• Korean Journal of Veterinary Research
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• v.46 no.3
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• pp.225-234
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• 2006

In the present study toxicity and immunostimulating activity of the lectin(KML-C), which was extracted from Korean mistletoe(Viscum album coloratum) were investigated in swine. To determine the toxicity, lectin was injected into thigh or cervical muscles of 4-week-old piglets(Landrace) and observed clinically and pathologically. For determination of the immnunostimulating activity, lectin($0.7{\mu}g/kg$ of body weight)-adjuvanted vaccine of Aujeszky's disease virus(ADV)(NYJ1-87) which was inactivated by 0.2% formalin was injected into the cervical muscle of antibody-negative piglets in the same age group. Subpopulation of the immune cells and serum neutralizing(SN) antibodies in the piglets were examined after vaccination, and resistance of the piglets against challenge by virulent NYJ1-87 was further examined. The results were also compared with those from piglets injected with aluminum hydroxide [$Al(OH)_3$]-adjuvanted vaccine of inactivated NYJ1-87 and NYJ1-87 vaccine without adjuvant, and the results are as follows. By injection of lectin with $30{\mu}g/kg$ of body weight to the thigh muscle, all of 12 piglets died after signs such as dyspnea, fever, systemic erythema and subcutaneous hemorrhages, and lesions pertaining to poisonous hepatitis and dysfunction of kidney were observed. By injection of lectin with $7{\mu}g/kg$ of body weight to the thigh muscle, all of 12 piglets showed signs such as edema and cutaneous hemorrhage in the injected area, lameness and depression, and lesions pertaining to poisonous hepatitis and dysfunction of kidney were observed. By injection of lectin with 1, 3 and $5{\mu}g/kg$ of body weight to the thigh muscle of each one piglet, signs such as congestion, induration and grayish coloration in the injected area, depression and inappetence were observed in all piglets. Toxic changes were also observed in the liver and kidney of piglets by lectin of 3 and $5{\mu}g$. By injection of lectin with 0.5 and $0.7{\mu}g/kg$ of body weight to the cervical muscle of each 9 piglets, all piglets were clinically normal and there were no significant changes in blood counts and chemistry values. Whereas, epithelial swelling and vacuolation of convoluted tubules were observed from one piglet injected with lectin of $0.7{\mu}g$, and necrosis and fibrosis of muscular fiber were observed in the muscle of one piglet injected with lectin of $0.5{\mu}g$. Only population of sIgM+ B lymphocytes increased among immune cells in all of 15 piglets immunized with lectin($0.7{\mu}g/kg$ of body weight)-adjuvanted vaccine, while compared to those in $Al(OH)_3$-adjuvanted vaccine and vaccine without adjuvant. No additional stimulation to the immune cells was recognized when lectin was added to $Al(OH)_3$-adjuvanted vaccine. In piglets immunized with lectin-adjuvanted vaccine, SN titers in reciprocal values for loge were 1.3-4.0 at 1-4 weeks after vaccination, which was similar to those with 1.0-3.3 by vaccine without adjuvant but lower than those with 2.0-5.7 by $Al(OH)_3$-adjuvanted vaccine. Also, no additional increase in the SN titers was recognized when lectin was added to $Al(OH)_3$-adjuvanted vaccine. Piglets immunized with lectin-adjuvanted vaccine were resistant to challenge by the virulent NYJ1-87 at 4 weeks after vaccination, and the SN titers reached to 5.0 one week after challenge, which was higher than those with 4.0 by vaccine without adjuvant but somewhat lower than those with 7.7 by $Al(OH)_3$-adjuvanted vaccine.

