• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.2
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• pp.85-93
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• 2012

Purpose: Developmental delay is caused by very diverse etiologic diseases. Most chronic disorders has some influence on development. Chronic or acute disorders of CNS are main etiologic diseases of developmental delay. Up to now, over 60 diseases are included in organic acidopathies and most of them causes acute or chronic recurrent CNS damage and developmental delay. We have done this study to find out the importance of organic acidopathies causing developmental delay in Korean childhood and adolescent patients. Method: Retrograde analysis for 738 patients with developmental delay whose clinical informations are available and have done urine organic acid analysis for 5 years period, between Jan. 1st 2007 to Dec. 31th 2011. Statistical analysis was done with Student's t test using SPSS. Result: Out of 738 patients, 340 patients (46.1%) showed abnormalities on urine organic acid analysis. The most frequent disease was mitochondrial respiratory chain disorders (MRCD) (253, 34.3%), followed by ketolytic defects(39, 5.3%), 3-hydroxyisobutyric aciduria (26, 3.5%), glutaric aciduria type II (8, 1.1%), pyruvate dehydrogenase deficiency (3, 0.4%), 3-methylglutaric aciduria (2, 0.3%), glutaric aciduria type I (2, 0.3%), ethylmalonic aciduria (1, 0.15%), methylmalonic aciduria (1, 0.15%), HMG-CoA lyase deficiency (1, 0.15%), 3-methylcrotonylglycinuria (1, 0.15%), fatty acid oxidation disorders(1, 0.15%) and FAOD (1, 0.15%). Conclusion: Mitochondrial disorders are most frequent etiologic disease on all age group, followed by ketolytic defects and various organic acidopathies. The number and diversities of organic acidopathies emphasize meticulous evaluation of basic routine laboratory examinations and organic acid analysis with initial sample on every developmental patient.

• Tuberculosis and Respiratory Diseases
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• v.46 no.6
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• pp.846-855
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• 1999

Background : Secondary pulmonary hypertension is an important final endpoint in patients with chronic hypoxic lung disease, accompanied by deterioration of pulmonary hemodynamics. The clinical diagnosis of pulmonary hypertension and/or cor pulmonale could be difficult, and simple noninvasive evaluation of pulmonary artery pressures has been an relevant clinical challenge for many years. Doppler echocardiography might to be a more reliable method for evaluating pulmonary hemodynamics in such patients in terms of the accuracy, reproducibility and easiness for obtaining an appropriate echocardiographic window than M-mode echocardiography. The aim of this study was to assess echocardiographic parameters associated with pulmonary arterial hypertension, defined by increasing right ventricular systolic pressure(RVSP), calculated from trans-tricuspid gradient in patients with chronic hypoxic lungs. Method : We examined 19 patients with chronic hypoxic lung disease, suspected pulmonary hypertension under the clinical guidelines by two dimensional echocardiography via the left parasternal and subcostal approach in a supine position. Doppler echocardiography measured RVSP from tricuspid regurgitant velocity in continuous wave with 2.5MHz transducer and acceleration time(AT) on right ventricular outflow tract in pulsed wave for the estimation of pulmonary arterial pressure. Results : On echocardiography, moderate to severe degree of pulmonary arterial hypertension was defined as RVSP more than 40mmHg, presenting tricuspid regurgitation. Increased right ventricular endsystolic diameter and shortened AT were noted in the increased RVSP group. Increased RVSP was correlated negatively with the shortening of AT. Other clinical data, including pulmonary functional parameters, arterial blood gas analysis and M mode echocardiographic parameters were not changed significantly with the increased RVSP. Conclusion : These findings suggest that shortened AT on pulsed doppler can be useful when quantifying pulmonary arterial pressure with increased RVSP in patients with chronic lung disease with hypoxemia. Doppler echocardiography in pulmonary hypertension of chronic hypoxic lungs is an useful option, based on noninvasiveness under routine clinical practice.

