• Childhood Kidney Diseases
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• v.5 no.2
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• pp.109-116
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• 2001

Purpose Long- term use of steroid, cyclophosphamide and cyclosporin, which are frequently used in the therapy of SDNS, might cause severe side effects. Recently, the immune-modulator levamisole has been tried as a substitute therapy and it has been reported as a method with less side effects and more effectiveness. We started this research in order to observe the effects of levamisole and compare it to other therapy results. Patients and Methods : We chose 16 steroid dependent nephrotic syndrome children, those who had shown frequent relapse during the immunocompromised therapy period. Mean age was $9.1{\pm}1.4$ years in children and the male to female ratio was 15:1. All of subjects were diagonized with MCNS and had received cyclophosphamide or cyclosporin before receiving levamisole. Levamisole at a dose of 2.5mg/kg was used every other day for 1 year and the relapse rate was observed. Results : On average of 14 days after treatment, complete remission was visible in all of the children, and the relapse percentage was $50\%$, which represents 8 children, while remaining 8 children representing $50\%$ of the cases showed no relapse during treatment. During the levamisole therapy period, tile average relapse rate was reduced significantly from $2.18{\pm}0.9/year\;to\;0.77{\pm}0.9/year$(P=0.027). Also the average relapse rate after the therapy was reduced to $1.34{\pm}1.1/year$, which was a significant level compared to the level before treatment(P=0.003). There was no significant difference in terms of duration of remission maintenance. Duration of remission maintenance showed an average of $12.2{\pm}9.1$ months before the use of levamisole, but it was also $10.1{\pm}6.9$ month after therapy. No other side effects such as leukopenia, skin disease and other clinically significant symptoms appeared at all during therapy. Conclusion : The long-term medication of levamisole for the therapy of SDNS children is thought to be able to maintain stable remission by reducing the relapse frequency without causing severe side effects. Further study with a broader range of subjects is required to eluccidate the long-term effects of this treatment. (J. Korean Soc Pediatr Nephrol 2001;5 : 109-16)

• Journal of Chest Surgery
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• v.42 no.1
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• pp.53-58
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• 2009

Background: The goal of this study was to compare the patency and complications of femoropopliteal bypass with superficial femoral artery stenting for patients with atherosclerotic superficial femoral artery occlusive disease. Material and Method: Between July 2005 and July 2008, we reviewed 29 femoropopliteal bypass procedures (24 patients) with prosthetic grafts (the bypass group) and 19 superficial femoral artery stentings (15 patients) with nitinol stent (the stent group). There were 35 male patients (89.7%) and the mean age of the patients was 69.2 years (range: 48~84). The number of patients who had DM, hypertension and a smoking history was 25 patients (64.1%), 17 patients (43.6%) and 30 patients (76.9%), respectively. 23 (59.0%) patients had skin ulceration or tissue gangrene at admission. Result: There were 27 cases (93.0%) of TASC C&D lesion in the bypass group and 16 cases (84.2%) of TASC A&B lesion in the stent group. There were significant differences for the indications for a procedure between the two groups (p<0.01). The primary patency rates at 6 months, 12 months and 24 months were 91.9%, 79.7% and 79.7% for the bypass group and 93.3%, 86.2% and 86.2% for the stent group, respectively. There were no statistical difference between the two groups (p=0.48). Conclusion: There were no significant differences in the outcome between two groups. TASC C&D lesion and failed intervention therapy should be treated with femoropopliteal bypass surgery, and TASC A&B lesion and the high-risk patients should be treated with femoral artery stent insertion.

• Journal of Chest Surgery
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• v.40 no.11
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• pp.765-769
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• 2007

