• Tuberculosis and Respiratory Diseases
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• v.43 no.3
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• pp.377-387
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• 1996

Background : In 1980, WHO made a definition in which the term "impairment" as applied to the respiratory system is used to describe loss of lung function, "disability" the resulting diminution in exercise capacity. The measurement of pulmonary function during exercise would give us information about overall functional capacity and respiratory performance that would be lacking in tests performed at rest. We conducted this study to investigate the role of resting pulmonary function test and exercise test for assessing impairment/disability in patients with chronic airflow obstruction(CAO). Method : We studied 19 patients with CAO. The spirometry and body plethysmograph were performed in stable condition. And then patients performed a progressive incremental exercise test to a symptom-limited maximum using cycle ergometer. Patients were divided in two groups, severe and non-severe impairment, according to the resting PFTs and compaired each other. A patient was considered to be severely impaired if FVC < 50 %, FEV1 < 40 % or FEV1/FVC < 40 %. Results : 1) The airway obstruction and hypoxemia of severe impairment group were more severe and exercise performance was markedly reduced compairing to non-severe impairment group. 2) The severe impairment group showed ventilatory limitation during exercise test and the limiting symptomes ware dyspnea in 9/10 patients. 3) The impairment and disability of the patients with tuberculous destructed lung were most marked in patients with CAO. 4) The FEV1 was the most prevalent criterion for the determination of severe impairment based on resting PFTs and was the valuable best correlated to V02max(r=0.81, p < 0.001). 5) The sensitivity of exercise limits for predicting severe disability according to resting PFTs was 80 % and specificity 89 %. Conclusion : In patients with severe CAO, FEV1 is a good predictive of exercise performance and impairment measured by resting PFTs can predict a disability by exercise test.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.24-33
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• 2016

Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.

• Tuberculosis and Respiratory Diseases
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• v.65 no.3
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• pp.191-197
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• 2008

Background: Due to the irreversible nature of chronic obstructive pulmonary disease (COPD), the treatment aim in patients with COPD is not to cure but to reduce the symptoms, increase lung function, and improve the quality of life. It has been suggested that depression is a common emotional disturbance in patients with COPD who are faced with a major physical impairment and embarrassing symptoms. This study evaluated the prevalence and risk factors of depression in patients with chronic obstructive pulmonary disease. Methods: A total of 59 patients with a registered diagnosis of chronic obstructive pulmonary disease were selected. Depression was assessed using the Centers for Epidemiologic Studies Depression (CES-D) scale. The quality of life was assessed using the Korean version of the St. George's Respiratory Questionnaire. Results: The prevalence of depression was 17.0%. In the correlation model, the interaction of the $FEV_1%$ over predicted value and SGRQ score(symptom, activity, impact, overall score) was statistically significant. The interaction of the $FEV_1%$ over predicted value and depression scale(CES-D) was also statistically significant. There was a positive correlation between the SGRQ scores(symptom, activity, impact, overall score) and the depression scale. Conclusion: The prevalence of depression in patients with chronic obstructive pulmonary disease is relatively high. The pulmonary function and the living standards were found to be significant risk factors for depression.

• Tuberculosis and Respiratory Diseases
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• v.42 no.6
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• pp.888-899
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• 1995

Background: Topical inhaled steroids, budesonide(Bu) and beclomethasone dipropionate (BOP), are now established as effective drugs in the management of chronic asthma. These drugs have high topical anti-inflammatory effect with low systemic activity. This study was performed to determine the effects of two inhaled corticosteroids, Bu and BOP, on the adrenocortical supression in 44 patients with bronchial asthma or chronic obstructive pulmonary disease. Methods: The adrenocortical function was assessed by measurement of serum cortisol concentration at 8 o'clock in morning and free cortisol in 24-hour urine collection at interval in 44 patients. No steroid was administered during the pretreatment period of 10 days and the final 6 days of the study. Each subject inhaled BOP or Bu, in daily doses of 800 or 1,600 micrograms for 12 days. The dose was delivered by metered dose inhaler (MDI) or diskhaler or large spacing device attached to MDI. Results: The levels of serum cortisol and 24-hour urinary free cortisol were decreased during the treatment period in patients inhaled Bu delivered by MDI in daily doses of 800 and 1,600 micrograms. In contrast, serum cortisol level was decreased on 6 and 12th day of treatment period in patients with BDP diskhaler in daily doses of 800 micrograms. In daily doses of 1,600 micrograms, the serum cortisol and 24hour urine free cortisol levels were decreased on 6, 9 and 12th day of treatment period in patients with BDP disk haler. The serum cortisol and 24-hour urinary free cortisol levels were not significantly decreased during the treatment period in patients inhaled Bu delivered by large spacing device attached to a MDI. Conclusion: These results showed that 1) the endogenous cortisol secretion was suppressed after inhalation of BDP and Bu in daily doses of 800 and 1,600micrograms, 2) Bu with MDI suppressed the adrenocortical function more than BDP with diskhaler, in daily doses of 1600 micrograms. and 3)large spacing device attached to a MDI might decrease the risk of suppression in the hypothalamic -pituitary- adrenal axis.

