• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.683-692
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• 1996

Background: Although most of the patients with tuberculous pleural effusions completely reabsorbed their effusions and became asymptomatic within 2 to 4 months, later surgical procedures such as decortication is needed in some patients because of dyspnea caused by pleural loculations and thickening despite anti-tuberculous chemotherapy. It is obligatory to secure adequate drainage to prevent the development of complications. But, the best methods for treating loculated tuberculous pleural effusions remain debatable. Recent several reports revealed that intrapleural instillation of fibrinolytic agents is an effective adjunct in the management of complicated empyema and may reduce the need of surgery. Purpose : The effects of catheterization with intrapleural urokinase instillation were prospectively evaluated in the patients with septated tuberculous pleural effusion, and compared with other therapeutic effects of different modalities of therapy such as repeated thoracentesis and small-bored catheterization. Methods : Forty-eight patients diagnosed with tuberculous pleurisy were randomly separated into three groups; control group(n=13), catheter group(n=12), urokinase group(n=22). In urokinase group, dose of 100.000U urokinase was instilled into the pleural cavity via a percutaneous drainage catheter for complete drainage or total dose of 700,000U of urokinase. After two hours clamping, the catheter was opened and intermittently irrigated. The early and late effectiveness of therapies was assessed by radiographically and by measuring the volume of fluid drained from the catheter. Results : There was statistically significantly better result in the urokinase group in respect of frequency of catheterization, frequency of catheter obstruction and the duration of catheterization in early effectiveness(p < 0.05). There were no difference in radiologic improvement of follow-up in later phase chest X-ray between urokinase group and catheter group in later phase(p > 0.05). But there were more failure rates in control group especially honeycomb septa in pleural effusion sonographically than former two groups. And there were no complications of urokinase such as fever or hemorrhage. Conclusion : In the treatment of septated tuberculous pleurisy, there were better results in urokinase than those of catheterization alone in early effectiveness. And there was no difference in radiographic improvement between urokinase group and catheter group. Intrapleural instillation of urokinase is an effective and safe mode of treatment for septated tuberculous pleural effusions and alleviates the need for thoracotomy.

• Tuberculosis and Respiratory Diseases
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• v.41 no.3
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• pp.222-230
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• 1994

Background: Paraquat, a widely used herbicide, is extremely toxic, causing multiple organ failure in humans. Paraquat especially leads to irreversible progressive pulmonary fibrosis, which is related to oxygen free radicals. However, its biochemical mechanism is not clear. Natural mechanisms that prevent damage from oxygen free radicals include changes in glutathione level, G6PDH, superoxide dismutase(SOD), catalase, and glutathione peroxidase. The authors think catalase is closely related to paraquat toxicity in the lungs Method: The effects of 3-amino-1,2,4-triazole(aminotriazole), a catalase inhibitor, on mice administered with paraquat were investigated. We studied the effects of aminotriazole on the survival of mice administered with paraquat, by comparing life spans between the group to which paraquat had been administered and the group to which a combination of paraquat and aminotriazole had been administered. We measured glutathion level, glucose 6-phosphate dehydrogenase(G6PDH), superoxide dismutase(SOD), catalase, and glutathione peroxidase(GPx) in the lung tissue of 4 groups of mice: the control group, group A(aminotriazole injected), group B(paraquat administered), group C(paraquat and aminotriazole administered). Results: The mortality of mice administered with paraquat which were treated with aminotriazole was significantly increased compared with those of mice not treated with aminotriazole. Glutathione level in group B was decreased by 20%, a significant decrease compared with the control group. However, this level was not changed by the administration of aminotriazole(group C). The activity of G6PDH in all groups was not significantly changed compared with the control group. The activities of SOD, catalase, and glutathione peroxidase(GPx) in the lung tissue were significantly decreased by paraquat administration(group B); catalase showed the largest decrease. Catalase and GPX were significantly decreased by aminotriazole treatment in mice administered with paraquat but change in SOD activity was not significant(group C). Conclusion: Decrease in catalase activity by paraquat suggests that paraquat toxicity in the lungs is closely related to catalase activity. Paraquat toxicity in mice is enhanced by aminotriazole administration, and its result is related to the decrease of catalase activity rather than glutathione level in the lungs. Production of hydroxyl radicals, the most reactive oxygen metabolite, is accelerated due to increased hydrogen peroxide by catalase inhibition and the lung damage probably results from nonspecific tissue injury of hydroxyl radicals.

