Baba M, Mikami M, Nakamura S, Takizawa J, Kawakami M
Department of Pneumology, Tokyo Metropolitan Hiroo General Hospital, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1997 Nov;35(11):1223-7.
A 32-year-old woman was admitted with persistent dyspnea. Chest roentgenogram showed hyperinflation of the lungs, and diffuse reticular shadows in both lung fields. Chest CT showed diffuse cystic lesions and thickened vasculature. Examinations revealed severe hypoxemia, restrictive and obstructive ventilatory impairments, increased residual volume, and decreased carbon monoxide diffusing capacity. Lymphagioleiomyomatosis was strongly suspected, and a diagnosis confirmed histopathologically by lung biopsies. Inhalation of flutropium bromide was remarkably effective in relieving dyspnea and impaired pulmonary functions. It is suggested that flutropium bromide is beneficial for symptomatic improvement in patients with lymphangioleiomyomatosis.
一名32岁女性因持续性呼吸困难入院。胸部X线片显示肺过度充气,双肺野弥漫性网状阴影。胸部CT显示弥漫性囊性病变和血管增粗。检查发现严重低氧血症、限制性和阻塞性通气功能障碍、残气量增加以及一氧化碳弥散量降低。高度怀疑为淋巴管平滑肌瘤病,经肺活检病理组织学确诊。吸入氟替卡松溴化物对缓解呼吸困难和肺功能损害非常有效。提示氟替卡松溴化物对淋巴管平滑肌瘤病患者的症状改善有益。