• Tuberculosis and Respiratory Diseases
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• v.66 no.2
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• pp.141-151
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• 2009

Background: There is limited data on the epidemiology and relative frequency of idiopathic interstitial pneumonia (IIP) worldwide. This survey was performed to assess the epidemiology and relative frequency of IIP in Korea. Methods: The patients with IIP and who were confirmed by lung biopsy, except those patients with idiopathic pulmonary fibrosis, (IPF) over a 5 year period (from Jan. $1^{st}$, 2003 to Dec. $31^{st}$, 2007) were registered by a web-base questionnaire. Results: A total of 3,156 cases were registered, but 970 cases were excluded due to duplicative registration, inadequate data and the unmet ATS/ERS diagnostic criteria. A total of 2,186 cases were analyzed. The male to female ratio was about 2 : 1 and their mean age was 65 (range: 11-94). The most frequent disease was IPF (77.1%), followed in decreasing order by nonspecific interstitial pneumonia (NSIP) (11.9%), cryptogenic organizing pneumonia (COP) (8.5%), acute interstitial pneumonia (AIP) (1.1%), desquamative interstitial pneumonia (DIP) (0.9%), respiratory bronchiolitis-interstitial lung disease (RB-ILD) (0.4%) and lymphocytic interstitial pneumonia (LIP) (0.1%). The mean age of the patients with IPF, NSIP and COP was 67.8, 57.1 and 57.7 years old, respectively. The most frequent symptom was dyspnea on exertion (69%) followed by coughing (61%) and sputum (33%) for the whole population. The three year survival rate was 62% for the patients with IPF and the five year survival rate was 85% in both the NSIP and COP patients. Conclusion: This survey provides helpful information for the management of IIP and to produce management guidelines for this illness in Korea.

• Tuberculosis and Respiratory Diseases
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• v.53 no.3
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• pp.309-318
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• 2002

Background : Usual interstitial pneumonia (UIP) is a fatal progressive fibrotic disorder of the lung with unknown etiology and characterized by a poor response to conventional immunosuppressive therapy. The histologic hallmark of UIP is parchy distribution of subpleural fibrosis and fibroblastic foci(FBF) with interposed normal appearing lung. Because FBF is a collection of actively proliferating myofibroblasts, it can be a marker of activity and prognosis of UIP. However, there were contradictory reports about the correlation between the degree of FBF and survival. Therefore we performed this study to investigate the value of FBF as prognostic marker of UIP. Methods : This was a retrospective study on the 46 patients(M:F=33:13, mean age:$59{\pm}12$ years) with UIP diagnosed by the surgical lung biopsy at the Asan Medical Center, Seoul, Korea between 1990 and 2000 and had follow-up of more than a year. All the biopsy specimens were reevaluated and diagnosed as UIP according to the ATS/ERS classification. Semiquantitative grading of FBF(absent, 0; mild 1; moderate 2; marked 3) by the experienced pathologists who did not know the clinical findings were compared to the clinical data and the follow up course. Results : Thirteen patients(28.2%) died of UIP progression during the study period. The median survival time of all the subjects was 26 months after the biopsy. At the univariate analysis, FVC, $D_Lco$, smoking history and the grade of FBF were significantly related to the survial. The survival was longer in subjects with lesser degrees of FBF, higher DLco, higher FVC and history of smoking. However the multivariate analysis with Cox regression test showed the extent of FBF was the only independent prognostic marker of UIP. Conclusion : These data suggested that the extent of FBF on the surgical lung biopsy can be used as a prognostic marker of UIP.

• Clinical and Experimental Pediatrics
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• v.51 no.4
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• pp.383-390
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• 2008

