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儿童高IgE综合征伴反复气胸1例 被引量:1

A Case of Childhood Hyper-IgE Syndrome Accompanied with Recrurrent Pneumothorax
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摘要 患儿男,6岁,全身反复湿疹样皮疹伴脓肿6年。皮肤科情况:头面部散在红色丘疹,伴瘙痒。躯干及四肢多处陈旧性皮损。左侧腹股沟可触及包块,红肿及压痛明显。实验室检查:总IgE>1 190.0 IU/mL;嗜酸性粒细胞2.33×10^(9)/L。胸部CT:右侧气胸伴右肺压缩,两肺多发含气囊腔。基因检测:信号转导和转录活化因子3(STAT3)有1个杂合变异。诊断:高IgE综合征,肺囊肿,气胸。治疗:系统抗菌、免疫球蛋白替代治疗及胸腔闭式引流1周后症状明显缓解。 A 6-year-old male presented with recurrent multiple skin eczema-like rash and abscess for six years.Skin examination revealed rough and multiple red papules with pruritus in the skin of head and face.Old rashes were found in the skin of trunk and limbs.A red, swollen and tender mass was found in the left inguinal area.Laboratory investigations showed total IgE>1 190.0 IU/mL,eosinophils(2.33×10^(9)/L).Imaging examination of CT showed right pneumothorax with compression of the right lung, and multiple cyst of both lung.Results of genetic detecting showed a heterozygous mutation in signal transducer and activator of transcription 3(STAT3).Diagnosis: Hyper-IgE syndrome, cyst of pulmonary, pneumothorax.After one week of systemic treatment with antibiotics, immunoglobulin replacement therapy and thoracic drainage, the symptoms were significantly alleviated.
作者 韩絮 高跃 HAN Xu;GAO Yue(Department of Clinical Laboratory,the Children's Hospital,Zhejiang University School of Medi-cine,National Clinical Research Center for Child Health,National Regional Medical Center for Chil-dren,Hangzhou 310052,China;Department of Paediatric Cardio-Thoracic Surgery,the Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,National Regional Medical Center for Children,Hangzhou 310052,China)
出处 《中国皮肤性病学杂志》 CAS CSCD 北大核心 2022年第1期90-93,共4页 The Chinese Journal of Dermatovenereology
关键词 高IGE综合征 STAT3突变 气胸 Hyper-IgE syndrome STAT3-mutations Pneumothorax
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