A 62 years old, post-menopausal female was admitted to the Internal Medicine Ward due to dyspnoea, cough and sputum of at least 2 months. Shortness of breath, cough and hypoxaemia persisted and the patient was submitt...A 62 years old, post-menopausal female was admitted to the Internal Medicine Ward due to dyspnoea, cough and sputum of at least 2 months. Shortness of breath, cough and hypoxaemia persisted and the patient was submitted to a pulmonary angiogram ct which revealed numerous thin-walled air cysts affecting upper and medial zones of both lungs, typical images of pulmonary lymphangioleiomyomatosis. After discharge to Internal Medicine Consultation Service with Metilprednisolone, the patient was no longer hypoxaemic and remained asymptomatic, even after withdrawal of oral corticosteroid to inhalatory formulation. Further surveillance in short time was scheduled in order to implement rapid imunossupressive treatment when necessary.展开更多
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(...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.展开更多
文摘A 62 years old, post-menopausal female was admitted to the Internal Medicine Ward due to dyspnoea, cough and sputum of at least 2 months. Shortness of breath, cough and hypoxaemia persisted and the patient was submitted to a pulmonary angiogram ct which revealed numerous thin-walled air cysts affecting upper and medial zones of both lungs, typical images of pulmonary lymphangioleiomyomatosis. After discharge to Internal Medicine Consultation Service with Metilprednisolone, the patient was no longer hypoxaemic and remained asymptomatic, even after withdrawal of oral corticosteroid to inhalatory formulation. Further surveillance in short time was scheduled in order to implement rapid imunossupressive treatment when necessary.
基金Supported by National Basic Research Program of China (973 Program) (2009CB522106)
文摘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.