Background: The curative treatment of choice for empyema is decortication of the pleura. The risks of this treatment however are increased for the patient with reduced pulmonary function, complicated calcification or septic shock. In the past, open window thoracostomy was a final stage treatment for chronic empyema. Relatively safe treatment of empyema could be achieved in difficult cases with a closure of the open window after open drainage and use of a myocutaneous flap (one stage or staged). Material and Method: A retrospective study of the cause, progression and final outcome of empyema patients who received open window thoracostomy was performed. 21 patients were followed from 1995 to 2004 in the department of Thoracic and Cardiovascular Surgery in the College of Medicine, Pusan National University. Result: The average age of the patients was $57.5{\pm}15.5$ years (range $25{\sim}78$ years), of whom 16 (76.2%) were men and five (23.8%) were women. Pulmonary function test results showed an average FEV1 of $1.58{\pm}0.49 L$. The type of empyema was tuberculous empyema in 13 cases (61.9%), aspergillosis in three cases (14.3%), parapneumonic empyema in three cases (14.3%) and post-resectional empyema in two cases (10%). Bronchopulmonary fistula was seen in 14 cases. Eight cases were complicated by severe calcification of the pleura. For the four cases of bronchopulmonary fistula, the patients' serratus anterior muscle was covered in their first operation. The average number of ribs resected was $4{\pm}1$. Closure of the open window thoracostomy was performed in 12 cases. The average time to closure after open drainage was $10.22{\pm}3.11$ months and the average defect of the empyemal cavity before the final operation was $330{\pm}110 cc$. Among the 12 cases, there were two cases of spontaneous closure. In two cases closure was only achieved by using the reserved skin fold during the first surgery. Of the remaining eight cases, in seven we used the myocutaneous flap (four cases of lattisimus dorsi muscle and three cases of pectoralis major muscle), and in one case we used soft tissue. As regards complications of the closure, tissue necrosis occurred in one case, which led to failed closure, and there was one case of abdominal hernia in the rectus abdominis muscle flap. One patient died within 30 days of the surgery and one patient died of metastatic cancer. Conclusion: A staged operation with a final closure using open window thoracostomy, which consists of open drainage, transposition of the muscle and a myocutaneous flap, can be a safe and effective option for the chronic empyema patient who is difficult to cure with traditional surgical methods.

• Clinical and Experimental Pediatrics
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• v.46 no.8
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• pp.751-757
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• 2003

Purpose : Obesity is a significant health problem with medical and psychological consequences for children and adolescents. The purpose of this study was to assess the correlation between body fat percent using bioelectrical impedance(BI) and other variable methods. Methods : We measured height, weight, body mass index(BMI) and body fat percent by skinfold thickness(ST) and BI in 1,035(496 male; 539 female) children from seven to 18 years of age. The correlation coefficients between BI and each of the other different methods were obtained. The sensitivity and specificity to predict obesity by these several methods were studied. Results : Fat percent estimated by BI analysis and BMI showed a strong correlation(r=0.749). Fat percent estimated by BI analysis and ST showed a very strong correlation(r=0.835). At the 95th percentile cut-off point for BI, ST showed a sensitivity of 57.7%, and a specificity of 97.6% for estimating body fat. At the 95th percentile cut-off point for BI, BMI showed a sensitivity of 84.9%, and a specificity of 99.3% for estimating body fat. Conclusion : The fat percent estimated by BI analysis correlated strongly with ST or BMI. BI analysis is an objective and accurate method for estimating body fat in childhood obesity.

• Clinical and Experimental Pediatrics
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• v.48 no.10
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• pp.1132-1138
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• 2005

Purpose : The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is important because recombinant enzyme replacement therapy is now available for MPS. We studied the clinical characteristics of 80 MPS children with the object of determining the epidemiological, clinical and radiological features in Korean MPS children. Methods : Diagnosis of MPS was confirmed by skin fibroblast enzyme analysis in 80 patients between February 1995 and December 2004. Charts were retrospectively reviewed for clinical and radiological findings, as well as for intelligence and speech evaluations. Results : Hunter syndrome (MPS type II) was the most prevalent type, appearing in 51/80 cases (64 %), followed by Sanfilippo syndrome (MPS III-18%), Hurler syndrome (MPS I-15%), and Morquio syndrome (MPS IV-4%). The average age at diagnosis was 5.5 years (range 1 to 20), and the male-to-female ratio was 4.7 : 1. Typical radiographic changes were observed in 45/54 cases (83%). Mitral regurgitation was the most common cardiac defect. Moderate to profound mental retardation and hearing loss were present in 14/35 cases (56%) and 33/38 cases (82%), respectively. Four MPS II patients had bone marrow transplantation, with mixed outcomes. Five MPS I patients are currently on enzyme replacement therapy. Conclusion : Our study showed a high proportion of MPS II cases (64%), which may represent population variability. By studying the clinical features of these patients, we hope to alert pediatricians of the warning signs of MPS.

