The St.George's Respiratory Questionnaire in Lymphangioleiomyomatosis  

作　　者：Kai-feng Xu LanWang Xin-lun Tian Yao-song Gui Min Peng Bai-qiang Cai Yuan-jue Zhu 

机构地区：[1]Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences ＆ Peking Union Medical College, Beijing 100730, China

出　　处：《Chinese Medical Sciences Journal》2010年第3期140-145,共6页中国医学科学杂志（英文版）

基　　金：Supported by National Basic Research Program of China (973 Program) (2009CB522106)

摘　　要：Objective To examine the correlation between the health-related quality of life measured by the St.George's Respiratory Questionnaire(SGRQ) and the commonly used physiological measures in lymphangioleiomyomatosis(LAM).Methods This study retrospectively analyzed the SGRQ scores and other measures(the Borg scale of breathlessness at rest,6-minute walking distance,blood oxygen levels,and pulmonary function) of patients diagnosed and confirmed with LAM.Altogether 38 patients between June 2007 and November 2009 were included.Results The mean values of the SGRQ three components(symptoms,activity,and impacts) and total scores in the LAM patients were 46.95±28.90,58.47±25.41,47.89±29.66,and 51.11±26.35,respectively.The SGRQ total or component scores were correlated well with the Borg scale of breathlessness,6-minute walking distance,partial pressure of oxygen in arterial blood,spirometry and diffusion capacity of lung.There were poor correlations between SGRQ score and residual volume or total lung capacity.In our preliminary observation,sirolimus improved the SGRQ total and three component scores and the Borg scale of breathlessness significantly after 101-200 days of treatment(n=6).Conclusions The SGRQ score in LAM is correlated well with physiological measures(Borg scale of breathlessness,6-minute walking distance,blood oxygen levels,and pulmonary function tests).The SGRQ could therefore be recommended in baseline and follow-up evaluation of patients with LAM.Treatment with sirolimus,an inhibitor of mammalian target of rapamycin,may improve the quality of life and patient's perception of breathlessness in LAM.

关 键 词：LYMPHANGIOLEIOMYOMATOSIS St. George＇s Respiratory Questionnaire pulmonary function quality of life SIROLIMUS 

分 类 号：S858.316.3[农业科学—临床兽医学] Q445[农业科学—兽医学]