• Tuberculosis and Respiratory Diseases
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• v.54 no.4
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• pp.378-385
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• 2003

Background : Wheezing is an important clue in the diagnosis of asthma. Previously, a Korean National asthma survey used a written questionnaire, containing the question, "Have you ever experienced a breathing sound-like 'sack-sack' or a flute sound (the Korean description for wheezing) during the last 12 months?" The response to this question showed a large discrepancy between the prevalence of wheezing and physician diagnosed asthma. This might have resulted partly from a misunderstanding of the question, due to an inadequate description for wheezing. This study was aimed at finding how well the layman understands the term "wheezing" when described as a breathing sound-like 'sack-sack', a whistle or a flute. Subjects and method : Sixty subjects, without experience of wheezing(group I), and 45 subjects, with chronic cough alleging wheezing(group II), were recruited from the Hallym University's Sacred Heart Hospital, in Anyang, Korea. Four different breathing sounds; vesicular, wheezing, tracheobronchial and crackle, were played for the subjects, without any experience with wheezing, and they were asked "which sound is most like that you would imagine when asked about a breathing sound-like 'sack-sack', a whistle or a flute?" This was followed by replaying the true wheezing sound, and then a global assessment was requested for the concordance between the real wheezing sound and the imagined wheezing sound. The wheezing sound was played for those subjects alleging wheezing, and they were asked, "have you really experienced that sound". Results : Only 46.7% of group I answered correctly, with 13.3% choosing the vesicular sound, 16.7% the tracheobronchial sound, 5.0% the crackle and 18.3% failed to answer. The concordance between their imagined wheezing and the real sound was $69.3{\pm}22.4%(mean{\pm}S.D.)$. 77.8% of group II recognized the correct sound as the one they had experienced. Conclusions : Language is not sufficient to the layman for describing natural sounds, such as wheezing.

• Tuberculosis and Respiratory Diseases
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• v.59 no.5
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• pp.497-503
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• 2005

Backgrounds : The exacerbations of asthma and chronic obstructive pulmonary disease (COPD) have been suggested to be associated with respiratory tract viral infections (RTVIs). However, the rates of virus detection in previous studies have been quite variable, with lower rates for the exacerbation of COPD. Therefore, the virus detection of patients with exacerbation of asthma and COPD were investigated. Methods : 20 and 24 patients with exacerbation of asthma and COPD, respectively, were enrolled. Nasal and sputum samples were taken, and polymerase chain reaction (PCR) for rhinovirus and coronavirus and virus culture for influenza A, B, RSV and parainfluenza virus performed. Results : The mean $FEV_1/FVC$ in the exacerbation of asthma and COPD patients were 1.9/2.9 L (65.5%) and 1.1/2.6 L (42.3%), respectively. Respiratory virus was detected in 13 (65%) patients with exacerbation of asthma and rhinovirus was detected in 9. Coronavirus, influenza A, RSV and parainfluenza virus were detected in 2, 2, 1 and 1 patients with asthma. Among patients with exacerbation of COPD, a virus was detected in 14 (58.3%) patients, with rhinovirus, coronavirus and influenza A detected in 10, 3 and 4, respectively. Conclusions : This study suggested that RTVIs may have a role in the exacerbation of COPD as well as asthma.

• Journal of Chest Surgery
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• v.30 no.11
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• pp.1077-1082
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• 1997

To assess the early results, risk factors and optimal timing for coronary artery bypass graft surgery(CABG) after an acute myocardial infarction(AMI), we reviewed our 19 patients who underwent CABG within 30 days after AMI, between June 1994 and October 1996. This study excluded 1 patient whose diagnosis was AMI with ventricular septal rupture. 14 of the patients were male and 5 were female. Their ages ranged from 41 to 77 years(mean age, 60.6$\pm$ 10.4 years), and the amount of time between AMI and CABG ranged from 8 hours to 24 days(mean time, 10.6$\pm$6.4 days). There were 11 anteroseptal infarctions and 8 inferior wall infarctions. 11 patients had trsnsmural infarctions and 8 had subendocardial infarctions. Indications of operations were p imary revascularization and postinfarction angina. Three patients required preoperative intra-aortic balloon pump(IABP) support, and 4 additional patients required IABP to be separated from cardiopulmonary bypass. An average of 3.6 $\pm$ 0.6 vessels per patient were bypassed. The early mortality rate for these 19 patients was 5.3% and late mortality rate was 5.5%, 1-year and 2-year actuarial survival rates were 89.5% Univariate analysis of mortality showed that an ejection fraction less than 30% and intraopretative IABP supports were associated with risk factors(p value=0.018 and 0.015 respectively). Age, sex, time to CABG, emergency operations, types and locations of infarctions were not significant. Although our studies have weak p.oints in that there was only a small number of patients and the lack of long-term results, we could conclude that early myocardial revascularization is relatively safe after AMI for those individuals with an ejection fraction greater than 30%.

• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.740-745
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• 2007

Purpose : There is a dominant opinion that in vitro fertilization (IVF) leads to an increased incidence of twins, low birth weight (LBW) infants, prematurity and mortality. On the other hand, technical development of IVF and improvement of neonatal intensive care have increased the survival rate of neonates. The purpose of this study was to verify the tendency by comparing the clinical aspects of IVF and spontaneously conceived twins, and to establish methods to increase the survival rate of neonates after IVF. Methods : Retrospective reviews were performed on all twin infants who were admitted to the nursery and NICU at Kangnam Sacred Heart Hospital, Hallym University from January 1, 2000 to December 31, 2006. Medical records of IVF twins (study group, n=92) and spontaneously conceived twins (control group, n=265) were analyzed and compared. Neonatal outcomes and complications, as well as obstetric outcomes, were analyzed and compared. Results : Mean gestational age and birth weight of the study group ($34.6{\pm}3.5$ weeks, $2,203.9{\pm}617.2g$) were considerably lower than those of the control group ($36.3{\pm}2.4$ weeks, $2,367.0{\pm}517.9g$). The frequency of prematurity less than 37 weeks (68.5% vs 51.3%) and extremely LBW (15.2% vs 6.4%) were also significantly higher in the study group. Other neonatal outcomes were all insignificant. The obstetric characteristics, maternal age ($32.6{\pm}3.3$ years vs $30.3{\pm}3.9$ years) and the frequency of cesarean delivery(95.7% vs 79.9%) were significantly higher in the study group. Other obstetric outcomes were insignificant except for the frequency of incompetent internal os of cervix (36.2% vs 3.6%) and cerclage operation (38.3% vs 4.3%). Conclusion : Based on the above results, clinical outcomes of twin infants will be further improved by careful attention and thorough antenatal care of the IVF twins.

• Tuberculosis and Respiratory Diseases
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• v.51 no.6
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• pp.570-578
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• 2001

Background: With cases of chronic obstructive pulmonary disease(COPD), weight loss and low body weight have been found to correlate with increased mortality and poor prognosis. Therefore, nutritional aspects are an important part of the treatment in cases of COPD. In Korea, there is only limited data available for the changes of resting pulmonary function in relation to nutritional status. This study was carried out to investigate the differences of resting pulmonary function in relation to the nutritional status of patients with COPD. Method : 83 stable patients, with moderate to severe COPD, were clinically assessed for their nutritional status and resting pulmonary function. The patients' nutritional status was evaluated by body weight and fat-free mass (FFM), which was assessed by bioelectrical impedance analysis. According to their nutritional status, the 83 patients were divided into two groups, designated as the depleted, and non-depleted, groups. Result : Of the 83 patients, 31% were characterized by body weight loss and depletion of FFM, whereas 28% had either weight loss or depleted FFM. In the depleted group, significantly lower peak expiratory flow rate(p<0.05) and Kco(p<0.01), but significantly higher airway resistance(Raw, p<0.05) were noted. There was no difference for the non-depleted group in forced expiratory volume at one second, residual volume, inspiratory vital capacity, or total lung capacity. Maximal inspiratory pressure($P_{Imax}$) was also significantly lower in the depleted group(p<0.05). Conclusion : We conclude, from our clinical studies, that nutritional depletion is significantly associated with the change in resting pulmonary function for patients with moderate to severe COPD.

• Tuberculosis and Respiratory Diseases
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• v.50 no.1
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• pp.106-116
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• 2001

Background : Pulmonary thromboembolism is relatively frequent and potentially fatal. However, it is commonly misdiagnosed. The incidence of pulmonary thromboembolism is not decreasing despite advances in diagnosis and effective prophylatic measures. Its potential for significant sequela necessitates a prompt diagnosis and treatment. Unfortunately, there are many difficulties and problems regarding accurate diagnosis. There is a low prevalence of deep vein thrombosis and pulmonary thromboembolism in Korea and only few reports on this subject are available. Method : The clinical features of 36 patients, who were diagnosed with pulmonary thromboembolism at the Korea University medical center, were reviewed. Results : 1) There was no significant difference in prevalence between men an women, and the mean age was 50.9 years in men 59.2 years in women. 2) The frequent causes of pulmonary thromboembolism were malignancies (22.2%), surgery (22.2%), and heart disease(8.2%). Specific causes were not identified in 33.3%. 3) The most common symptom was dyspnea(72.2%), and the most common sign was tachypnea(61.1%). 4) The EKG findings were normal in 28.6%, an S1Q3T3 pulmonale pattern in 25.7%, ST or QRS changes in others. 5) The chest X-ray findings indicated pulmonary infiltration in 37.5%, cardiomegaly in 15.6%, pleural effusion in 12.5%, and normal in 27.8%. The perfusion lung scan showed a high probability in 66.7%, and intermediate or low probability in 33.3%. 6) The pulmonary arterial pressure(PAP) in the high probability groups was 57.9mmHg with a higher mortality rate(35%). Conclusion : Pulmonary thromboembolism is not uncommon in Korea and its clinical features do not differ greatly from thase reported in the literature. When pulmonary thromboemblism of unknown causes are diagnosed, a search for an occult malignancy is recommended. Rapid diagnosis and treatment are achieved when thromboemblism is suspected.