• Clinical and Experimental Pediatrics
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• v.49 no.11
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• pp.1186-1193
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• 2006

Purpose : Enterovirus infection is a type of viral infection that occurs relatively frequently in children during summer. It has clinical symptoms of non-specific fever, aseptic encephalomeningitis, gastrointestinal diseases, skin rash and, hand-foot-mouth disease. However, it can also occcaisionally, result in fatal symptoms like myocarditis, epicardial inflammation, transverse myelitis, quadriplegia and etc. There have been epidemic enterovirus studies, but not in the Chungnam area. Therefore, we undertook this study in order to comprehend the cause viruses in this area. Methods: We enlisted 157 children hospitalized with enteroviral infections at Soonchunhyang University hospital in Cheonan between May and August 2005. Cerebrospinal fluids or feces were collected during the acute phase after hospitalization, and observed the cytopathic effects caused by enterovirus and using reverse transcription polymerase chain reaction (RT-PCR). Results : The number of children hospitalized due to possible enteroviral infection during the period of study was 157. The number of children who tested positive with the reverse transcription polymerase chain reaction totalled 32 cases (20.4 percent). Among the children with entroviral diseases, 20 were male and 12 were female, thus the sex ratio of male to female was 1.67:1. Their clinical symptoms included fever most frequently (93.7 percent), was followed by headaches (90.0 percent), meningeal irritation signs (65.0 percent), and abdominal pain (30.0 percent). As for the type of isolated enterovirus, there were 17 cases of echovirus 18 and 6 cases of coxsackievirus B5. Furthermore, there were 2 cases of echovirus 9, 1 case of coxsackievirus A6 and coxsackievirus B3, respectively. But 5 cases were not determined by genotype. Conclusion : Echovirus 18 is circulating in Korea. We reported on identified enteroviruses, including echovirus 18, using RT-PCR in the Chungnam area during the summer of 2005.

• Tuberculosis and Respiratory Diseases
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• v.51 no.4
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• pp.346-353
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• 2001

Background : Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. Method : Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. Result : There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. Conclusion : In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.

• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.1-8
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• 2000

Purpose: This study was undertaken to evaluate the clinical features and complication such as esophageal stricture in children with corrosive esophagitis. Methods: We retrospectively reviewed medical records of 31 children who accidentally ingested corrosive materials and visited to emergency room of Chonnam National University Hospital from Jan. 1992 to Dec. 1999. Twenty-one children were examined by upper gastrointestinal (UGI) endoscopy to evaluate location and severity of caustic injury. Results: 1) Among 31 patients, there were 20 males and 11 females and the ratio of male to female was 2:1. Average age at diagnosis was 2.3 years (12 months to 9.8 years). Twenty-seven (87.1%) patients were accidentally ingested vinegar. 2) Initial presenting symptoms were dysphagia (54.8%), vomiting (48.3%), chemical burn on lips and skin (45.2%), excessive salivation (45.2%), coughing and respiratory grunting (32.3%) and aspiration pneumonia (9.8%). 3) UGI endoscopic examination showed caustic injury in 17 children: grade I in 8, grade II in 7 and grade III in 2. The region of caustic injury was proximal esophagus in 5, distal esophagus in 3, entire esophagus in 9 and stomach in 6. 4) Corrosive esophageal strictures developed in 6 children (19.4%) and gastric outlet stricture in 1 (3.2%). All of them showed grade II or III caustic injury on endoscopic examination. Conclusion: The development of esophageal stricture was related to the severity of the caustic injury. Early UGI endoscopic examination in caustic ingestion seems to be useful for prediction of development of caustic stricture.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.592-597
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• 2010

Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency disease which is characterized by high serum IgE levels, eczema, and recurrent infections. Herein we present the case of a patient with HIES associated with STAT3 gene ($stat3$) mutation. A 16 year-old girl was admitted to our hospital due to hemoptysis caused by pneumonia with bronchiectasis. She had a history of recurrent skin and respiratory tract infections, such as pneumonia caused by MRSA (methicillin-resistant $Staphylococcus$ $aureus$) and $Pseudomonas$ $aeruginosa$. On physical examination, a broad round shaped nose, oral thrush, and chronic eczematous skin rash over her whole body were found. Laboratory data showed an elevated eosinophil count ($750/{\mu}L$) and total IgE level (5,001 U/mL). The patient's National Institutes of Health (NIH) score for HIES was 44. Direct sequencing of the STAT3 gene revealed that the patient was heterozygous for a missense mutation in the DNA binding domain of the STAT3 protein (c.1144C>T, p. Arg382Trp). HIES should be suspected in patients with recurrent infections and can be confirmed by clinical scoring and genetic analysis.