| 标题： | 高IgE综合征中国专家共识 |
| title： | Chinese expert consensus on the hyper-IgE syndrome |
| 版本： | 原创版 |
| version： | Original |
| 分类： | 专家共识 |
| classification： | Experts consensus |
| 领域： | 诊疗 |
| field： | Diagnosis and Treatment |
| 国家和地区： | 中国 |
| Country and region： | China |
| 指南使用者： | 临床医生 |
| Guide users： | Clinician |
| 证据分级方法： | NO |
| Evidence grading method： | NO |
| 制定单位： | 中国医药教育协会儿科专业委员会 |
| Formulating unit： | China Medicine Education Association Committee on Pediatrics |
| 注册时间： | 2024-01-03 |
| Registration time： | |
| 注册编号： | PREPARE-2024CN120 |
| Registration number： | |
| 指南制订的目的： | 高IgE综合征（The hyper-IgE syndromes，HIES），是以血清IgE显著升高，湿疹及反复感染为三联征的一组罕见原发免疫缺陷病的总称。最早在1966年Davis首次报道 “Job综合征”而被认识。近年来，基因检测的进展能够识别更多HIES类型。然而，HIES的早期诊断仍然是一个挑战，因为在生命的早期，HIES很难与其他以湿疹和高IgE为特征的疾病区分开来，因此，为临床医生更好的早期识别和规范治疗HIES制定此专家共识。 |
| Purpose of the guideline： | The hyper-IgE syndromes (HIES) is a group of rare primary immunodeficiency diseases with significantly elevated serum IgE, eczema and recurrent infections as the triad. It was first recognized in 1966 when Davis first reported "Job Syndrome". In recent years, advances in genetic testing have enabled the identification of more HIES types. However, the early diagnosis of HIES remains a challenge, as it is difficult to distinguish HIES from other diseases characterized by eczema and high IgE early in life, therefore, this expert consensus was developed for clinicians to better identify early and standardize the treatment of HIES. |