• Tuberculosis and Respiratory Diseases
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• v.62 no.2
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• pp.98-104
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• 2007

Background: Although there is an increasing incidence of Mycobacterium abscessus pulmonary disease in Korea, the optimal therapeutic regimen has not yet been established and there are no reports of the long-term treatment outcomes. This study examined the long-term treatment outcomes of M. abscessus pulmonary disease. Methods: Twenty-nine patients diagnosed with M. abscessus pulmonary according to the American Thoracic Society criteria and treated from January 1996 to December 2003 were enrolled in ghis study. The clinical characteristics, radiological findings, treatment outcome, and follow up data were analyzed retrospectively. Results: The mean age of the 29 patients was 56.1 (${\pm}13.6$) years and there was a female (22/29) dominance. The chest radiography revealed the nodular bronchiectatic type to be dominant (69%, 20/29). Twenty-seven (93.1%) were prescribed clarithromycin-containing regimens, and injectable drugs, mainly aminoglycosides, were included in the regimen of nineteen patients. The most predominant regimen (48.3%) consisted of clarithromycin and amikacin. The treatment success, failure, and default were achieved in 19(65.5%), 9(31.0%), and 1(3.4%), respectively. The median duration to culture conversion was 42 days (range 15-362) and the median duration of treatment in the success group was 543 days (range 176-1,160). An adjunctive surgical resection was performed in five patients, which resulted in treatment success in two patients. After the completion of treatment, nineteen patients were followed up for a median duration of 931 days (range 230-2,294). Only one (5.3%) patient relapsed 45 days after completing treatment. Conclusion: Treatment with clarithromycin-containing regimens resulted in a successful treatment in approximately two thirds of patients with M. abscessus pulmonary disease. The long-term relapse rate was also quite low.

• Tuberculosis and Respiratory Diseases
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• v.40 no.6
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• pp.677-682
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• 1993

Background: Massive hemoptysis is a major clinical problem with high mortality. Bronchial artery embolization is well accepted and widely used for treatment of massive and recurrent hemoptysis, especially in patients with chronic diffuse pulmonary disease who are poor candidates for surgery. We evaluated the therapeutic effect of transcatheter arterial embolization for immediate control and prevention of recurrent hemoptysis. Method: We reviewed 20 cases(M:F=13:7) of bronchial artery embolization for the management of massive hemoptysis from Jun 1989 to Aug 1992 retrospectively. Results: Underlying causes of hemoptysis were pulmonary tuberculosis(n=14), bronchiectasis(n=3), aspergilloma(n=2) and paragonimiasis(n=1). Embolization material was choosed randomly gelfoam(n=7) or Ivalon(n=11) and in 2 cases both were used simultaneously. Target arteries of embolization were bronchial artery only in 15 cases, non-bronchial systemic arteries with or without bronchial artery in 5 cases. After the arterial embolization, immediate cessation of hemoptysis was achieved in 17 cases(85%) and total recurrence rate including 3 cases of immediate treatment failure was 50%. Among recurrences 3 cases were achieved lobectomy, 1 case was expired by asphyxia due to massive hemoptysis and remained 6 were managed by medical conservative treatment with no further recurrence of hemoptysis during follow up periods. Conclusion: Bronchial artery embolization for treatment of massive or recurrent hemoptysis was effective in immediate bleeding control. Despite high recurrence rate the rebleeding after embolization was less severe and controllable by conservative management. Bronchial artery embolization is valuable as primary trial to massive hemoptysis.

• Tuberculosis and Respiratory Diseases
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• v.44 no.3
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• pp.639-648
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• 1997

Background : Arterial hypoxemia has been noted in patients with liver cirrhosis because of bronchial vessel dilatation. Cabenes et al. reported that bronchial hyperresponsiveness to the metacholine inhalation was observed in patients of left side heart failure, he suggested that one of the mechanism was bronchial vessel dilatation. We hypothesized that patients of liver cirrhosis might have bronchial hyperresponsiveness to metacholine inhalation due to portal hypertension. We evaluate the relationship between bronchial responsiveness and severity of liver cirrhosis, severity of portal hypertension. Methods : In the 22 patients of the liver cirrhosis with clinical portal hypertension, metacholine provocation test was done and determined $PC_{20}FEV1$. We classified liver cirrhosis according to Pugh-Child classification. Esophagogastroscopies were performed for the evaluation of the relationship between bronchial hyperresponsiveness and severity of esophageal varix. Results : In the 22 cases of the liver cirrhosis with clinical portal hypertension. The causes of liver cirrhosis, alcoholic hepatitis was 9 cases, hepatitis B virus was 12 cases, hepatitis C virus was 1 case, and 151 cases (68.18%) of total 22 cases were positive in metacholine provocation test. In positive cases. There was no significant relationship between $PC_{20}FEV1$ and severity of liver cirrhosis which were classified by Pugh-Child classification or severity of esophageal varix(p<0.05). Conclusion : we observed that bronchial responsiveness to metacholine increased in the patients of liver cirrhosis and there was no significant relationship between the severity of liver cirrhosis and the severity of esophageal varix.

