• Journal of Life Science
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• v.17 no.12
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• pp.1654-1659
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• 2007

We examined total body irradiation (TBI)-induced effects by complete blood count (CBC) and fluorescence-activated cell sorter analysis (FACS) in the piglet following radiation irritation. A CBC included red blood cell count, white blood cell count, and platelet cell count. Four piglets were examined in this study and each piglet was divided by irradiation dose, two piglets with 4 Gy, two with 6 Gy, one with 8 Gy. All piglets showed leukopenia, thrombocytopenia after irradiation. In 6 and 8 Gy group, three piglets showed severe hemostatic disorder and gastrointestinal disorder suchas diarrhea and anorexia, and they died between 10 and 15 days after radiation irritation. In 4 Gy, two piglets showed no clinical sign after radiation injury, but persistent leukopenia was shown in blood examination. We suggest that a single TBI dose less than 6 Gy is adequate for conditioning piglet for bone marrow transplantation.

• Laboraroty Animal Research
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• v.34 no.4
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• pp.166-175
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• 2018

Recombination activating gene-2 (RAG-2) plays a crucial role in the development of lymphocytes by mediating recombination of T cell receptors and immunoglobulins, and loss of RAG-2 causes severe combined immunodeficiency (SCID) in humans. Rag-2 knockout mice created using homologous recombination in ES cells have served as a valuable immunodeficient platform, but concerns have persisted on the specificity of Rag-2-related phenotypes in these animals due to the limitations associated with the genome engineering method used. To precisely investigate the function of Rag-2, we recently established a new Rag-2 knockout FVB mouse line ($Rag-2^{-/-}$) manifesting lymphopenia by employing a CRISPR/Cas9 system at Center for Mouse Models of Human Disease. In this study, we further characterized their phenotypes focusing on histopathological analysis of lymphoid organs. $Rag-2^{-/-}$ mice showed no abnormality in development compared to their WT littermates for 26 weeks. At necropsy, gross examination revealed significantly smaller spleens and thymuses in $Rag-2^{-/-}$ mice, while histopathological investigation revealed hypoplastic white pulps with intact red pulps in the spleen, severe atrophy of the thymic cortex and disappearance of follicles in lymph nodes. However, no perceivable change was observed in the bone marrow. Moreover, our analyses showed a specific reduction of lymphocytes with a complete loss of mature T cells and B cells in the lymphoid organs, while natural killer cells and splenic megakaryocytes were increased in $Rag-2^{-/-}$ mice. These findings indicate that our $Rag-2^{-/-}$ mice show systemic lymphopenia with the relevant histopathological changes in the lymphoid organs, suggesting them as an improved Rag-2-related immunodeficient model.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1058-1064
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• 2008

Purpose : This study aimed to assess current knowledge of and training experiences with the intraosseous (IO) line among emergency medicine (EM) and pediatric residents who care for critically ill children and to evaluate the educational effectiveness of the IO line workshop. Methods : During May and June 2008, a workshops on IO line insertion was held for EM and pediatric residents. The workshop comprised a 45-min lecture and a 15-min hands-on session. A semi-drill type EZ-IO machine was used for education. Self-assessment questionnaires gauged residents knowledge of and experiences with IO line insertion or bone marrow (BM) examination and their confidence with IO line insertion before and after the workshop. Performance tests were completed for skill evaluation. Results : Forty-five pediatric residents and 22 EM residents participated in the workshop. The pre-educational questionnaire revealed that EM residents had more educational experience in IO line insertion than pediatric residents (P<0.001), while pediatric residents reported more experience in BM examination (P<0.001). The post-educational questionnaire showed a statistically significant higher percentage of correct answers (P<0.001). Although the pediatric residents inserted an IO line more quickly (P=0.001), most residents (88.7%) succeeded in IO line insertion on their first attempt; there was no difference in the groups success rates. Both groups showed higher confidence in performing IO line insertion after training (P<0.001). Conclusion : Observed educational effectiveness in both knowledge and confidence of IO line insertion skill suggest educational opportunities for pediatric and EM residents should be increased.

