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Diffuse Leptomeningeal Glioneuronal Tumor with FGFR1 Mutation in a 29-Year-Old Male (29세 남성에서 발생한 FGFR1 돌연변이를 동반한 미만성 연수막성 신경교종)

  • Minsu Kim;Ki Rim Lee;Gheeyoung Choe;Kihwan Hwang;Jae Hyoung Kim
    • Journal of the Korean Society of Radiology
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    • v.84 no.4
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    • pp.970-976
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    • 2023
  • This study reports on diffuse leptomeningeal glioneuronal tumor (DL-GNT) in a 29- year-old male. DL-GNT is a rare central nervous system (CNS) tumor mostly seen in children and only few cases have been reported in adult patients. Our patient presented with a chronic headache that lasted for five months. MR imaging showed mild hydrocephalus, multiple rim-enhancing nodular lesions in the suprasellar cistern, diffuse leptomeningeal enhancement in the lumbosacral area, and multiple small non-enhancing cyst-appearing lesions not suppressed on fluid attenuated inversion recovery (FLAIR) images in the bilateral basal ganglia, thalami, and cerebral hemispheres. Under the impression of germ cell tumor with leptomeningeal seeding, the patient underwent trans-sphenoidal tumor removal. DL-GNT was pathologically confirmed and FGFR1 mutation was detected through a next-generation sequencing test. In conclusion, a combination of leptomeningeal enhancement and multiple parenchymal non-enhancing cyst-appearing lesions not suppressed on FLAIR images may be helpful for differential diagnosis despite overlapping imaging features with many other CNS diseases that have leptomeningeal enhancement.

National Survey of Sarcoidosis in Korea (유육종증 전국실태조사)

  • 대한결핵 및 호흡기학회 학술위원회
    • Tuberculosis and Respiratory Diseases
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    • v.39 no.6
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    • pp.453-473
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    • 1992
  • Background: National survey was performed to estimate the incidence of sarcoidosis in Korea. The clinical data of confirmed cases were analysed for the practice of primary care physicians and pulmonary specialists. Methods: The period of study was from January 1991 to December 1992. Data were retrospectively collected by correspondence with physicians in departments of internal medicine, dermatology, ophthalmology and neurology of the hospitals having more than 100 beds using returning postcards. In confirmed and suspicious cases of sardoidosis, case record chart for clinical and laboratory findings were obtained in detail. Results: 1) Postcards were sent to 523 departments in 213 hospitals. Internal medicine composed 41%, dermatology 20%, ophthalmology 20% and neurology 19%. 2) Postcards were returned from 241 departments (replying rates was 48%). 3) There were 113 confirmed cases from 50 departments and 10 cases. The cases were composed from internal medicine (81%), dermatology (13%), ophthalmology (3%) and neurology (3%). 78 confirmed cases were analysed, which were composed from department of internal medicine (92%), dermatology (5%), and neurology (3%). 4) The time span for analysed cases was 1980 to 1992. one case was analysed in 1980 and the number gradually increased to 18 cases in 1991. 5) The majority of patients (84.4%) were in the age group of 20 to 49 years. 6) The ratio of male to female was 1 : 1.5. 7) The most common chief complains were respiratory symptoms, dermatologic symptoms, generalized discomforts, visual changes, arthralgia, abdominal pains, and swallowing difficulties in order. 16% of the patients were asymptomatic. 8) Mean duration between symptom onset and diagnosis was 2 months. 9) The most common symptoms were respiratory, general, dermatologic, ophthalmologic, neurologic and cardiac origin in order. 10) Hemoglobin, hematocrits and platelet were in normal range. 58% of the patients had lymphopenia measuring less than 30% of white cell count. The ratio of CD4 to CD8 lymphocytes was $1.73{\pm}1.16$ with range of 0.43 to 4.62. ESR was elevated in 43% of the cases. 11) Blood chemistry was normal in most cases. Serum angiotensin converting enzyme (S-ACE) was $66.8{\pm}58.6\;U/L$ with the range of 8.79 to 265 U /L. Proteinuria of more than 150 mg was found in 42. 9% of the patients. 12) Serum IgG was elevated in 43.5%, IgA in 45.5%, IgM in 59.1% and IgE in 46.7%. The levels of complement C3 and C4 were in the normal range. Anti-nuclear antibody was detected in 11% of the cases. Kweim test was performed in 3 cases, and in all cases the result was positive. 13) FVC was decreased in 17.3%, FEV1 in 11.5%, FEV1/FVC in 10%, TLC in 15.2%, and DLco in 64.7%. 14) PaO2 was decreased below 90 mmHg in 48.6% and PaCO2 was increased above 45 mmHg in 5.7%. 15) The percentage of macrophages in BAL fluid was $51.4{\pm}19.2%$, lymphocytes $44.4{\pm}21.1%$, and the ratio of CD4 to CD8 lymphocytes was $3.41{\pm}2.07$. 16) There was no difference in laboratory findings between male and female. 17) Hilar enlargement on chest PA was present in 87.9% (bilaterally in 78.8% and unilaterally in 9.1%). 18) According to Siltzbach's classification, stage 0 was 5%, stage 158.3%, stage 228.3%, and stage 38.3%. 19) Hilart enlargement on chest CT was present in 92.6% (bilaterally 76.4% and unilaterally in 16.2%). 20) HRCT was done in 16 cases. The most common findings were nodules, interlobular thickening, focal patchy infiltrations in order. Two cases was normal finding. 21) Other radiologic examinations showed bone change in one case and splenomegaly in two cases. 22) Gallium scan was done in 12 cases. Radioactivity was increased in hilar and mediastinal lymph nodes in 8 cases and in parenchyme in 2 cases. 23) The pathologic diagnosis was commonly performed by transbrochial lung biopsy (TBLB, 47.3%), skin and mediastinal lymph nodes biopsy (34.5%), peripheral lymph nodes biopsy (23.6%), open lung biopsy (18.2%) and bronchial biopsy in order. 24) The most common findings in pathology were non·caseating granuloma (100%), multi-nucleated giant cell (47.3%), hyalinized acellular scar (34.5%), reticulin fibrin network (20%), inclusion body (10.9%), necrosis (9.1%), and lymphangitic distribution of granuloma (1.8%) in order. Conclusion: Clinical, laboratory, radiologic and pathologic findings were summarized. This collected data will assist in finding a test for detection and staging of sarcoidosis in Korea in near future.

