• Tuberculosis and Respiratory Diseases
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• v.44 no.3
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• pp.534-546
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• 1997

Background : The p53 and retinoblastoma(Rb) tumor suppressor genes are associated with the pathogenesis of several types of human cancer. Substantial proportion of the primary lung cancers or cell lines have been reported to have the p53 and/or the Rb gene mutations. But, so far there is no report on the analysis of the Rb gene polymorphism as one of the genetic susceptibility marker. This study was undertaken to establish the gene frequencies of the polymorphic genotypes of the p53 and Rb genes in Koreans to evaluate the possible involvement of these genotypes as a risk factor of lung cancer. Methods : In this study 145 controls without previous and present tumor history and 128 lung cancer patients were subjected to analysis. The two intragenic polymorphisms of the p53 gene(exon 4/ AccII, intron 6/MspI) and one intron 17/XbaI polymorphism of the Rb gene were analysed by the method of polymersae chain reaction- restriction fragment length polymorphisms(PCR-RFLPs). The genotype of the intron 3/16 bp repeat polymorphism of p53 was determined by PCR and direct gel electrophoresis. Results : There were no significant differences in the genotype distributions of the p53 gene between lung cancer patients and controls. But heterozygotes(Arg/Pro) of the exon 4/AccII polymorphisms were slightly over-represented than controls, especially in the Kreyberg type I cancer, which was known to be associated with smoking. The intron 3/16 bp duplication and the intron 6/MspI polymorphisms were in complete linkage disequilibrium. About 95% of the individuals were homozygotes of the common alleles both in the 16 duplication and MspI polymorphisms, and no differences were deteced in the genotype distributions between lung cancer patients and controls. Overall genotype distributions of the Rb gene polymorphisms between lung cancer patients and controls were not significantly different However, the genotype distributions in the Kreyberg type I cancer were significantly different from those of controls(p = 0.0297) or adenocarcinomas(p = 0.0008). It was noticeable that 73.4% of the patients with adenocarcinomas were heterozygotes(r1/r2) whereas 39.2% of the Kreyberg type I cancer were heterozygous at this polymorphisms. In the lung cancer patients, significant differences were also noted between the high dose smokers and low dose smokers including non-smokers(p = 0.0258). The relative risk to Kreyberg type I cancer was significantly reduced in the individuals with the genotype of r1/r2(odds ratio = 0.46, 95% C.I. = 0.25-0.86, p = 0.0124). The combined genotype distribution of the exon 4 AccII of the p53 and the intron 17 Rb gene polymorphisms in Kreyberg type I cancers were significantly different from dose of controls or adenocarcinomas. The highest odds ratio were observed in the individuals with the genotypes of Arg/Pro and r2/r2(odds ratio = 1.97,95% C.I. = 0.84-4.59) and lowest one was in the patients with Arg/Arg, r1/r2 genotype(odds ratio = 0.54, 95% C.I. = 0.25-1.14). Conclusion : The p53 and the Rb gene polymorphisms modulate the risk of smoking induced lung cancer development in Koeans. However, the exact mechanism of risk modulation by these polymorphism remains to be determined. For more discrete clarification of associations between specific genotypes and lung cancer risk, the evaluations of these polymorphisms in other ethnics and more number of patients will be needed.

• Tuberculosis and Respiratory Diseases
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• v.56 no.1
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• pp.51-66
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• 2004

