摘要
对中南大学湘雅二医院呼吸内科成功收治的首例肺及腹膜后淋巴管肌瘤病的临床资料进行报道分析,并对相关文献进行复习。女性患者,45岁,活动后气促半年,胸痛4 d入院。入院检查发现乳糜胸,双肺间质病变,腹膜后淋巴结肿大。剖腹探查腹膜后淋巴结活检证实肺及腹膜后淋巴管肌瘤病,经雷帕霉素治疗半年后症状、肺功能、血气、影像学均明显好转。肺淋巴管肌瘤病常见临床表现为进行性呼吸困难,反复发作的气胸,以及乳糜胸,特征性高分辨率CT(high-resolution computed tomography,HRCT)表现或病理检查可确诊。雷帕霉素为该病的治疗带来了希望。
To improve the diagnosis and treatment of pulmonary lymphangiomyomatosis, clinical data for the first successfully treated case of pulmonary and retroperitoneal lymphangiomyomatosis in our hospital has been comprehensively analyzed, and the relevant literature has been reviewed. A 4S-year-old Han Chinese woman initially presented six months ago with increasing shortness of breath on exertion and was admitted to our hospital after four days of chest pain. Admission examination revealed chylothorax, interstitial lung disease, and enlarged retroperitoneal lymph nodes. The patient was finally diagnosed with pulmonary and retroperitoneal lymphangiomyomatosis based on laparotomy examination and biopsy of the retroperitoneal lymph nodes. After six months of rapamycin treatment, the symptoms - lung function, arterial blood gas, and imaging of the patientwere improved significantly Pulmonary lymphangiomyomatosis clinically manifests as progressive dyspnea, recurrent pneumothorax, and chylothorax, and can be diagnosed by its characteristic features in high-resolution computed tomographic images or pathological examination. The successful treatment of pulmonary lymphangiomyomatosis with rapamycin brings new hope to those afflicted with this disease.
出处
《中南大学学报（医学版）》
CAS
CSCD
北大核心
2012年第9期963-967,共5页
Journal of Central South University ：Medical Science
基金
湖南省自然科学基金(07JJ3037)~~
关键词
肺淋巴管肌瘤病
雷帕霉素
诊断
治疗
lymphangioleiomyomatosis
rapamycin
diagnosis
treatment