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Skin Regeneration, Anti-wrinkle, Whitening and Moisturizing Effects of Cheongsangbangpung-tang Aqueous Extracts with Cytotoxicity (청상방풍탕 열수 추출물의 피부재생, 주름개선, 미백, 보습 효과 및 세포독성 평가)

  • Woo, Chang-Yoon;Kim, Dong-Chul
    • The Journal of Korean Obstetrics and Gynecology
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    • v.30 no.2
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    • pp.49-70
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    • 2017
  • Objectives: The present study is to observe the skin-regeneration, anti-wrinkle, whitening and skin moisturizing effects of Cheongsangbangpung-tang (CSBPT) with cytotoxicity. Methods: In the present study, cytotoxicity of CSBPT lyophilized aqueous extracts (yield=18.71%) was experimented against human normal fibroblast cells and B16F10 murine melanoma cells by 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium Bromide (MTT) assay, and skin regeneration and anti-wrinkle effects were also showed through the assay of collagen type I synthesis by an enzyme immunoassay (EIA) kit as comparing with transforming growth factor (TGF)-${\beta}1$, hyaluronidase, collagenase and matrix metalloproteinase (MMP)-1 inhibitory assays as comparing with oleanolic acid (OA), and elastase inhibitory effects as comparing with phosphoramidon disodium salt (PP). In addition, whitening effects of CSBPT were observed by tyrosinase inhibitory assay and melanin formation test in B16/F10 melanoma cells as comparing with arbutin, and skin moisturizing effects were measured through mouse skin water contents test, respectively. Results: No CSBPT treatment related cytotoxic effects were demonstrated against human normal fibroblast cells and B16/F10 murine melanoma cells. CSBPT concentration-dependent increased collagen type I synthesis at human normal fibroblast cells. It also effectively suspreessed hyaluronidase, collagenase, elastase and MMP-1 activities, which were enzymes that related to declining of ECM and formation of wrinkle. CSBPT supressed B16/F10 melanoma cells's melanin productions with tyrosinase activity, which was an enzyme connected with melanin formation, and dose-dependent and significant increases of skin water contents were detected in CSBPT treated mouse skin as compared with vehicle control skins. Conclusions: CSBPT showed favorable and enough skin regeneration, anti-wrinkle, whitening and skin moisturizing effects at least in a condition of this experiment. However, more detail mechanism and in vivo skin protective efficacy studies should be conducted in future with the screening of the biological active compounds in individual herbs of Cheongsangbangpung-tang.

