Clinical features of 280 hospitalized patients with lymphangioleiomyomatosis in Japan
| Autor: | Hiroki Matsui, Mitsuhiro Sunohara, Takahide Nagase, Yasuhiro Yamauchi, Taisuke Jo, Kiyohide Fushimi, Wakae Hasegawa, Hideo Yasunaga, Kazutaka Takami |
|---|---|
| Rok vydání: | 2014 |
| Předmět: |
Pulmonary and Respiratory Medicine
medicine.medical_specialty Angiomyolipoma business.industry medicine.medical_treatment Mortality rate medicine.disease Comorbidity Transplantation surgical procedures operative Pneumothorax Respiratory failure Internal medicine Lymphangioleiomyomatosis Medicine Lung transplantation business Intensive care medicine |
| Zdroj: | Respirology. 20:160-165 |
| ISSN: | 1323-7799 |
| Popis: | Background and objective 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. Methods 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. Results 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. Conclusions 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text