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A nationwide survey of lymphangioleiomyomatosis in Korea: recent increase in newly diagnosed patients.

Park HY, Nam HS, Chung MP, Jeong SH, Kim YJ, Cha SI, Kim YW, Park JS, Uh ST, Park CS, Park MS, Moon JA, Jung KS, Jegal YJ, Kim DS, Song JW, Yum HK, Park YB - J. Korean Med. Sci. (2010)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

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.

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Related in: MedlinePlus

Kaplan-Meier survival analysis of mortality of 63 patients with LAM. The probability of being alive was 84% at 5 yr and 65% at 8.5 yr after diagnosis.
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Figure 2: Kaplan-Meier survival analysis of mortality of 63 patients with LAM. The probability of being alive was 84% at 5 yr and 65% at 8.5 yr after diagnosis.

Mentions: During follow-up, 12 patients died within a median duration of 60 months (1-144 months). Cause of death was attributed to LAM in all patients, directly from respiratory failure (n=11) and lung transplantation morbidity (n=1). The survival probability, evaluated by the Kaplan-Meier method, is shown in Fig. 2. The probability of being alive was 84% at 5 yr and 65% at 8.5 yr after diagnosis.


A nationwide survey of lymphangioleiomyomatosis in Korea: recent increase in newly diagnosed patients.

Park HY, Nam HS, Chung MP, Jeong SH, Kim YJ, Cha SI, Kim YW, Park JS, Uh ST, Park CS, Park MS, Moon JA, Jung KS, Jegal YJ, Kim DS, Song JW, Yum HK, Park YB - J. Korean Med. Sci. (2010)

Kaplan-Meier survival analysis of mortality of 63 patients with LAM. The probability of being alive was 84% at 5 yr and 65% at 8.5 yr after diagnosis.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2908788&req=5

Figure 2: Kaplan-Meier survival analysis of mortality of 63 patients with LAM. The probability of being alive was 84% at 5 yr and 65% at 8.5 yr after diagnosis.
Mentions: During follow-up, 12 patients died within a median duration of 60 months (1-144 months). Cause of death was attributed to LAM in all patients, directly from respiratory failure (n=11) and lung transplantation morbidity (n=1). The survival probability, evaluated by the Kaplan-Meier method, is shown in Fig. 2. The probability of being alive was 84% at 5 yr and 65% at 8.5 yr after diagnosis.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

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.

Show MeSH
Related in: MedlinePlus