The Utility of MAGE Gene Detection in Bronchial Washing Fluid for Patients with Peripheral NSCLC (말초 비소세포폐암 환자에서 기관지 세척액 MAGE 유전자 진단의 유용성)

  • Kim, Suhyun;Kim, Hojoong;Kwon, O Jung;Chung, Man Pyo;Suh, Gee Young;Koh, Won-Jung;Ham, Cho Rom;Nam, Hae Seong;Um, Sang-Won;Kwon, Yong Soo;Park, Sung-Hoon
    • Tuberculosis and Respiratory Diseases
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    • v.64 no.1
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    • pp.15-21
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    • 2008
  • Background: The melanoma antigen-encoding (MAGE) genes are known to be expressed in various cancer cells, including non-small cell lung cancer (NSCLC), and are silent in all normal tissues except for the testis. In patients with peripheral NSCLC, bronchial washing fluid can be used to detect the MAGE genes, suggesting a diagnosis of lung cancer. In order to evaluate the diagnostic utility of the MAGE test in patients with peripheral NSCLC, bronchial washing fluid was investigated in patients with peripheral pulmonary nodules, which were invisible as detected by bronchoscopy. Methods: Bronchial washing fluid from 37 patients was used for cytological examinations and MAGE gene detection, using RT-nested-PCR of common A1-A6 mRNA. Results were compared to a final diagnosis of patients as confirmed by pathology. Results: Among the 37 subjects, NSCLC was diagnosed in 21 patients, and benign pulmonary diseases were diagnosed in 16 patients. MAGE mRNA was detected in 10 of 21 (47.6%) NSCLC patients, while conventional cytology examinations were positive for MAGE expression in 2 of 21 (9.5%) cases. MAGE expression was observed in 4 of 16 (25%) benign pulmonary disease patients. Conclusion: The MAGE test of bronchial washing fluid can be used as a sensitive predictor of peripheral NSCLC patients.

A Study on the Stability Test for the Cream Containing Suaeda Asparagoides Extract (나문재 추출물 함유 크림의 안정성 평가에 관한 연구)

  • Park, Soo-Nam;Jeon, So-Mi;Ahn, Jeung-Youb
    • Journal of the Society of Cosmetic Scientists of Korea
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    • v.33 no.4
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    • pp.231-238
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    • 2007
  • In the previous study, the anti-oxidant activity of extract/fraction of Sueada asparagoides (SA) was investigated and the results showed that the ethylacetate (EtOAc) fraction and its aglycone fraction had the best performance on the free radical scavenging activity, reactive oxygen species scavenging (ROS) activity and cell protective activity (J. Soc. Cosme. Scientists Korea, 33(3), 145 (2007)). In this study, the stability of cream containing 0.3% SA EtOAc extract (called extract below) was evaluated. pH, viscosity and absorbance (363 nm) were measured under the 4 different temperatures ($0^{\circ}C,\;25{\circ}C,\;37{\circ}C\;and\;45{\circ}C$) and under the sun light at the 4 week intervals during the 12 weeks in total. The control cream without containing the extract did not show pH change under the different temperatures mentioned above. However, the pH of the cream the extract was decreased 0.08 at the temperature ranges of $0^{\circ}C\;to\;37^{\circ}C$. Under the $45^{\circ}C$ and sun light condition, the pH was decreased 0.51 and 0.66, respectively. The cream containing the extract did not show absorbance change at the temperature ranges of 0 to $37^{\circ}C$ for 12 weeks. Instead, the absorbance of the cream treated under $45^{\circ}C$ and sun light condition was decreased 7.6 % and 7.4 %, respectively. This decrease in absorbance is relatively small compared to the 48.3 % decrease of the extract sampled from the cream using ethanol solution. This indicates that the extract is stabilized in the cream. After treating the cream for 12 weeks under the different temperatures, the viscosity was measured for the cream containing the extract and control cream. The values were increased by 1,748 cPs in average compared to the initial value for the former and by 951 cPs in average for the latter. On the other hand, the viscosity of control cream treated under the sun light for 12 weeks was significantly decreased (4,022 cPs) relative to the cream containing the extract, which showed 2,484 cPs increase in viscosity. This indicates that the SA extract contributes to the stability of the emulsion product by protective effect to maintain the viscosity of the cream against sun light. In addition, any change in color or smell was not observed through 12 weeks of the experimental time period. Thus, it is concluded that it is still not clear in the stability of the cream containing the extract when it is stored for the long time. Accordingly, it is suggested that further study is needed to provide more information to the manufactures, who are seeking for the application of the extract to improve the anti-oxidant activity and stability of cosmetic products.