• Advances in pediatric surgery
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• v.9 no.1
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• pp.45-51
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• 2003

A nationwide survey on choledochal cyst was undertaken among 39 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and the case registration form for each patient during the five year period of 1997 to 2001. Three hundred and forty eight patients were registered from 32 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:3.4. The age of patients on diagnosis was $49.0{\pm}44.4$ months. The geographic distribution was 34.8% in Seoul and Kyoungki-do, 33.3% in Kyoungsang-do, 17.9% in Cholla-do, and 8.5% in Choongchung-do, in order of frequency. The three common clinical presentations were abdominal pain (63.8%), vomiting (35.3%), and jaundice (29.1%). Only seven patients (2%) presented with classic triad, and 25 patients were diagnosed by antenatal ultrasonographic examination. According to the Todani Classification, 238 patients (7l.3%) were type 1, 3 (0.9%) type 11, and 93 (27.8%) type IV. At the time of the operation, three important associated conditions were choledocholithiasis in 45 patients (15.1%), liver fibrosis (Grade 1-4) in 35, and previous operative procedure for biliary diseases in 10. Associated anomalies were observed in 13 patients (3.8%). Three hundred thirty nine (98.8%) of 343 lesions were treated by Cyst excision and Roux-Y hepaticoiejunostomy. One hundred seventy-six patients had an anomalous arrangement of the pancreatobiliary ductal system (APBD): APBD was not in 92 patients, biliary duct joined to the pancreatic duct in 51, and pancreatic duct joined to the biliary duct in 26. There were 8.5% early, and 7.7% late phase operative complications. The major complications were bleeding, anastomotic leakage, and acute pancreatitis. The combination of acute abdomen and choledochal cyst may suggest spontaneous rupture. Because of the development of late intrahepatic bile duct stones, long term follow up after cyst excision and hepaticojejunostomy is required. The optimal time of surgical intervention should also be considered in the situation of routine use of antenatal ultrasonographic examination. This is the first review of the choledochal cyst in Korea and provides baseline data for future comparisons.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.

• Korean Journal of Poultry Science
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• v.46 no.1
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• pp.31-37
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• 2019

A chicken clathrin-associated adaptor protein $3-{\delta}$ subunit 2 (AP3S2) is a subunit of AP3, which is involved in cargo protein trafficking to target membrane with clathrin-coated vesicles. AP3S2 may play a role in virus entry into host cells through clathrin-dependent endocytosis. AP3S2 is also known to participate in metabolic disease developments of progressions, such as liver fibrosis with hepatitis C virus infection and type 2 diabetes mellitus. Chicken AP3S2 (chAP3S2) gene was originally identified as one of the differentially expressed genes (DEGs) in chicken kidney which was fed with different calcium doses. This study aims to characterize the molecular characteristics, gene expression patterns, and transcriptional regulation of chAP3S2 in response to the stimulation of Toll-like receptor 3 (TLR3) to understand the involvement of chAP3S2 in metabolic disease in chicken. As a result, the structure prediction of chAP3S2 gene revealed that the gene is highly conserved among AP3S2 orthologs from other species. Evolutionarily, it was suggested that chAP3S2 is relatively closely related to zebrafish, and fairly far from mammal AP3S2. The transcriptional profile revealed that chAP3S2 gene was highly expressed in chicken lung and spleen tissues, and under the stimulation of poly (I:C), the chAP3S2 expression was down-regulated in DF-1 cells (P<0.05). However, the presence of the transcriptional inhibitors, BAY 11-7085 (Bay) as an inhibitor for nuclear factor ${\kappa}B$ ($NF{\kappa}B$) or Tanshinone IIA (Tan-II) as an inhibitor for activated protein 1 (AP-1), did not affect the expressional level of chAP3S2, suggesting that these transcription factors might be dispensable for TLR3 mediated repression. These results suggest that chAP3S2 gene may play a significant role against viral infection and be involved in TLR3 signaling pathway. Further study about the transcriptional regulation of chAP3S2 in TLR3 pathways and the mechanism of chAP3S2 upon virus entry shall be needed.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.1 no.1
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• pp.56-78
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• 1998