Objectives : The matrix metalloproteinases (MMPs) that participate in the extracellular matrix metabolism play a important role in the progression of pulmonary fibrosis. The effects of the MMPs are regulated by several factors including Th-1 cytokines, $interferon-{\gamma}$ ($IFN-{\gamma}$). Up to now, $IFN-{\gamma}$ is known to inhibit pulmonary fibrosis, but little is known regarding the exact effect of $IFN-{\gamma}$ on the regulation of the MMPs. This study investigated the effects of $interferon-{\gamma}$ on the pulmonary fibrosis and the expression of the lung MMP-2,-9, TIMP-1,-2, and Th-2 cytokines in aa rat model of bleomycin induced pulmonary fibrosis. Materials and methods : Male, specific pathogen-free Sprague-Dawley rats were subjected to an intratracheal bleomycin instillation. The rats were randomized to a saline control, a bleomycin treated, and a bleomycin+$IFN-{\gamma}$ treated group. The bleomycin+$IFN-{\gamma}$ treated group was subjected to an intramuscular injection of $IFN-{\gamma}$ for 14 days. At 3, 7, 14, and 28 days after the bleomycin instillation, the rats were sacrificed and the lungs were harvested. In order to evaluate the effects of the $IFN-{\gamma}$ on lung fibrosis and inflammation, the lung hydroxyproline content, inflammation and fibrosis score were measured. Western blotting, zymography and reverse zymography were performed at 3, 7, 14, 28 days after bleomycin instillation in order to evaluate the MMP-2,-9, and TIMP-1,-2 expression level. ELISA was performed to determine the IL-4 and IL-13 level in a lung homogenate. Results : 1. 7 days after bleomycin instillation, inflammatory changes were more severe in the bleomycin+$IFN-{\gamma}$ group than the bleomycin group (bleomycin group : bleomycin+$IFN-{\gamma}$ group=$2.08{\pm}0.15:2.74{\pm}0.29$, P<0.05), but 28 days after bleomycin instillation, lung fibrosis was significantly reduced as a result of the $IFN-{\gamma}$ treatment (bleomycin group : bleomycin+$IFN-{\gamma}$ group=$3.94{\pm}0.43:2.64{\pm}0.13$, P<0.05). 2. 28 days after bleomycin instillation, the lung hydroxyproline content was significantly reduced as a result of $IFN-{\gamma}$ treatment (bleomycin group : bleomycin+$IFN-{\gamma}$ group=$294.04{\pm}31.73{\mu}g/g:194.92{\pm}15.51{\mu}g/g$, P<0.05). 3. Western blotting showed that the MMP-2 level was increased as a result of the bleomycin instillation and highest in the 14 days after bleomycin instillation. 4. In zymography, the active forms of MMP-2 were significantly increased as a result of the $IFN-{\gamma}$ treatment 3 days after the bleomycin instillation, bleomycin+$IFN-{\gamma}$ group (bleomycin group : bleomycin+$IFN-{\gamma}$ group=$209.63{\pm}7.60%:407.66{\pm}85.34%$, P<0.05), but 14 days after the bleomycin instillation, the active forms of MMP-2 were significantly reduced as a result of the $IFN-{\gamma}$ treatment (bleomycin group : bleomycin+$IFN-{\gamma}$ group=$159.36{\pm}20.93%:97.23{\pm}12.50%$, P<0.05). 5. The IL-4 levels were lower in the bleomycin and bleomycin+$IFN-{\gamma}$ groups but this was not significant, and the IL-13 levels showed no difference between the experiment groups. Conclusion : The author found that lung inflammation was increased in the early period but the pulmonary fibrosis was inhibited in the late stage as a result of $IFN-{\gamma}$. The inhibition of pulmonary fibrosis by $IFN-{\gamma}$ appeared to be associated with the inhibition of MMP-2 activation by $IFN-{\gamma}$. Further studies on the mechanism of the regulation of MMP-2 activation and the effects of MMP-2 activation on pulmonary fibrosis is warranted in the future.

• Clinical and Experimental Pediatrics
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• v.48 no.3
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• pp.306-309
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• 2005

Purpose : The aim of study is to investigate the clinical manifestations and the etiology of markedly-elevated serum alkaline phosphatase(ALP) levels in children without accompanying liver or bone disease. Methods : The serum ALP and other biochemical laboratory data of 4,989 patients attending the pediatric department of Ewha Womans University Mokdong Hospital between January 2002 to December 2002 were studied. The subjects' ages ranged from 4 months to 14 years. Among them, serum ALP levels were markedly elevated over 1,000 IU/L in 114 children. Among those 114 children, 97 children without liver or bone disease were reviewed retrospectively. Results : Of the 114 children with serum ALP activity of over 1,000 IU/L, 97(85.8 percent) children had neither liver or bone disease. The average ALP activity level was $1,539{\pm}948IU/L$. The male : female ratio was 1 : 0.7. Forty-four children(45.3 percent) were between 4 months to 1-year-old, 19 children(19.3 percent) were between 1- to 2-year-old. We observed a seasonal clustering of cases in during October with 10 cases(10.2 percent) and in during December with 11 cases(11.3 percent). The most common clinical presentation was respiratory tract infection in 25 cases(26.9 percent), and acute gastroenteritis in 17 cases(17.5 percent). Forty-four cases had follow-up ALP activity level, 1 month later and the activity decreased under 1,000 IU/L in 37 cases(84 percent). Conclusion : Hyperphosphatasemia is a benign and transient phenomenon. If there are no clinical and laboratory abnormalities of liver and bone, we suggest monitoring monitor the high serum ALP level 1 month later. If decreased, it seems that it is not necessary for specific examinations to exclude other diseases of malignant condition.