Long Term Results of Radiation Therapy in Early Glottic Cancer (초기 성문암의 방사선치료: 장기 추적결과)

  • Kim, Jin-Hee;Byun, Sang-Jun
    • Radiation Oncology Journal
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    • v.27 no.1
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    • pp.29-34
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    • 2009
  • Purpose: This study was designed to evaluate long-term results in terms of failure, survival and voice preservation after radiation therapy for early glottic cancer. Materials and Methods: From February 1988 to December 2003, 70 patients with early glottic cancer were treated with radiation therapy at Keimyung University Dongsan Medical Center. Patient age distribution was from 39 to 79 years, with a median age of 62 years. All patients had squamous cell carcinoma. According to the TNM stage, 58 patients had stage I disease, 12 patients had stage II disease; 67 patients were male. The laryngeal area was irradiated with the use of bilateral opposing fields with/without a wedge filter with 6 MV photons at a total dose of $54{\sim}70.2$ Gy in $1.8{\sim}2.2$ Gy fractions over $6{\sim}8$ weeks. We delivered a median radiation dose of 60 Gy for stage I patients and a median radiation dose of 66 Gy for stage II patients. Salvage surgery was performed in patients with local recurrence. The voice preservation rate was analyzed after all treatments including salvage surgery. Follow-up periods were from 13 to 180 months, with a median follow-up period of 77.5 months. The survival rate was analyzed by the use of the Kaplan Meier method and log rank test. A comparison of two groups was performed with the use of the chi-squared test. Results: The local control rate was 98.5% (69/70). The five-year-overall survival rate was 93.9%. The five-year disease free survival rate (5YDFS) was 84.1% and the 5YDFS after radiation and salvage surgery was 92.8%. According to stage, the 5YDFS was 93.1% and 91.7% for stage I and stage II respectively. Thirteen patients (18.5%) had local failure with 24 months of median time to local failure and nine patients received salvage surgery; however, four patients were lost to follow-up after a diagnosis of recurrence. Only two patients died due to a distant metastasis at 33 months and 71 months after radiation therapy, respectively. Nine patients died due to other diseases with a median time of 73 months. There were no severe acute or chronic complications after radiation therapy. Voice preservation was ultimately achieved in 88.5% (62/70) of patients. Conclusion: We considered that radiation therapy was effective and we achieved excellent survival and voice preservation in early laryngeal cancer. The use of radiation therapy should be the first choice for the treatment of early glottic cancer.

Keeping Quality and Taste Compounds in the Extracts from Rapid Fermented Anchovy Sauce (속성 멸치간장 엑기스분의 저장 안정성 및 정미성분)

  • Lee, Eung-Ho;Ahn, Chang-Bum;Kim, Jin-Soo;Lee, Kang-Hee;Kim, Myung-Chan;Chung, Bu-Kil;Park, Hee-Yeol
    • Journal of the Korean Society of Food Science and Nutrition
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    • v.18 no.2
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    • pp.131-142
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    • 1989
  • As a part of investigation for utilizing anchovy more effectively as a food source, this work was undertaken the changes in keeping quality and taste compounds in the extracts from rapid fermented anchovy sauce during storage at room temperature. Rapid fermented products was made of chopped anchovy, water, koji and soybean protein isolate (20:10:2:1, w/w) thorough hydroxazine for 6 hours at $50^{\circ}C$. The liquified anchovy sauce extracts, contained 15% salt(w/w), were stored for 60 days at room temperature. The changes in pH, acidity, amino nitrogen and contents of taste compounds of the products were negligible during storage. The viable cell counts and histamines of the products were less than 30(colony/e extracts), 7.2-21.8(mg/100g extracts) during storage predominant free amino acids showed in the extracts from products were alanine, glutamic acid, histidine, lysine, leucine, valine and the total contents of those free amino acids were 60.4-64.3% of total free amino acids at final stage of storage. The major nucleotides and their related compounds of the products were revealed hypoxanthine, which were 69% over the total nucleotides and their related compounds. Using the omission test, the major taste compounds in the products were revealed free amino acids, nucleotides and their related compounds. The non-volatile organic acids, total creatinine, betaine, and TMAO were seemed to act an auxiliary role in taste of the extracts from rapid fermented anchovy sauce.