• Tuberculosis and Respiratory Diseases
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• v.60 no.6
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• pp.625-630
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• 2006

Background: Pulmonary hypertension in COPD patients is the result of a direct effect of tobacco smoke on the intrapulmonary vessels with the abnormal production of the mediators that control vasoconstriction, vasodilatation, and vascular cell proliferation, which ultimately lead to aberrant vascular remodeling and physiology. COPD patients are prone to the developmint of an acute and chronic thromboembolism with an elevation of the plasma procoagulant and fibrinolytic markers However, the roles of the coagulation and fibrinolysis system on the right ventricular dysfunction in COPD patients are not well defined. We examined the alteration of the coagulation and fibrinolysis system in COPD patients according to the right ventricular function measured using cardiac multidetector computed tomography (MDCT). Methods: The right ventricular ejection fraction (RVEF) was measured using cardiac MDCT in 26 patients who were diagnosed with COPD according to the definition of the GOLD guideline. The plasma level of thrombin antithrombin (TAT) and plasminogen activator inhibitor (PAI)-1 were measured using an enzyme linked immunoassay. Results: The plasma TAT was markedly elevated in COPD patients ($10.5{\pm}19.8{\mu}g/L$) compared with those of the control ($3.4{\pm}2.5{\mu}g/L$) (p<0.01). However, the plasma PAI-1 in COPD patients ($29.6{\pm}20.7ng/mL$) was similar to that in the controls. The plasma TAT showed a significant inverse relationship with the RVEF measured by the cardiac MDCT in COPD patients (r=-0.645, p<0.01). However, the plasma PAI-1 did not show a relationship with the RVEF (r=0.022, p=0.92). Conclusion: These results suggest that the coagulation system in COPD patients is markedly activated, and that the plasma level of TAT might be a marker of a right ventricular dysfunction in COPD patients.

• Tuberculosis and Respiratory Diseases
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• v.61 no.1
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• pp.46-53
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• 2006

Background: Intra-abdominal hypertension (IAH) is defined as the presence of either an intra-abdominal pressure (IAP) ${\geq}12mmHg$ or an abdominal perfusion pressure (APP = mean arterial pressure - IAP) ${\leq}60mmHg$. Abdominal compartment syndrome (ACS) is defined as the presence of an IAP ${\geq}20mmHg$ together with organ failure. The purpose of this study was to investigate the prevalence of IAH and ACS on the day of admission and the effects of these maladies on the prognosis of critically ill patients in the ICU. Methods: At the day of admission to the ICU, the IAP was recorded by measuring the intravesicular pressure via a Foley catheter. The APACHE II and III scores were checked and SAPS II was also scored during the days the patients were in the ICU. The primary end point was the prevalence of IAH and ACS at the day of admission and the correlation between them with the 28-days mortality rate. The measurement of IAP continued until the 7th day or the day when the patient was transferred to the general ward before 7th day, unless the patient died or a Foley catheter was removed before 7th day. Patients were observed until death or the 28th day. Results: A total of 111 patients were enrolled. At the day of admission, the prevalence of IAH and ACS were 47.7% and 15.3%, respectively and the mean IAP was $15.1{\pm}8.5mmHg$. The rates of IAH for the survivor and the non-survivor groups were 56.5% and 71.4%, respectively, and these were not significantly different (p=0.593). Yet the rates of ACS between these two groups were significantly different (4/62, 6.5% vs. 13/49, 26.5%; Odds Ratio = 5.24, 95% CI = 1.58-17.30, p=0.004). Conclusion: In the present study, the prevalence of IAH was 47.7% and the prevalence of ACS was 15.3% on the day of admission. ACS was associated with a poor outcome for the critically ill patients in the ICU.

