一名17岁女孩患气管狭窄，为免疫增殖性高IgG4病的孤立形式病例。
A Case of Tracheal Stenosis as an Isolated Form of Immunoproliferative Hyper-IgG4 Disease in a 17-Year-Old Girl.
作者信息
Gabrovska Natalia, Velizarova Svetlana, Spasova Albena, Kostadinov Dimitar, Yanev Nikolay, Shivachev Hristo, Rangelov Edmond, Pahnev Yanko, Antonova Zdravka, Kartulev Nikola, Terziev Ivan, Gabrovski Kaloyan
机构信息
Department of Pulmonary Diseases, Multiprofile Hospital for Active Treatment of Pulmonary Diseases "St. Sofia", Medical University-Sofia, 1431 Sofia, Bulgaria.
Department of Pediatric Thoracic Surgery, Pediatric Surgery Clinic, University Multiprofile Hospital for Active Treatment and Emergency Medicine "N. I. Pirogov", 1606 Sofia, Bulgaria.
出版信息
Children (Basel). 2021 Jul 12;8(7):589. doi: 10.3390/children8070589.
Immunoglobulin G4-related disease (IgG4-RD) is a lymphoproliferative disease which is described almost exclusively in adults. There are only a few pediatric patients who have been observed with this disorder. Here, we describe a rare case of IgG4-RD in a 17-year-old girl with a single manifestation-tracheal stenosis without previous intubation or other inciting event. She had mixed dyspnea and noisy and weakened breathing. Immunoproliferative hyper-IgG4 disease was diagnosed, based on elevated serum IgG4 and histological findings. Until now we have chosen to treat the girl only with corticosteroids with a good response so far. The general condition as well as the respiratory function are regularly monitored. The tracheal involvement of IgG4-RD is uncommon. Nonetheless, it is a manifestation that should be included in the differential diagnosis of tracheal stenosis.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种几乎仅在成人中被描述的淋巴增殖性疾病。仅有少数儿科患者被观察到患有这种疾病。在此，我们描述了一名17岁女孩罕见的IgG4-RD病例，其仅有气管狭窄这一单一表现，既往无插管或其他诱发事件。她有混合性呼吸困难以及呼吸嘈杂和减弱。基于血清IgG4升高和组织学检查结果，诊断为免疫增殖性高IgG4疾病。到目前为止，我们选择仅用皮质类固醇治疗该女孩，迄今为止反应良好。定期监测一般状况以及呼吸功能。IgG4-RD累及气管并不常见。尽管如此，它是气管狭窄鉴别诊断中应包括的一种表现。
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