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Adenoviral Lower Respiratory Tract Infection of Children in Korea from 1990 Through 1998 (소아의 Adenovirus 하기도 감염증에 관한 연구 - 유행 양상과 임상 양상에 대하여 -)

  • Hong, Jung Youn;Lee, Hoan Jong
    • Pediatric Infection and Vaccine
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    • v.7 no.1
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    • pp.94-107
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    • 2000
  • Purpose : The purpose of this study is to know the clinical manifestations and the severity of adenoviral lower respiratory tract infections(LRTI) in Korean children. Methods : Adenoviral respiratory infection was diagnosed by viral culture in HEp-2 cell and indirect immunofluorescent technique with nasal aspirates. Isolated adenoviruses were typed by neutralization test. Retrospective chart review was done in patients with adenoviruses were typed by neutralization test. Retrospective chart review was done in patients with adenoviral lower respiratory tract infection, who were brought to Seoul National University Children's Hospital from November 1990 through February 1998. Results : Adenovirus was isolated in 87 cases. Of 84 cases serotyped, type 1 was recovered in 3 cases, type 2 in 13 cases, type 3 in 13, type 4 and 5 in 4 cases each other, type 6 in 1 cases, type 7 in 36 cases, type 11 in 1 case and the other types in 9 cases. Adenoviral lower respiratory infection occurred sporadically throughout the year but from November 1995 through February 1998, an outbreak of adenovirus type 7 lower respiratory infection was observed in number upto 36 case. The incidence of adenoviral infection peaked in young children between 6 months and 5 years of age and the mean age was 1 year 11 months old. There were 10 cases of mixed infection with another pathogen. Clinical diagnosis were pneumonia(88%), acute broncholitis(5.4%), acute tracheobronchitis(5.4%), croup(1.3%). The clinical features of adenoviral lower respiratory infection were severe especially in type 3 and 7 infections in aspect of fever duration, ventilator care. Extrapulmonary manifestations were gastrointestinal symptoms in 23 cases(31%), hepatomegaly in 36 cases(53%), seizure and mental alteration in 13 cases(20.3%). In chest radiographic findings, parahilar and peribronchial infiltration were in 49 cases(67%), hyperaeration in 21 cases(29%), atelectasis in 14 cases(19%), consolidation in 39 cases(53%) and bilateral pneumonic infiltration in 28 cases(38%). Among thirty six adenovirus type 7 LRTI, 15 patients(41.6%) had pleural effusion and 3 patients had chest tube insertion. Number of fetal cases related to adenovirus were 9 cases(12%) and fetal cases due to ventilatory failure were 7(11%). Conclusion : During 7 year period of studying adenoviral lower respiratory infection, we identified the serotypes of adenovirus. Among the serotypes, adenovirus type 7 were epidemically isolated. Adenovirus were isolated in severe lower respiratory infection of young children aged between 6 months and 5 years and related to death of the patients, especially when the patients had underlyng diseases or were infected by adenovirus type 7.

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Epidemics of Aseptic Meningitis in Kyoungsangnamdo from March to October, 1997 (1997년 경상남도 중부지방에서 유행한 무균성 뇌막염의 임상적 고찰)

