Clinical features of 280 hospitalized patients with lymphangioleiomyomatosis in Japan.

[lymphangioleiomyomatosis]

Lymphangioleiomyomatosis (LAM ) is rare , but potentially life threatening owing to respiratory failure . However , knowledge is limited about the condition of hospitalized LAM patients . The objectives of this study were to investigate patient characteristics , comorbidities and causes of death among hospitalized LAM patients in Japan .Using a national inpatient database in Japan , information on 280 LAM patients hospitalized between July 2010 and March 2013 was retrospectively collected . We divided the 280 patients into three groups according to their status regarding lung transplantation .For the study period , we identified 32 patients who had undergone lung transplantation ('after-transplantation ' group ), 12 patients admitted for lung transplantation ('for-transplantation ') and 236 patients who had not undergone transplantation ('no -transplantation '). Although the clinical features of LAM patients in the 'no -transplantation ' group were similar to previously reported findings , patients hospitalized in connection with transplantation showed the following : the activities of daily living score using the Barthel Index in the 'after-transplantation ' group (89 .4 ) was significantly higher than in the 'for-transplantation ' group (64 .6 ); the mortality rates in the after-transplantation group (3 .1 %) were significantly lower than in the for-transplantation group (25 %). The most frequent comorbidity was pneumothorax , followed by respiratory failure and angiomyolipoma , although there was no significant difference in the prevalence among the three groups .We determined the clinical features , comorbidities and fatalities in hospitalized LAM patients . Patients with LAM after transplantation had higher activities of daily living scores than those before transplantation , which suggests that lung transplantation may improve activities of daily living .

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lymphangioleiomyomatosis

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