A New Patient with Inherited TYK2 Deficiency
Sign Up to like & getrecommendations! 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
Clinical Manifestation of Hyper IgE Syndrome Including Otitis Media
Sign Up to like & getrecommendations! 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.
Sign Up to like & getrecommendations! 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
A set of clinical and laboratory markers differentiates hyper-IgE syndrome from severe atopic dermatitis.
Sign Up to like & getrecommendations! 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
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
Sign Up to like & getrecommendations! 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.
Sign Up to like & getrecommendations! 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
Sign Up to like & getrecommendations! 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
Sign Up to like & getrecommendations! 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
Clinical Profile of Hyper-IgE Syndrome in India
Sign Up to like & getrecommendations! Published in 2021 at "Frontiers in Immunology"
DOI: 10.3389/fimmu.2021.626593
Abstract: Introduction: Hyper-IgE Syndrome (HIES) is a rare inborn error of immunity (IEI) characterized by a constellation of symptoms related to susceptibility to Staphylococcal skin and pulmonary infections, eczema, raised serum IgE (>2,000 IU/ml), craniofacial anomalies,… read more here.
Keywords: hyper ige; stat3 hies; ige syndrome; exon exon ... See more keywords
Diagnostic challenge in a series of eleven patients with hyper IgE syndromes
Sign Up to like & getrecommendations! Published in 2023 at "Frontiers in Immunology"
DOI: 10.3389/fimmu.2022.1057679
Abstract: Hyper IgE syndromes (HIES) is a heterogeneous group of Inborn Errors of Immunity characterized by eczema, recurrent skin and lung infections associated with eosinophilia and elevated IgE levels. Autosomal dominant HIES caused by loss of… read more here.
Keywords: diagnostic challenge; hyper ige; eleven patients; ige syndromes ... See more keywords
Hyper-IgE and Carcinoma in CADINS Disease
Sign Up to like & getrecommendations! Published in 2022 at "Frontiers in Immunology"
DOI: 10.3389/fimmu.2022.878989
Abstract: Background Atopic dermatitis (AD) affects up to 25% of children and 10% of adults in Western countries. When severe or recurrent infections and exceedingly elevated serum IgE levels occur in AD patients, an inborn error… read more here.
Keywords: carcinoma cadins; cadins disease; hyper ige; ige carcinoma ... See more keywords