• Microbiology and Biotechnology Letters
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• v.35 no.3
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• pp.196-202
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• 2007

Mutations in the cystathionine ${\beta}$-synthase (CBS) gene cause homocystinuria, the most frequent inherited disorder in sulfur metabolism. CBS is the unique enzyme using both heme and pyridoxal 5-phosphate (PLP) for activity. Among the reported 140 mutations, one of the most common disease-causing alterations in human CBS is G307S mutation. To investigate the pathogenic mechanism of G307S by spectroscopic methods, we engineered the full length and the truncated G247S mutation of yeast CBS that is corresponding mutation to human G307S. Yeast CBS does not contain heme and thus gives a merit to study the spectroscopic properties. The UV-visible spectra of the purified full length and the truncated G247S yeast CBSs showed the total absence of PLP in the protein. The absence of PLP in G247S mutation was also confirmed by the PLP-cyanide adduct formation experiment, which was conducted by the incubation of the purified enzyme with KCN. The adducts were detected using a circular dichroism (CD) and a spectrofluorimeter. Radio isotope activity assay of full length and truncated G247S proteins also gave no activity. Our yeast G247S mutation data suggested that G307S might make the distortion of the active site so that cofactor PLP and substrate can not fit inside the active site. Our yeast CBS study addressed the reason why the G307S mutation in human CBS makes the enzyme inactive that consequently leads to severe clinical phenotype.

• Pediatric Infection and Vaccine
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• v.14 no.2
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• pp.179-187
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• 2007

Purpose : Acute viral respiratory tract infection is the most common illness among children. Involved organisms are respiratory syncytial virus (RSV), influenza virus, adenovirus, parainfluenza virus, etc. The objective of the present study is to determine epidemiology of each viral infection in Busan, South Korea between January 2004 and December 2006. Methods : We retrospectively analyzed the results of clinical samples of throat and nasal swab collected from Busan city, South Korea between January 2004 and December 2006. We collected these samples from the children with acute respiratory illness. Viruses were detected by virus culture Results : The identified pathogens were influenza A in 131 cases (46.6 percent), RSV in 40 cases (14.2 percent), influenza B in 37 cases (13.2 percent), adenovirus in 23 cases (8.2 percent), enterovirus in 37 cases (13.2%) [coxsackie virus in 20 cases (7.1 percent), atypical enterovirus in 9 cases (3.2 percent), poliovirus in 4 cases (1.4 percent), echovirus in 4 cases (1.4 percent)], rhinovirus in 9 cases (3.2 percent), parainfluenza virus in 2 cases (0.7 percent), coronavirus and bocavirus in each 1 case (0.4 percent). Influenza A were detected every year, mostly in December through April and RSV in October through February. Adenovirus were detected through out the year. Bocavirus occurred in December, 2006. Conclusion : We analyzed epidemiologic characteristics of viruses to cause the respiratory disease that prevailed at Busan area for recent three years, and from now on, a further continuous study will be necessary.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.166-173
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• 2003

Purpose : The cause and pathogenesis of $Henoch-Sch\"{o}nlein$ purpura has been studied for many years but the results are disappointing. Recently the hypothesis that abnormalities involving the glycosylation of the hinge region of immunoglobulin Al(IgAl) may have an important role in the pathogenesis of $Henoch-Sch\"{o}nlein$ purpura is being approved. $Henoch-Sch\"{o}nlein$ purpura is the most common vasculitis Ihat affects children and the prognosis is good. But if kidney invovement occurs, the course may be chronic and troublesome. So we evaluated children with $Henoch-Sch\"{o}nlein$ purpura especially from the point of epidemiology and clinical manifestations. Methods : Investigation of 124 children who were diagnosed with $Henoch-Sch\"{o}nlein$ purpura at Inje University Ilsan Paik Hospital from December 1999 to July 2003 was performed retrospectively through chart review. Efforts were made to get informations about the profile, epidemiology, clinical manifestations, progress of the disease and recurrence rate of patients. Results : The patients were 69 boys and 55 girls, with a mean age of $6.1{\pm}2.7$ years at the time of data collection. The male to female ratio was 1.25 : 1. The occurrence rate was much higher in autumn(from September to November, 31.5%) and winter(from December to February, 28.2%) than in spring and summer, with a peak in November. Joint involvement was shown in 66.9% of patients mostly on the foot/ankle(75.9%), knee(39.8%). Seventy(56.5%) out of 124 patients had abdominal pain and 10 patients(8.1%) showed bloody stools. Renal involvement was observed in 24 patients(19.4%) after 21.1 days on the average. IgA was elevated in 10 of 21 patients(47.6%). $C_3$ and $C_4$ levels were normal in 40 of 49 patients (81.7%) and 47 of 48 patients(97.9%), respectively Antistreptolysin-O(ASO) titer was elevated over 250 Todd units in 29 of 62 Patients(46.8%). Mycoplasma antibody titer was elevated in 21 of 49 patients(42.9%) equal or greater than 1:80. Radiologic studies were peformed in 23 patients. Seven patients(30.4%) showed bowel wall thickening and one of them received intestinal resection and anastomosis operation due to terminal ileum necrosis. Eighty four patients took steroid 1.4 mg/kg/day in average. Recurrence rate was 2.5 in 37 patients(29.8%). Conclusion : $Henoch-Sch\"{o}nlein$ purpura in childhood appears most in about 6 years of age. The occurrence rate is much higher in autumn and winter relatively. Diagnosis can be made through the perspective history taking and the inspection of clinical manifestations, but the laboratory findings are not of great help. A small portion of the patients might show abdominal pain or arthritis before purpura develops, therfore various diagnosis can be made. Radiologic evaluation should be performed to avoid surgical complications in cases accompanying abdominal pain, and long term follow up should be needed especially in patients suffering from kidney involvement. In about 30% of the patients $Henoch-Sch\"{o}nlein$ purpura would recur. Steroid can be used safely without side effects.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1972.03a
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• pp.6-7
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• 1972