• Journal of Chest Surgery
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• v.41 no.3
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• pp.335-342
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• 2008

Background: Congenital cystic diseases of the lung are uncommon, and they share similar embryogenic and clinical characteristics. But they are sometimes vary widely in their presentation and severity. Therefore they are often difficult to make different diagnosis each other, and all require surgical treatment. Material and Method: From 1993 to 2006, 38 patients underwent surgical procedures under these diagnostic categories in the Depart. of Thoracic and. Cardiovascular Surgery, Busan-Paik Hospital, College of Medicine, Inje University. And we retrospectively reviewed these patients' charts for clinical presentations, surgical procedures, pathologic findings and postoperative morbidity and mortality. Result: There were 22 males and 16 females, ages ranged from 1 month after birth to 51 years and mean age was 20.8 years. The main symptoms were 19 fever, cough, sputum production due to recurrent infection, 7 dyspnea, 8 chest discomfort, 4 hemoptysis, but eight patients were asymptomatic. Computed tomography was chosen as diagnostic modalities and available for operation plan for all of patients. For all the cases, surgical resection were performed. Lobectomy was performed in 28 patients, simple excision (resection) in 8 patients, segmentectomy or wedge resection in 2 patients. There were 10 pulmonary sequestrations, 15 congenital cystic adenomatoid malformations (CCAM), 11 bronchogenic cysts, and 2 congenital lobar emphysemas. They all were confirmed by pathologic exams. The complications were 6 wound disruption or infection, 2 chylothorax, 1 ulnar neuropathy, but all of them were resolved uneventful. There was no persistent air leakage, respiratory failure, operative mortality and recurrence. Conclusion: We performed immediate surgical removal of congenital cystic lung lesions after diagnosis and obtained good results, so reported them with literature review.

• Journal of the Korean Society of Radiology
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• v.4 no.4
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• pp.17-24
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• 2010

Hemoptysis which is comparatively common symptom in respiratory disease patients is a clinical symptom which has high risk of death in spite of many curative means. Bronchial tube embolization is a very useful medical procedure when hemoptysis does not stop for treatment of internal medicine or surgery and in addition to the purpose of instant hemostasis, for patients for whom surgical operation is impossible or for the purpose of gaining time to improve the state of the patient before surgical operation. In relation to this, this study is to know of the usefulness of bronchial tube artery embolization. The objects were 60 persons for whom bronchial tube artery embolizations are conducted because of large hemoptysis occurred from March 2007 to December 2009 in J hospital. They had large hemoptysis of 400ml or more per day and 200ml or more at a time or though the quantity of hemoptysis was less than 400ml they did not respond to the treatment of internal medicine for 10 days or longer. The average age was 60.5 years and cause diseases were tuberculosis, bronchiectasis, and pneumonia and lung cancer. Embolus parts were Rt bronchial artery 19 examples, Lt bronchial artery, both bronchial artery, Rt Intercostobronchial artery, and they were the case where embolization for many blood vessels were simultaneously carried out. As embolus materials, PVA (conteour) and microcoil were used. In 76.6% of 60 persons of patients hemorrhage stopped with the lapse of time after the procedure and in 4 examples (6.6) re-embolization was carried out due to re-bleeding after the procedure. Bronchial tube artery embolization has high early success rate and effectively controls hemoptysis in the treatment of hemoptysis and is an effective emergency remedy for hemorrhage due to large hemoptysis and will be a good medical procedure which reduces death rate. In addition, primarily if it is conducted together with the treatment of internal medicine it will be, as an effective curative means for hemorrhage due to large hemoptysis, a good mediate radial rays medical procedure which reduces death rate.

