (STAT3-HIES), a rare immunosuppressive disorder, shares pathogenic similarities… read more here.

Keywords: ige levels; hyper ige; stat3 hies; stat3 ... See more keywords

Successful Use of Fosmanogepix for Treatment of Rare Highly Resistant Cutaneous Fusariosis in a Pediatric Patient with STAT3 Hyper-IgE Syndrome and End-Stage Kidney Disease

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Published in 2023 at "Open Forum Infectious Diseases"

DOI: 10.1093/ofid/ofad285

Abstract: We describe the successful use of the novel antifungal drug fosmanogepix to treat a chronic case of multidrug-resistant cutaneous Fusarium suttonianum infection in a pediatric patient with STAT3 hyper-IgE syndrome and end-stage kidney disease on… read more here.

Keywords: patient stat3; hyper ige; stat3 hyper; resistant cutaneous ... See more keywords

Acquired Immune Deficiency Syndrome in Differential Diagnosis of Hyper-IgE-Immunoglobulinemia: Pediatric Case Report.

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Published in 2017 at "Journal of tropical pediatrics"

DOI: 10.1093/tropej/fmw053

Abstract: Acquired immune deficiency syndrome can be encountered with hypereosinophilia and hyperimmunoglobulin E (hyper-IgE) values, though these levels are rarely so high to be compared with hyperimmunoglobulin E syndrome. A 9-year-old boy presented with the complaint… read more here.

Keywords: ige; hyper ige; deficiency syndrome; acquired immune ... See more keywords

Autosomal recessive hyper‐IgE syndrome in two brothers of a Chinese family with a novel mutation in DOCK8 gene

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Published in 2018 at "Journal of the European Academy of Dermatology and Venereology"

DOI: 10.1111/jdv.14847

Abstract: The hyper-IgE syndromes (HIES) are complex primary immunodeficiency disorders, including autosomal dominant form (AD-HIES) and recessive form (AR-HIES). Here we describe two boys with AR-HIES in a Chinese family and a novel mutation of DOCK8… read more here.

Keywords: family novel; hyper ige; chinese family; novel mutation ... See more keywords

Antibiotic use favors early‐life allergies, intrauterine blood flow may influence respiratory allergies, and features of hyper‐IgE syndrome

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Published in 2019 at "Pediatric Allergy and Immunology"

DOI: 10.1111/pai.13062

Abstract: This editorial opens this new issue of Pediatric Allergy and Immunology, the leading journal in the field, aiming at providing you with the latest science and state‐of‐the‐art review articles. Two educational articles begin this issue:… read more here.

Keywords: hyper ige; antibiotic use; respiratory allergies; life ... See more keywords

Rubinstein–Taybi Syndrome With Severe Eczema, Recurrent Infections, and Hyper IgE Profile Responsive to Dupilumab Treatment

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Published in 2025 at "Pediatric Dermatology"

DOI: 10.1111/pde.15874

Abstract: Rubinstein–Taybi syndrome (RTS) is a rare autosomal dominant disorder characterized by post‐natal growth retardation, facial dysmorphism, large thumbs and halluces, and intellectual deficits. RTS has a broad phenotypic spectrum including cardiac, genitourinary, digestive, ear, nose… read more here.

Keywords: rubinstein taybi; hyper ige; taybi syndrome; ige profile ... See more keywords

STAT3 haploinsufficiency is associated with autosomal dominant hyper-IgE syndrome

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Published in 2025 at "Science Advances"

DOI: 10.1126/sciadv.adw2464

Abstract: The autosomal dominant hyper-IgE syndrome (AD-HIES) is a primary immunodeficiency, which originates from heterozygous missense mutations in the signal transducer and activator of transcription 3 (STAT3) gene. It is accepted that most STAT3 variants causing… read more here.

Keywords: hyper ige; ige syndrome; stat3; dominant hyper ... See more keywords