• Advances in pediatric surgery
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• v.17 no.2
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• pp.133-138
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• 2011

Extracorporeal membrane oxygenation (ECMO) has been utilized in congenital diaphragmatic hernia (CDH) patients with severe respiratory failure unresponsive to conventional medical treatment. We retrospectively reviewed 12 CDH patients who were treated using ECMO in our center between April 2008 and February 2011. The pre ECMO and on ECMO variables analyzed included gestational age, sex, birth weight, age at the time of ECMO cannulation, arterial blood gas analysis results, CDH location, timing of CDH repair operation, complications and survival. There were 9 boys and 3 girls. All patients were prenatally diagnosed. Mean gestational age was $38.8{\pm}1.7$ weeks and mean birth weight was $3031{\pm}499$ gram. Mean age at the time of ECMO cannulation was $29.9{\pm}28.9$ hours. There were 4 patients who survived. Survivors showed higher 5 min Apgar scores ($8.25{\pm}0.96$ vs. $7.00{\pm}1.20$, p=0.109), higher pre ECMO mean pH ($7.258 {\pm}0.830$ vs. $7.159{\pm}0.986$, p=0.073) and lower pre ECMO $PaCO_2$ ($48.2{\pm}7.9$ vs. $64.8{\pm}16.1$, p=0.109) without statistical significance. The hernia was located on the left side in 10 patients and the right side in 2 patients. The time interval from ECMO placement to operative repair was about 3~4 days in 5 early cases and around 24 in the remaining cases. There were 3 cases of post operative bleeding requiring re operation and 2 cases of abdominal compartment syndrome requiring abdominal fascia reopening. ECMO catheter reposition was required in 4 cases. Three cases of arterial or venous thrombosis were detected and improved with follow up. Our data suggests that ECMO therapy could save the lives of some neonates with CDH who can not be maintained on other treatment modalities. Protocolized management and accumulation of case experience might be valuable in improving outcomes for neonates with CDH treated with ECMO.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.102-108
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• 2016

Mitochondrial encephalopathy, lactic acidosis, and stroke-like episode (MELAS) syndrome is one of mitochondrial encephalopathy. As the early clinical manifestations can be variable, it is important to suspect the disease, especially in patients with multiple organ dysfunctions. A boy was diagnosed with epilepsy when he was 9 years old. Two years later, severe headache and blurred vision developed suddenly. On examination, left homonymous hemianopsia was detected with corresponding cerebral parenchymal lesions in right temporo-occipito-parietal areas. MELAS syndrome was confirmed by genetic test, which showed m.3243 A>G mitochondrial DNA mutation. Multivitamins including coenzyme Q10 were added to anticonvulsant. He experienced 4 more events of stroke-like episodes over 5 years, but he is able to perform normal daily activities. A 13-year-old boy was brought to the hospital due to suddenly developed respiratory arrest and asystole associated with pneumonia. Past medical history revealed that he had multiple medical problems such as epilepsy, failure-to-thrive, optic atrophy, and deafness. He has been on valproic acid as an anticonvulsant which was prescribed from local clinic. He recovered after the resuscitation, but his cognition and motor function were severely damaged. He became bed-ridden. He was diagnosed with MELAS syndrome by brain MRI, muscle biopsy, and clinical features. Genetic test did not reveal any mitochondrial gene mutation. Four years later, he expired due to suddenly developed severe metabolic acidosis combined with hyperglycemic hyperosmolar nonketotic coma. The clinical features of MELAS syndrome are variable. Early diagnosis before the presentation to the grave clinical course may be important for the better clinical outcome.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.1
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• pp.29-35
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• 2020

Joubert syndrome (JS) is a rare genetic disorder that is characterized by ataxia, hypotonia, developmental delay, respiratory abnormalities such as apnea-hyperpnea, and abnormal eye movements. The pathognomonic diagnostic finding is the "molar tooth sign" (MTS) on brain magnetic resonance imaging (MRI), described as cerebellar vermis hypoplasia or dysplasia, thick and horizontally oriented superior cerebellar peduncles, and an abnormally deep interpeduncular fossa. JS is characterized by genetic heterogeneity: pathogenic variants in over 30 genes have been identified to date. The CEP290 protein, which is on chromosome 12q21.3, is most frequently mutated in patients with JS, especially with renal involvement. Here, we report a case of JS in a 14-year-old male patient with end-stage renal disease. To the best of our knowledge, this is the first Korean report of a patient with JS due to CEP290 mutation (c.6012-12T> A) whose diagnosis was confirmed after repetitive MRI. We suggest consultation with an experienced neuro-radiologist and follow-up MRI studies to detect a "hidden" MTS if clinical findings suggest a diagnosis of JS. Furthermore, even in the absence of an MTS, whole exome sequencing should be considered.