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A Nationwide Survey of Lymphangioleiomyomatosis in Korea: Recent Increase in Newly Diagnosed Patients

Cited 12 time in Web of Science Cited 13 time in Scopus
Authors
Park, Hye Yun; Nam, Hae-Seong; Chung, Man Pyo; Jeong, Sung Hwan; Cha, Seung-Ick; Park, Jong Sun; Park, Choon-Sik; Moon, Ji Ae; Jegal, Yang Jin; Park, Young Bum; Yum, Ho-Kee; Song, Jin Woo; Kim, Dong Soon; Jung, Kyung Soo; Park, Moo Suk; Uh, Soo-Taek; Kim, Young Whan; Kim, Yu Jin
Issue Date
2010-08
Publisher
KOREAN ACAD MEDICAL SCIENCES
Citation
JOURNAL OF KOREAN MEDICAL SCIENCE; Vol.25 8; 1182-1186
Keywords
LymphangioleiomyomatosisRespiratory Function TestsRegistriesKorea
Abstract
In 2007, the Korean Interstitial Lung Disease Society had collected clinical data of patients who have diagnosed as Lymphangioleiomyomatosis (LAM) since 1990 through nationwide survey, which showed that LAM patients had increased sharply after 2004. The present study was performed to show the clinical features of Korean patients with LAM, and to establish the reason for the recent increase in the diagnosis. All 63 patients were women and the mean age at diagnosis was 36 yr. The most common presenting symptom was dyspnea and 8 patients had tuberous sclerosis complex. The survival rate at 5 yr after diagnosis was 84%. Compared with patients diagnosed after 2004 (n=34), the patients diagnosed before 2004 (n=29) complained with dyspnea more (P=0.016) and had lower FEV(1)% predicted (P=0.003), and DLco% predicted (P=0.042). The higher proportion of patients diagnosed after 2004 showed the normal chest radiography, and they were detected by routine chest CT screening (P=0.016). This study showed that clinical features of Korean patients with LAM were not different from those reported elsewhere. It is concluded that the reason for the increase of newly diagnosed patients is the result of increase in detection of the early stage LAM by the widespread use of chest CT screening.
ISSN
1011-8934
Language
English
URI
http://hdl.handle.net/10371/76475
DOI
https://doi.org/10.3346/jkms.2010.25.8.1182
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College of Medicine/School of Medicine (의과대학/대학원)Internal Medicine (내과학전공)Journal Papers (저널논문_내과학전공)
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