• Investigative Magnetic Resonance Imaging
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• v.13 no.2
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• pp.146-151
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• 2009

Purpose : To evaluate magnetic resonance (MR) imaging features of non-surgically treated tuberculous spondylitis and to evaluate the relationships between these features and clinical outcomes. Materials and Methods : Data from ten patients (male:female=6:4, mean age=45 years) with clinically proven tuberculous spondylitis who were treated nonsurgically over three months were analyzed retrospectively from 2000 to 2007. MRI was performed at least three times for each patient, at baseline, every three or six months, and at the end of treatment. All images were analyzed by two radiologists. Results : The mean follow-up period for the MR examination was 10.1 months (range, 4-17 months). Six patients had clinically complete resolution of tuberculous spondylitis with medication treatment only. Four patients were treated with surgical management alongside medication. All ten patients were divided into two groups by clinical outcome; six patients with complete treatment and four patients with incomplete treatment. In the complete treatment group, follow-up MR findings showed a loss of subligamentous spread of abscesses, decreased size of abscesses, no interval changes in vertebral body heights, and fatty changes in spinal lesions. MR findings in the incomplete treatment group showed bone marrow edema extension to adjacent vertebra, extension of the abscesses, and decreased height of the vertebral bodies. Conclusion : During the nonsurgical management of tuberculous spondylitis, MR imaging may play a role in predicting patient response to antituberculous drug treatment.

• Tuberculosis and Respiratory Diseases
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• v.53 no.1
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• pp.17-26
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• 2002

Background : The detection and early elimination of the causes for acute respiratory distress syndrome(ARDS) at the initial stage can result in a more favorable prognosis. Miliary tuberculosis as a cause of the ARDS is quite rare. A diagnosis of miliary tuberculosis is difficult due to the diversity of radiological patterns and non-specific clinical finfings, and low sensitivity of sputum examinations for acid-fast bacilli(AFBs). An analysis of the clinical data is the first step in diagnosing these unusual, rare cases. Materials and Methods : In this study the clinical features, laboratory data, radiological findings and diagnostic methods were analyzed in 9 cases with an initial presentation of ARDS due to miliary tuberculosis. The ARDS was defined by the definition of the American-Europian consensus conference 1992. Results : The mean age of the patients was $67{\pm}18$ years (F:M=7:2). The chief complaints were dyspnea(5/9), coughing (3/9) and fever(5/9). On a physical examination, fine or coarse crackles were noted(6/9). The ARDS developed on average 6.7 days after the initial respiratory symptoms. The mean $PaO_2/FiO_2$ of the patients was $133.5{\pm}53.4$, the number of cases with a WBC<5000/$mm^3$ was 4 out of 9 cases. A platelet count<70,000/$mm^3$ was observed in 2 out of 9 cases, and the serum albumin level was $2.6{\pm}0.6$ g/dL. The initial simple chest PA showed ground glass appearances and consolidation in all cases, However, the miliary nodular densities were observed in only 4 out of the 9 cases. HRCT revealed alveolar densities and a consolidation in 5 out of 6 cases, and miliary nodules in 5 out of 6 cases, The diagnosis of tuberculosis was made by a liver biopsy (4/4, 100% sensitivity), a bone marrow biopsy (1/2, 50% sensitivity), and an open lung biopsy (1/1), the sputum AFB was positive in only 2 out of 9 cases. The patient was treated with INH, RFP, EMB, PZA, and steroids. The survival rate was 55.5%. Conclusion : Miliary tuberculosis should be considered as one of the causes for ARDS in areas where there is a high prevalence of tuberculosis. The chief complaints of the patients on admission are dyspnea, fever and coughing without any specific riskfactors. A liver biopsy is particularly useful in ARDS patients with mechanical ventilation to determine the causes of the ARDS if miliary tuberculosis is suspected as being the underlying disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.213-221
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• 2005

Purpose: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. Methods: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. Results: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). Conclusion: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.