• Pediatric Infection and Vaccine
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• v.23 no.1
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• pp.31-39
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• 2016

Purpose: The aim of this study was to investigate the prognostic factors for Pneumocystis jirovecii pneumonia (PCP) and to evaluate the influence of PCP prophylaxis in pediatric patients. Methods: From January 2002 to April 2015, patients aged <18 years with a diagnosis of confirmed PCP at our institute were reviewed retrospectively. Clinical characteristics and outcomes were compared according to the groups with or without PCP prophylaxis. Risk factors associated with PCP-related death were analyzed by logistic regression analysis. Results: During study period, a total of 24 patients were diagnosed with PCP by immunofluorescence assay and/or PCR. The median age of the patients was 5 years (range, 3 months-18 years) and 23 (96%) had immunocompromised conditions including hematologic disorders with or without hematopoietic stem cell transplantation (n=15), solid organ transplantation (n=4), and primary immune deficiency (n=4). Most common presenting symptoms were tachypnea and cough (92%, each). At the time of diagnosis, 79% (19/24) and 25% (6/24) suffered from respiratory failure and multi-organ dysfunction syndrome (MODS), respectively. Mechanical ventilation was required in 8 (33%) patients and 5 (21%) patients died of PCP. Multivariate analysis showed that MODS at initial presentation was an indicator of poor prognosis (OR, 17.1 [95% CI 1.13-257.67]; P=0.04). Compared to the patients without PCP prophylaxis, the frequency of MODS at diagnosis, need for mechanical ventilation and length of hospital days were significantly less common in the children who received PCP prophylaxis. Conclusions: MODS at presentation was a significant predictor for poor outcome and PCP prophylaxis could alleviate the clinical courses of pediatric PCP. Prospective study will be mandatory to determine the risk factors for development and deterioration of PCP in children.

• Journal of Chest Surgery
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• v.35 no.8
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• pp.573-579
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• 2002

Background: In this study, the role and the surgical outcome of the modified Blalock-Taussig shunt in the treatment of the infants with cyanotic complex congenital heart diseases were investigated. Material and Method: Over the last 12 years, 105 modified BT shunts were performed in 100 infants. Postoperative course, shunt patency rate, complications, mortality and its risk factors were reviewed restrospectively. Result: The mean age at operation was 43.0$\pm$36.6 days. Sex ratio was 60:40(M:F). The postoperative oxygen saturations were lowest after mean duration of 11 hours after the shunt procedure. The operative mortality was 8%(8) with 3 late deaths. Causes of operative death included failure of maintenance of minimum oxygenation during the procedure(2), immediate postoperative shunt occlusion(2), respiratory failure(2), low cardiac output due to heart failure and pericardial effusion(2) and sepsis(2). Late deaths resulted from acute cardiac arrest during the follow up cardiac catheterization, hypoxic myocardial failure, and arrhythmia. Year of surgery, shunt size, age at operation, and complexity of the anomalies were not the risk factors for mortality. Six month shunt patency rate was 97% and overall patency rate was 96%. Postoperative complications comprised of shunt occlusion(6), phrenic nerve palsy(3), and wound infection(2). Conclusion: We demonstrated that modified Blalock-Taussig shunt was a useful tool to palliate the infants with complex cyanotic heart disease in whom early complete repair was not feasible with acceptable mortality and patency rate. An adequate postoperative management and a meticulous surgical technique may be key factors for the better results.

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.37-47
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• 2002

