Primary Ciliary Dyskinesia with Double Lung Transplantation: a case report and literature review
10.3760/cma.j.cn421203-20190805-00315
- VernacularTitle:原发性纤毛运动不良症双肺移植术一例并文献复习
- Author:
Ao CHEN
1
;
Qiaoyan LIAN
;
Xin XU
;
Bing WEI
;
Danxia HUANG
;
Xiuhua LI
;
Rongchang CHEN
;
Jianxing HE
;
Chunrong JU
Author Information
1. 广州医科大学附属第一医院移植科 广州呼吸疾病研究院 510012
- From:
Chinese Journal of Organ Transplantation
2020;41(6):341-345
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the diagnosis and treatment strategies of primary ciliary dyskinesia (PCD).Methods:A 37-year-old male recipient who had repeated cough and sputum from childhood, with shortness of breath after activity and progressive development, the number of hospitalizations per year was ≥6 times, the MRC score was 2~3 diagnosed with primary ciliary dyskinesia through medical examination and multidisciplinary consultation, and received allogeneic double lung transplantation after medical treatment failure. Search related domestic and foreign literatures to explore and analyze the etiology, pathogenesis, clinical manifestations and imaging features, diagnosis and treatment of PCD.Results:PCD is an autosomal hereditary disease. Due to abnormal skeletal structure and/or functional development, clinical manifestations are mostly chronic sinusitis, bronchiectasis, otitis media and infertility. Chest imaging showed situs inversus totalis, right heart and diffuse cystic bronchiectasis infection with bronchial wall thickening, diagnosis depends on clinical manifestations, saccharin test, nasal nitric oxide test, high-speed video microscopy analysis, transmission electron microscopy, genetic testing and immunofluorescence.Conclusions:Lung transplantation is the only effective treatment for end-stage PCD. It not only saves patients' lives, but also significantly improves their quality of life.
