摘要
Abstract
Objective To discuss the clinical features of lymphangioleiomyomatosis ( LAM ).Methods Clinical features of LAM were summarized through analyzing in details the clinical manifestations and laboratory results of 12 cases.Results All the 12 patients were female and 10 0f them were in their reproductive period; Respiratory symptoms including recurrent pneumothorax, dysnpea, chylothorax, and hemoptysis were found in 10 cases as initial symptoms; initial symptoms for the other 2cases were abdominal pain, yet one patient also complained associated dysnpea and the other patient had a history of chylothorax ;II patients underwent examination of lung functions, which showed either obstructive ventilatory disturbance or mixed ventilatory disturbance; all 12 patients underwent CT examinations and bilateral diffused cystic airspace changes were noted in all cases,pulmonary bullae were found in 2 cases, enlargement of mediastinal lymph nodes were found in 3 cases, pelvic masses were found in 2 cases, renal mass were found in 1 case, enlargement of retroperitoneal lymph nodes were found in 2 cases; all cases received anti - estrogen therapy which was barely effective.Conclusion LAM is a lung disease of women in reproductive period,the main symptoms of which include dysnpea, pneumothorax, chylothorax, hemoptysis, ahdominal pain, and abdominal pelvic mass.Associated obstructive ventilatory disturbance and cystic airspace CT manifestations are also noted among LAM patients.The disease lacks effective treatment so far.关键词
淋巴管肌瘤/症状和体征/诊断Key words
Lymphangiomyoma/ Symptoms & signs/ Diagnosis分类
医药卫生
