{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T22:50:12Z","timestamp":1791240612221,"version":"4.1.0"},"reference-count":26,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2007,6,6]],"date-time":"2007-06-06T00:00:00Z","timestamp":1181088000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2007,7]]},"abstract":"<jats:p>\n                    <jats:bold>Background and objective:\u2003<\/jats:bold>\n                    To evaluate the characteristics and prognostic factors of Japanese patients with lymphangioleiomyomatosis (LAM).\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Methods:\u2003<\/jats:bold>\n                    A nationwide survey to identify patients with LAM was conducted by questionnaire. Survival probability was estimated using the Kaplan\u2013Meier method, and the prognostic factors were analysed by Cox regression.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Results:\u2003<\/jats:bold>\n                    Data were collected on 173 patients with pulmonary LAM. The major presenting features were pneumothorax (43%) and exertional dyspnoea (37%). The survival probabilities for patients presenting with exertional dyspnoea (Group A) were 85%, 60% and 47% after 5, 10 and 15\u2003years, respectively, and for patients presenting with pneumothorax (Group B) were 95%, 89% and 89%, respectively. Although the age at symptom onset was higher among patients in Group A than in Group B, Cox regression revealed that the presenting feature was a prognostic factor independent of age at symptom onset (Group A\/B hazard ratio\u2003=\u20035.732,\n                    <jats:italic>P<\/jats:italic>\n                    \u2003&lt;\u20030.01). In the subgroup of patients whose initial FEV\n                    <jats:sub>1<\/jats:sub>\n                    was &gt;1000\u2003mL, or FEV\n                    <jats:sub>1<\/jats:sub>\n                    \/FVC &gt;40%, or %DL\n                    <jats:sub>CO<\/jats:sub>\n                    &gt;40%, the rate of deterioration in these tests was greater in Group A than in Group B (\n                    <jats:italic>P<\/jats:italic>\n                    \u2003&lt;\u20030.01 for FEV\n                    <jats:sub>1<\/jats:sub>\n                    ,\n                    <jats:italic>P<\/jats:italic>\n                    \u2003&lt;\u20030.05 for FEV\n                    <jats:sub>1<\/jats:sub>\n                    \/FVC and %DL\n                    <jats:sub>CO<\/jats:sub>\n                    ).\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Conclusions:\u2003<\/jats:bold>\n                    There are two possible subgroups of LAM patients. One subgroup that presented with pneumothorax, had onset of symptoms at a younger age and a more favourable prognosis; the other presented with exertional dyspnoea, had onset of symptoms at an older age and a poorer prognosis.\n                  <\/jats:p>","DOI":"10.1111\/j.1440-1843.2007.01101.x","type":"journal-article","created":{"date-parts":[[2007,6,6]],"date-time":"2007-06-06T14:33:59Z","timestamp":1181140439000},"page":"523-530","source":"Crossref","is-referenced-by-count":92,"title":["The epidemiology of lymphangioleiomyomatosis in Japan: A nationwide cross\u2010sectional study of presenting features and prognostic factors"],"prefix":"10.1111","volume":"12","author":[{"given":"Mie","family":"HAYASHIDA","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kuniaki","family":"SEYAMA","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshikazu","family":"INOUE","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keisaku","family":"FUJIMOTO","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keishi","family":"KUBO","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"The Respiratory Failure Research Group of the Japanese Ministry of Health, Labor, and Welfare","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,6,6]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000441-200101000-00004"},{"key":"e_1_2_5_3_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.163.1.2005004"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10038-002-8651-8"},{"key":"e_1_2_5_5_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.121.3_suppl.56S"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1165\/rcmb.2004-0289OC"},{"key":"e_1_2_5_7_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.2010014"},{"key":"e_1_2_5_8_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200409-1298OC"},{"key":"e_1_2_5_9_2","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2004.035154"},{"key":"e_1_2_5_10_2","doi-asserted-by":"publisher","DOI":"10.1136\/thorax.55.12.1052"},{"key":"e_1_2_5_11_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-199909000-00004"},{"key":"e_1_2_5_12_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.151.2.7842216"},{"key":"e_1_2_5_13_2","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.216.1.r00jl42147"},{"key":"e_1_2_5_14_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199011013231807"},{"key":"e_1_2_5_15_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1440-1843.2001.00343.x"},{"key":"e_1_2_5_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2003.11.013"},{"key":"e_1_2_5_17_2","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.120.4.832"},{"key":"e_1_2_5_18_2","first-page":"348","article-title":"Pulmonary lymphangiomyomatosis: a review","volume":"79","author":"Corrin B","year":"1975","journal-title":"Am. 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