Hyper IgE syndrome | Immune Deficiency Foundation
网页Hyper IgE syndromes (HIES) are rare forms of primary immunodeficiencies (PI) characterized by recurrent eczema, skin abscesses, lung infections, eosinophilia (high numbers of eosinophils in the blood), and high serum levels of immunoglobulin E (IgE).
可治性罕见病—高 IgE综合征 - 知乎 - 知乎专栏
网页高IgE综合征 (hyper IgE syndrome，HIES)又称Job综合征，由Davis等于1966年首次报道 [1]。. HIES是一组罕见的原发性免疫缺陷病，临床主要表现为反复发作的顽固性湿疹、反复细菌感染所致的皮肤及肺脓肿，伴有血清IgE异常增高。. 该综合征为常染色显性或隐性遗传 …
Job Syndrome - StatPearls - NCBI Bookshelf
网页2023年8月28日 · Job Syndrome (Hyper-IgE syndrome) is a rare, primary immunodeficiency distinguished by the clinical triad of atopic dermatitis, recurrent skin staphylococcal infections, and recurrent pulmonary infections. The disease is characterized by elevated IgE levels with an early onset in primary childhood.
Hyper IgE Syndrome (Job's Syndrome) | Symptoms & Treatment
网页What is Hyper IgE Syndrome (Job’s Syndrome)? Hyper IgE syndrome is an immune deficiency caused by a genetic mutation in either STAT3 (autosomal dominant, also known as Job’s syndrome) or DOCK8 (autosomal recessive). There may be other mutations in other genes that are not known at this time.
Hyper-IgE Syndrome - Hyper-IgE Syndrome - MSD Manual Professional Edition
网页Hyper-IgE syndrome is a hereditary combined B- and T-cell immunodeficiency characterized by recurrent staphylococcal abscesses of the skin, sinopulmonary infections, and severe pruritic eosinophilic dermatitis. Diagnosis is confirmed by measurement of serum IgE levels.
高IgE综合征 - 高IgE综合征 - 《默沙东诊疗手册大众版》
网页高IgE综合征是一种遗传性免疫缺陷病症，特征为婴儿期出现复发性疔疮、鼻窦感染和肺部感染及重度皮疹。. 免疫球蛋白E（IgE）水平极高。. 多数高IgE综合征患儿有皮肤、关节、肺或其它器官脓肿。. 血液检查可以诊断本病。. 治疗包括给予抗生素预防或治疗感染 ...
STAT3 Hyper IgE Syndrome - GeneReviews® - NCBI Bookshelf
网页2010年2月23日 · STAT3 hyper IgE syndrome (STAT3-HIES) is a primary immune deficiency syndrome characterized by elevated serum IgE, eczema, and recurrent skin and respiratory tract infections, together with several nonimmune features. This disorder typically manifests in the newborn period with a rash (often diagnosed as eosinophilic pustulosis) that ...
STAT3 Hyper-IgE Syndrome—an Update and Unanswered …
网页2021年5月1日 · The hyper-IgE syndromes (HIES) are a heterogeneous group of inborn errors of immunity sharing manifestations including increased infection susceptibility, eczema, and raised serum IgE. Since the prototypical HIES description 55 years ago, areas of significant progress have included description of key disease-causing genes and differentiation ...
Hyper-IgE Syndrome - Hyper-IgE Syndrome - The Merck Manuals
网页Hyper-IgE syndrome is a hereditary immunodeficiency disorder characterized by recurring boils, sinus and lung infections, and a severe rash that appear during infancy. Levels of immunoglobulin E (IgE) are very high. In infants with hyper-IgE syndrome, abscesses form in the skin, joints, lungs, or other organs.
Hyper-Immunoglobulin E Syndromes (HIES) | NIAID: National …
网页Many different syndromes are known to lead to high levels of an antibody called immunoglobulin E, or IgE. Many more such syndromes likely remain unknown. Collectively, these conditions are called hyper-IgE syndromes, or HIES. Other conditions, such as severe eczema, can lead to extremely high IgE levels …