Your browser doesn't support javascript.
loading
Primary Ciliary Dyskinesia with Double Lung Transplantation: a case report and literature review / 中华器官移植杂志
Article in Chinese | WPRIM | ID: wpr-870595
Responsible library: WPRO
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.
Full text: Available Index: WPRIM (Western Pacific) Language: Chinese Journal: Chinese Journal of Organ Transplantation Year: 2020 Type: Article

Similar

MEDLINE

...
LILACS

LIS

Full text: Available Index: WPRIM (Western Pacific) Language: Chinese Journal: Chinese Journal of Organ Transplantation Year: 2020 Type: Article