  • Park, Sun Young;Kwon, Oh Su;Kim, Won Youb;Jung, Won Jo;Ma, Sang Hyouk;Kim, Sang Ki;Nam, Sung Jin;Jo, Sung Rae;Gu, Bon Chun;Lee, Kyu Man
    • Pediatric Infection and Vaccine
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    • v.5 no.1
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    • pp.104-114
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    • 1998
  • Purpose : Enteroviruses are the most common cause of aseptic meningitis. The epidemics of aseptic meningitis in 1993 and 1996 were mostly caused by echovirus type 9. Identification of the causative virus of aseptic meningitis in epidemics, is very important not only for diagnosis but also for epidemiologic purpose. The purpose of this study was to identify the causative virus and investigate the relationship between aseptic meningitis, prevailed in Masan and surrounding areas in Kyoungsangnamdo in 1997, and its clinical manifestations. Methods : One hundred twenty eight cerebrospinal fluid(CSF) and 239 stool specimens were obtained from 239 patients(213 children and 26 adult patients) with aseptic meningitis were admitted to Masan Fatima Hospitals from March to October 1997. Viral isolation and serotype identification was performed by cell culture and immunofluorescent test. Enteroviruses not typed by immunofluorescent test was confirmed by reverse transcription-polymerase chain reaction(RT-PCR). Results : 1) The peak incidence was noted in June. 2) The age of 239 patients(pediatrics-213 cases, internal medicine-26 cases) that were diagnosed ranged from neonate to 35 years, the age of the patients of pediatrics ranged from neonate to 15years(mean 4.9 years), the age of the patients of internal medicine (above 16 years) ranged from 16 years to 35 years(mean 24.2 years). 3) Fifty-three(41.4%) of 128 CSF specimens were positive for enteroviruses, and 163(68.2%) of 239 stool specimens were positive for enteroviruses respectively. 4) Serotypes of 53 enteroviruses isolated from CSF were 16(30.2%) of echovirus type 30, 6(11.3%) of echovirus type 6, 1 of echovirus type 4, 4 of untyped echovirus, 1 of coxsackievirus type B5, and 24 isolates of untyped enteroviruses. Of 163 enterovirus isolated from stool were 72(44.2%) of echovirus type 30, 21(12.9%) of echovirus type 6, 1 of echovirus type 4, 17(10.4%) of undetermined subtyped echovirus, 1 of coxsackievirus type B5, 2 of A24, 3 of undetermined subtyped coxsackievirus type B, and 46 isolates of untyped enterovirus. Conclusion : There were epidemics of aseptic meningitis in the central areas of Kyoungsangnamdo from March to October 1997. The main causative organism was thought to be the echovirus type 30, and echovirus type 4, 6, coxsackievirus B5 and A24 were also thought to contribute to the epidemics.

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Clarithromycin Therapy for Scrub Typus (쯔쯔가무시(Tsutusgamushi)병에서 Clarithromycin의 치료 효과)

  • Kim, Soon;Jung, Eun Mi;Moon, Kyung Hyun;Yoe, Sung Yeob;Eum, Soo Jung;Lee, Joo Hyung;Jo, Sung Rae;Ma, Sang Hyuk
    • Pediatric Infection and Vaccine
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    • v.9 no.2
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    • pp.175-181
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    • 2002
  • Purpose : Scrub typhus(tsutsugamushi disease) is a febrile disease characterized by fever, rash, eschar, lymphadenopathy. Therapy with tetracycline(doxycycline) or chloramphenicol is currently recommended for the treatment for scrub typhus. But there are limitations in usage a tetracycline(doxycycline) for scrub typhus in the children. Recently, there was a report that azithromycin, a macrolide antibiotic was used for scrub typhus in pregnant woman successfully. So we evaluated the effectiveness of the Clarithromycin, other a macrolide antibiotic, for scrub typhus. Methods : Seven patients with scrub typhus at department of internal medicine and three patients with scrub typhus at department of pediatrics Masan Fatima Hospital were involved for this study. A serologic diagnosis for scrub typhus were performed by use of passive hemagglutination test. Clarithromycin(Abbott Laboratories, North Chicago, IL, USA) was administrated orally in a daily dose of 500 mg for adult patients and 15 mg/kg/bid/day for pediatric patients. Results : There were 7 cases of adult patients, varying from 28 to 76 years of age and 3 cases of pediatirc patients, varying from 4 to 7 years of age with scrub typhus. All of cases had fever, myalgia, headache, rash, eschar. Seven cases had positive passive hemagglutination test and eight cases had abnormal liver function. Mean duration for the removal of fever after medication was 1.3 day(1~2 days) and all cases were recovered without complications. Conclusion : Our results suggest that Clarithromycin therapy may be effective for scrub typhus.

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