The air pollutants can be classified into the irritant gas and the asphixation gas, and the irritant gas is closely related to the otorhinolaryngological diseases. The common irritant gases are nitrogen oxides, sulfur oxides, hydrogen carbon compounds, and the potent and irritating PAN (peroxy acyl nitrate) which is secondarily liberated from photosynthesis. Those gases adhers to the mucous membrane to result in ulceration and secondary infection due to their potent oxidizing power. 1. Sulfur dioxide gas Sulfur dioxide gas has the typical characteristics of the air pollutants. Because of its high solubility it gets easily absorbed in the respiratory tract, when the symptoms and signs by irritation become manifested initially and later the resistance in the respiratory tract brings central about pulmonary edema and respiratory paralysis of origin. Chronic exposure to the gas leads to rhinitis, pharyngitis, laryngitis, and olfactory or gustatory disturbances. 2. Carbon monoxide Toxicity of carbon monoxide is due to its deprivation of the oxygen carrying capacity of the hemoglobin. The degree of the carbon monoxide intoxication varies according to its concentration and the duration of inhalation. It starts with headache, vertigo, nausea, vomiting and tinnitus, which can progress to respiratory difficulty, muscular laxity, syncope, and coma leading to death. 3. Nitrogen dioxide Nitrogen dioxide causes respiratory disturbances by formation of methemoglobin. In acute poisoning, it can cause pulmonary congestion, pulmonary edema, bronchitis, and pneumonia due to its strong irritation on the eyes and the nose. In chronic poisoning, it causes chronic pulmonary fibrosis and pulmonary edema. 4. Ozone It has offending irritating odor, and causes dryness of na sopharyngolaryngeal mucosa, headache and depressed pulmonary function which may eventually lead to pulmonary congestion or edema. 5. Smog The most outstanding incident of the smog occurred in London from December 5 through 8, 1952, because of which the mortality of the respiratory diseases increased fourfold. The smog was thought to be due to the smoke produced by incomplete combustion and its byproduct the sulfur oxides, and the dust was thought to play the secondary role. In new sense, hazardous is the photochemical smog which is produced by combination of light energy and the hydrocarbons and oxidant in the air. The Yonsei University Institute for Environmental :pollution Research launched a project to determine the relationship between the pollution and the medical, ophthalmological and rhinopharyngological disorders. The students (469) of the "S" Technical School in the most heavily polluted area in Pusan (Uham Dong district) were compared with those (345) of "K" High School in the less polluted area. The investigated group had those with subjective symptoms twice as much as the control group, 22.6% (106) in investigated group and 11.3% (39) in the control group. Among those symptomatic students of the investigated group. There were 29 with respiratory symptoms (29%), 22 with eye symptoms (21%), 50 with stuffy nose and rhinorrhea (47%), and 5 with sore thorat (5%), which revealed that more than half the students (52%) had subjective symptoms of the rhinopharyngological aspects. Physical examination revealed that the investigated group had more number of students with signs than those of the control group by 10%, 180 (38.4%) versus 99 (28.8%). Among the preceding 180 students of the investigated group, there were 8 with eye diseases (44%), 1 with respiratory disease (0.6%), 97 with rhinitis (54%), and 74 with pharyngotonsillitis (41%) which means that 95% of them had rharygoical diseases. The preceding data revealed that the otolaryngological diseases are conspicuously outnumbered in the heavily polluted area, and that there must be very close relationship between the air pollution and the otolaryngological diseases, and the anti-pollution measure is urgently needed.