• Tuberculosis and Respiratory Diseases
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• v.47 no.5
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• pp.669-680
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• 1999

Background: Pulmonary thromboembolism(PTE) is a life threatening disease that needs early diagnosis. Spiral CT angiography depict thromboemboli in the central pulmonary vessels with greater than 90% sensitivity and specificity, which approaches the results of pulmonary angiography in the Prospective Investigation of Pulmonary Embolism Diagnosis(PIOPED) study. This study was performed to evaluate the findings and the diagnostic value(clinical utility) of the spiral CT angiography with 2D image(multiplanar reformation) and 3D images(Shaded surface display, Minimal intensity projection) in the pulmonary thromboembolism. Methods: We retrospectively analysed spiral CT angiography and pulmonary angiography, lung scan and clinical recordings of 20 patients who had PTE diagnosed by spiral CT angiography(n=19 cases) or pulmonary angiography(n=l case) from September 1997 to August 1998. Among 20 patients who had underwent spiral CT angiography, 14 patients could be performed lung perfusion scan at the same time. We analyzed the vascular and parenchymal change in spiral CT angiogram. Results: Anatomical distribution of PTE was as follows: 1) left lung(n= 103)

• Journal of Chest Surgery
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• v.31 no.9
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• pp.877-883
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• 1998

Background: Cardiopulmonary bypass(CPB), a standard adjunct for open heart surgery, can also play an important role in treating patients with noncardiac diseases. Material and Method: We report a collective analysis of noncardiac applications of cardiopulmonary bypass experienced at Seoul National University Hospital from 1969 to 1996. Out of a total of 20 patients, 8 were treated for membranous obstruction of inferior vena cava(MOVC), 5 for malignant melanoma, 3 for pulmonary embolism, 1 for double lung transplantation, 1 for intracranial giant aneurysm(GA), 1 for renal cell carcinoma(RC), and 1 for liposarcoma. CPB was used to induce profound hypothermia with circulatory arrest in 6 patients(MOVC 4, GA 1, RC 1). Result: CPB time was 113 mins on average for MOVC, 161 mins for GA, and 156 mins for RC, while the lowest rectal temperature was 26$^{\circ}C$ on average in MOVC, and 19$^{\circ}C$ in GA and RC. Postoperative recovery was good in all MOVC patients. The patient with GA, who underwent reoperation for the removal of hematoma, died 14 days postoperatively. The patient with RC recovered from the operation in a good condition but died from metastatic spread 6 months later. CPB was instituted for pulmonary embolectomy in 3 patients, in whom postoperative courses were uneventful, except in 1 patient who showed transient neurologic symptoms. CPB was used in a patient with double-lung transplantation for hemodynamic and ventilatory support. The patient was weaned successfully from CPB but died from low output and septicemia 19 days postoperatively. CPB without circulatory arrest was used to treat in 4 patients with MOVC. These patients showed good postoperative courses. CPB was used to administer high concentrations of chemotherapeutic agents to the extremities in 6 patients(malignant melanoma 5, recurrent liposarcoma 1). CPB time was 153 mins on average. No complications such as edema and neurologic disability were found. Conclusion: Although CPB has a limited indication in noncardiac diseases, if properly applied, it can be a very useful adjunct in a variety of surgical cases.

• Tuberculosis and Respiratory Diseases
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• v.55 no.1
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• pp.88-97
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• 2003

Background : Although smoking is a major cause of chronic obstructive pulmonary disease (COPD), only 10-20% of cigarette smokers develop symptomatic COPD, which suggests the presence of genetic susceptibility. This genetic susceptibility to COPD might depend on variations in the activities of the enzyme that detoxify hazardous chemical products, such as microsomal epoxide hydrolase (mEPHX) and glutathione-S transferase M1 subunit (GSTM1) genes. Methods : The genotypes of 58 patients with COPD, and 79 age matched control subjects, were determined by a polymerase chain reaction, followed by restriction fragment length polymorphism (PCR-RFLP) for the mEPHX, and multiplex PCR for the GSTM1. Results : GSTM1 was deleted in 53.3% of the subjects. There was no difference in GSTM1 deletion rates between the COPD patients (32/58, 55.2%) and the control subjects (41/79, 51.9%). The combination patterns of two polymorphisms of mEPHX showed slow enzyme activity in 29(21.2%), normal in 73(53.3%) and fast in 32(23.4%). The COPD group (7/57, 12.3%) showed a significantly lower incidence of slow enzyme activity compared to the control subjects (22/77, 28.6%, p<0.05). However, when the COPD and control groups were compared with smokers only, there were no significant differences in the genotypes of GSTM1 and mEPHX. Conclusion : The genotypes of GSTM1 and mEPHX were not significant risk factors of COPD in this cohort of study.

• Tuberculosis and Respiratory Diseases
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• v.54 no.1
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• pp.22-32
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• 2003

Background : Sputum smears for acid-fast bacilli(AFB) examined microscopically is the most important diagnostic test for pulmonary tuberculosis. However, the AFB observed on the smear may represent either M. tuberculosis or nontuberculous mycobacteria (NTM). This study examined the recovery rate of NTM from the AFB smear-positive sputum specimens in a tertiary hospital in Korea. Materials and Methods : This study analyzed the results for the 1,889 AFB smear-positive and culture-positive sputum specimens collected from 844 patients from July, 1997 to December, 2001. Results : The 1,889 sputum specimens collected from 844 patients tested positive on both microscopy and culture during the 4.5 years. The NTM were recovered from 10.3% (195/1,889) of the smear-positive sputum specimens and 11.0% (93/844) of patients with smear-positive sputum. The NTM were isolated more than two times in 44.1% (41/93) of the patients from whom the NTM was recovered. Trends of the recovery rate of the NTM from the AFB smear-positive sputum specimens were increasing from 6.5%(17/262) in the latter half of 1997 to 17.8%(36/202) in the latter half of 2001 (p<0.001, test for trend). Conclusions : These results suggest that some patients with AFB smear-positive sputum have NTM pulmonary disease rather than pulmonary tuberculosis in Korea.