• Tuberculosis and Respiratory Diseases
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• v.61 no.5
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• pp.463-472
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• 2006

Background: Since video-assisted thoracoscopic surgery (VATS) was introduced as a new treatment modality for empyema thoracis, numerous reports have suggested that VATS is a more effective treatment method than the traditional methods that mainly use antibiotics and drainage apparatus. However, no confirmative evidence of the superiority of VATS over the traditional method has been provided yet. Methods: We attempted to evaluate the efficacy of VATS for the treatment of empyema thoracis by reviewing past medical records and simple chest films. First, we divided the patients into two groups based on the treatment method: group A of 15 patients who were treated with the traditional method between January 2001 and December 2003, and group B of 9 patients who were treated with VATS between December 2003 and August 2006. The final outcomes used in this study were the number of days of hospital stay, chest tube duration, leukocytosis duration, febrile duration, and intravenous antibiotics usage duration. In addition, radiological improvements were compared. Results: The mean age(${\pm}$standard deviation) of 11 men and 4 women in group A was $58.2{\pm}15.7$ years, and of 9 men and 2 women in group B was $51.6{\pm}9.5$ years. Group B had a significantly shorter hospital stay ($16.6{\pm}7.4$ vs. $33.7{\pm}22.6$ days; p=0.014), shorter chest tube duration ($10.5{\pm}5.7$ vs. $19.5{\pm}14.4$ days; p=0.039), shorter leukocytosis duration ($6.7{\pm}6.5$ vs. $18.8{\pm}13.2$ days; p=0.008), shorter febrile duration ($0.8{\pm}1.8$ vs. $9.4{\pm}9.2$ days; p=0.004), and shorter duration of intravenous antibiotics usage ($14.9{\pm}6.4$ vs. $25.4{\pm}13.9$ days; p=0.018). However, radiological improvements did not show any statistical differences. Conclusion: Early application of VATS for empyema thoracis treatment reduced hospital stay, thoracostomy tube duration, leukocytosis duration, febrile duration, and antibiotics usage duration in comparison with the traditional methods. The early performing of VATS might be an effective treatment modality for empyema thoracis.

• Tuberculosis and Respiratory Diseases
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• v.61 no.1
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• pp.13-19
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• 2006

Background: Acinetobacter baumannii has emerged as an important nosocomial pathogen worldwide. The incidence of these infections has recently begun to increase. The mortality rate associated with these infections is high (bacteremia; 52%, pneumonia: 23%~73%) and multidrug resistance has been reported. For the effective control of multidrug-resistant Acinetobacter baumannii(MDR-AB), the impact of these organisms in clinical practice should be determined. This study compared the clinical characteristics, mortality and morbidity of Acinetobacter nosocomial pneumonia between MDR strain and non-MDR strain. Methods: From Jan. 1, 2002 to Nov. 1. 2004, 47 adult patients with Acinetobacter nosocomial pneumonia in Chuncheon Sacred Heart Hospital were recruited and analyzed retrospectively. MDR-AB was defined as showing in vitro resistance to all commercially available antibiotics against A. baumannii. Results: There were 47 patients with Acinetobacter nosocomial pneumonia. MDR-AB and non MDR-AB was the cause of the pneumonia in 17 and 30 patients, respectively. Mean age of the former was $69{\pm}11$ years old and the latter was $70{\pm}13$ years old. The mean APCHE II score, ICU days and mortality were not different between the two groups ($16.1{\pm}5.4$ vs. $14.9{\pm}4.8$, P=0.43, $25.1{\pm}13.6$ vs. $39.1{\pm}31.0$, P=0.2, 58.8% vs. 40%, P=0.21). Conclusion: There are no significant differences in mortality and morbidity between MDR and non-MDR Acinetobacter baumannii. The mortality of the two groups is surprisingly high, therefore proper infection control practices are essential.