Purpose : This study was aimed to analyze the clinical characteristics of patients with acute interstitial pneumonia who had presented similar clinical patterns from March to June, 2006 and to describe our experience of treatment and to identify risk factors associated with prognosis. Methods : The clinical characteristics, radiologic and histopathologic findings and response to steroids of 15 patients (non-survival group [n=7] and survival group [control, n=8]) with acute interstitial pneumonia were investigated through the review of medical records. Results : The mean age of the patients was 26 (range: 3-48) months. Cough, cyanosis and fever were frequent symptoms. The most frequent radiologic findings on admission were pneumomediastium and extensive ground glass opacity. Surgical lung biopsy was performed on 8/15 (53.3%) patients and diffuse alveolar damage was found. Mechanical ventilation was applied for 9/15 (60.0%) patients for 40 (range: 1-99) days. Five patients in survival group received steroid treatment and 7 patients in non-survivial group (P=0.20). One patient in survival group received steroid pulse treatment and 4 patients in non-survival group (P=0.12). Seven patients died all of respiratory failure. The survival rate was 53.4%. Conclusion : The patients with acute interstitial pneumonia which occurred on spring 2006 showed high mortality because of rapidly and extensively progressing pulmonary fibrosis and air leakage. Therefore, we should consider surgical lung biopsy and steroid application earlier. We should recognize this acute interstitial pneumonia occurring on spring in domestics and need to investigate the cause and treatment in large scale.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.174-182
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• 2003

Purpose: Non-alcoholic fatty liver disease (NAFLD) has been recognized as an important childhood liver disease, especially where the prevalence of childhood obesity is increasing. The purpose of this study is to clarify the usefulness of elevated serum aminotransferase activities and their ratio for predicting the presence of fatty liver and its severity in obese children. Methods: Forty-four children (M/F 29/15, age 4 to 16 years) with obesity (weight excess>20%) were analyzed retrospectively with medical records based on degree of obesity, bioelectrical impedence, serum aminotransferase activities, lipid profiles and ultrasonography. Results: 1) Ultrasonography was carried out in 34 cases. Elevated serum ALT was found in 89.7% (26/29) of the patients diagnosed as fatty liver by ultrasonography and decreased AST/ALT ratio (<1) was found in 96.6% (28/29). There was a strong correlation between elevated serum ALT (>45 IU/L) or decreased AST/ALT ratio (<1) and the fatty liver in obese children (p<0.05). 2) There was no significant correlation between the serum ALT or AST/ALT ratio and the degree of fatty liver (p>0.05). 3) There was a significant correlation between total cholesterol, triglyceride and fatty liver (p<0.05). Conclusion: Serum ALT activity and AST/ALT ratio were useful to predict the presence of fatty liver diagnosed by ultrasonography in obese children, whereas they were not useful to predict the degree of fatty liver. Therefore, to prevent fatty liver progressing to advanced liver disease, it is necessary to manage and monitor the obese children continuously, especially those who have predicting factors of fatty liver.

• Clinical and Experimental Pediatrics
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• v.50 no.3
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• pp.241-247
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• 2007

Purpose : Congenital muscular torticollis (CMT) is a common and benign congenital disorder of the musculoskeletal system in neonates and infants. The pathophysiology is that the sternocleidomastoid muscle (SCM) is shortened on the involved side by fibrosis, leading to ipsilateral tilt and contralateral rotation of the face and chin. In this study, we investigated the clinical features of CMT, the role of ultrasonography (USG) in prediction of prognoses and the clinical significance of early detection and treatment. Methods : Forty seven patients (M:F=31:16) were diagnosed as a CMT between March 2003 and May 2006. We reviewed age at diagnosis, physical findings, USG findings, treatment and therapeutic outcome from their medical records. Results : The median age at diagnosis was 90 days (18 days-9 years, 7 months) and the right side of neck was affected in more patients (right : left=26:21). Of 24 patients with a palpable neck mass, 21 had USG; 19 cases showed sternocleidomastoid tumor (SMT). In cases with no neck mass, USG was performed in 11 patients; seven had postural torticollis (POST), three had SMT and one had muscular torticollis (MT). Among 40 patients with follow-up, 36 had total resolution. There was negative correlation between the age at diagnosis and the recovery time, whereas the final outcome was not correlated with USG findings. However, the patients without positive findings in USG had earlier resolution (1 month vs 2.6 months, P=0.0008). The patients with SMT had earlier diagnosis and excellent outcomes. The patients with MT were delayed to diagnosis and had the longest time to resolve. Lastly, the patients with POST had delayed diagnoses, but they had excellent outcomes. Conclusion : Since the patients with delayed diagnoses, in despite of benign courses, may take a long time to resolve and rarely need surgical treatment, it is important to diagnose and treat early. This study showed that USG findings of the SCM may be used as predictive factors.