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肺是镰形细胞病的靶器官,晚期镰形细胞性慢性肺部疾病(SCLD)可发生肺动脉高压,并常伴有肺心病。SCLD的确诊依据是永久性肺部疾病的临床表现,慢性低氧血症,X线示肺弥漫性间质纤维化,肺心病伴有右室肥大及肺功能试验异常。本文报告SCLD 28例,男15例,女13例。SCLD共分四期,随病期进展,胸痛、慢性咳嗽、乏力逐渐加重,运动耐量降低,直至出现静息时呼吸困难。
Lung is the target organ of sickle cell disease, advanced falx cell chronic lung disease (SCLD) can develop pulmonary hypertension, and often accompanied by pulmonary heart disease. SCLD diagnosis based on the clinical manifestations of permanent lung disease, chronic hypoxemia, X-ray showed diffuse interstitial fibrosis, pulmonary heart disease accompanied by abnormal right ventricular hypertrophy and pulmonary function tests. This article reports 28 cases of SCLD, 15 males and 13 females. SCLD is divided into four phases, with the progress of the disease, chest pain, chronic cough, fatigue gradually increased, decreased exercise tolerance, breathing difficulties until resting.