Purpose: We reviewed the clinical manifestations, responsiveness to treatment, and prognosis in children with nephrotic syndrome. Methods: Medical records of 159 children with idiopathic nephrotic syndrome who were admitted to the pediatric department of Chonbuk National University Hospital from January 1979 to December 2000 w ere retrospectively reviewed. Results: There were 32 females and 127 males. The most common age group was between 3 and 5 years of age among the 159 children with nephrotic syndrome. Generalized edema ($75.5\%$), scrotal edema ($20.1\%$), upper respiratory infection ($19.5\%$), and ascites ($28.3\%$) were frequently observed. After the initial steroid therapy, diuresis occurred within tile first two weeks in 138 children, and proteinuria disappeared within the first two weeks in 105 children. Among 159 patients who received initial daily steroid therapy, 110 children were in complete remission, 29 children were in partial remission and 20 children were in poor response state. Hematuria, hypertension and elevated serum creatinine were more frequently observed in the partial and the poor response groups than in the complete remission group. Among 107 children who were followed up for more than one year, 78 children were in complete remission and 55 children were relapsed within the first one year after steroid therapy. Renal biopsy was undertaken in 76 children and 53 children had minimal change nephrotic syndronm. Conclusion: Our study showed that illost children with idiopathic nephrotic syndrome have a good responsiveness to steroid therapy and even most children show frequent relapse during 1st year after remission, long term prognosis is excellent.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.100-108
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• 2001

Purpose : We investigated the incidence and predisposing factors of pulmonary embolism in minimal change nephrotic syndrome(MCNS). Methods : Lung perfusion scan using 99mTC-MAA were done on 14 patients who were diagnosed to minimal change nephrotic syndrome. Group h: Five patients who had perfusion defects on scan, Group B; Nine patients who had no perfusion defect on scan. Between the two groups, the differences of platelet number, hematocrits, albumin, cholesterol, triglyceride, proteinuria were evaluated. Results : Five patients were found to have perfusion defect consistent with pulmonary embolism($35.7\%$). However, there were minimal or no respiratory symptoms and signs. In our laboratory studies, the mean proteinuria on admissions was $676{\pm}31\;mg/m2/hr$ in the group with pulmonary embolism, and $313{\pm}28\;mg/m2/hr$ in the group without pulmonary embolism. There were more severe proteinuria in group with pulmonary embolism(P<0.05). The mean platelet count at early stage of remission after steroid treatment was $746,600{\pm}280,000/mm3$ in the group with pulmonary embolism, $511,890{\pm}90,000/mm3$ in the group without pulmonary embolism. There were significant difference of platelet count between the two groups(P<0.01). In patients with pulmonary embolism, there were more higher and sustained increasement of platelet count. All cases of pulmonary embolism were treated with dipyridamole(5 mg/kg). In 4 cases the perfusion defects were improved in two weeks, however, one case showed persistent perfusion defect after 1 month. Conclusion : Our study suggested that pulmonry embolism might be one of tile major complications in childhood MCNS The occurrence rate was correlated with severity of proteinuria before treatment and sustained increasement of platelet counts in early remission state after steroid treatment. Therefore, the scintigraphic pulmonary perfusion study is mandatory in childhood MCNS, especially in the high risk patients, such as the patients with severe proteinuria and sustained increasement of platelet count. (J Korean Soc Pediatr Nephrol 2001;5 : 100-8)

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1248-1252
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• 2003

Purpose : Chest pain in the pediatric population is not rare and mostly benign. Causes of chest pain are diverse, and differential diagnosis is not easy. Chest pain in children is less likely to be cardiac in origin. Furthermore, chest pain in the pediatric population is rarely associated with life-threatening disease. This study was designed to evaluate children with chest pain and the usefulness of several diagnostic examinations. Methods : Between March 2001 and August 2002, 33 patients(15 boys and 18 girls, aged four to 15 years) presented with chronic chest pain. The records of these patients were reviewed. Chest radiography and electrocardiogram were performed in all patients. Cardiologic and gastrointestinal evaluations were carried out when considered necessary. Results : Chest pain was most common in the age group of 10 to 12 years old, and the four to six years old group. The most common diagnostic findings of chest pain were idiopathic(15 cases, 45.5 %), heart disease(9 cases, 27.3%), upper gastrointestinal disease(6 cases, 18.2%), respiratory disease (2 cases, 6%) and trauma(1 case, 3%). In children with abnormal results of cardiologic evaluation, these findings are not major etiologic categories of chest pain. Through history taking and physical examinations, six cases were evaluated concerning gastrointestinal disease and all of them showed gastrointestinal diseases(esophagitis, gastroesophageal reflux disease, nodular gastritis and chronic superficial gastritis). Conclusion : Chest pain is usually benign in children but the possibility of cardiovascular or gastrointestinal disease is considered. Careful history taking, physical examination and proper clinical examinations are usually required to find out the rare life-threatening causes of chest pain.