Objective: Chronic hepatitis B infection (CHB) occurs in 6% to 10% of population in Korea. In ethinic communities where prevalence of chronic infection is high such as Korea, transmission of hepatitis B infection is either vertical (ie, by perinatal infection) or by close family contact (usually from mothers or siblings) during the first 5 years of life. The development of chronic hepatitis B infection is increasingly more common the earlier a person is exposed to the virus, particularly in fetal and neonatal life. And it progress to cirrhosis and hepatocellular carcinoma, especially in severe liver damage and perinatal infection. Histopathology of CHB is important when evaluating the final outcomes. A numerical scoring system which is a semiquantitatively assessed objective reproducible classification of chronic viral hepatitis, is a valuable tool for statistical analysis when predicting the outcome and evaluating antiviral and other therapies. In this study, a numerical scoring system (Ludwig system) was applied and compared with the conventional histological classification of De Groute. And the comparative analysis of cinical findings, family history, serology, and liver function test by histopathological findings in chronic hepatitis B of children was done. Methods: Ninety nine patients [mean age=9 years (range=17 months to 16 years)] with clinical, biochemical, serological and histological patterns of chronic HBV infection included in this study. Five of these children had hepatocelluar carcinoma. They were 83 male and 16 female children. They all underwent liver biopsies and histologic evaluation was performed by one pathologist. The biopsy specimens were classified, according to the standard criteria of De Groute as follows: normal, chronic lobular hepatitis (CLH), chronic persistent hepatitis (CPH), mild to severe chronic active hepatitis (CAH), or active cirrhosis, inactive cirrhosis, hepatocellular carcinoma (HCC). And the biopsy specimens were also assessed and scored semiquantitatively by the numerical scoring Ludwig system. Serum HBsAg, anti-HBs, HBeAg, anti-HBe, anti-HBc (IgG, IgM), and HDV were measured by radioimunoassays. Results: Male predominated in a proportion of 5.2:1 for all patients. Of 99 patients, 2 cases had normal, 2 cases had CLH, 22 cases had CPH, 40 cases had mild CAH, 19 cases had moderate CAH, 1 case had severe CAH, 7 cases had active cirrhosis, 1 case had inactive cirrhosis, and 5 cases had HCC. The mean age, sex distribution, symptoms, signs, and family history did not differ statistically among the different histologic groups. The numerical scoring system was correlated well with the conventional histological classification. The histological activity evaluated by both the conventional classification and the scoring system was more severe as the levels of serum aminotransferases were higher. In contrast, the levels of serum aminotransferases were not useful for predicting the degree of histologic activity because of its wide range overlapping. When the histological activity was more severe and especially the cirrhosis more progressing, the prothrombin time was more prolonged. The histological severity was inversely related with the duration of seroconversion of HBeAg. Conclusions: The histological activity could not be accurately predicted by clinical and biochemical findings, but by the proper histological classification of the numerical scoring system for the biopsy specimen. The numerical scoring system was correlated well with the conventional histological classification, and it seems to be a valuable tool for the statistical analysis when predicting the outcome and evaluating effects of antiviral and other therapies in chronic hepatitis B in children.

• Tuberculosis and Respiratory Diseases
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• v.42 no.3
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• pp.277-294
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• 1995

Background: The prevalence of tuberculosis in Korea decreased remarkably for the past 30 years, while the incidence of disease caused by mycobacteria other than tuberculosis is unknown. Korean Academy of Tuberculosis and Respiratory Diseases performed national survey to estimate the incidence of mycobacterial diseases other than tuberculosis in Korea. We analyzed the clinical data of confirmed cases for the practice of primary care physicians and pulmonary specialists. Methods: The period of study was from January 1981 to October 1994. We collected the data retrospectively by correspondence with physicians in the hospitals that referred the specimens to Korean Institute of Tuberculosis, The Korean National Tuberculosis Association for the detection of mycobacteria other than tuberculosis. In confirmed cases, we obtained the records for clinical, laboratory and radiological findings in detail using protocols. Results: 1) Mycobacterial diseases other than tuberculosis were confirmed that 1 case was in 1981, 2 cases in 1982, 4 cases in 1983, 2 cases in 1984, 5 cases in 1985, 1 case in 1986, 3 cases in 1987, 1 case in 1988, 6 cases in 1989, 9 cases in 1990, 14 cases in 1990, 10 cases in 1992, 4 cases in 1993, and 96 cases in 1994. Cases since 1990 were 133 cases(84.2%) of a total. 2) Fifty seven percent of patients were in the age group of over 60 years. The ratio of male to female patients was 2.6:1. 3) The distribution of hospitals in Korea showed that 61 cases(38.6%) were referred from Double Cross Clinic, 42 cases(26.6%) from health centers, 21 cases(13.3%) from tertiary referral hospitals, 15 cases(9.5%) from secondary referral hospitals, and 10 cases(6.3%) from primary care hospitals. The area distribution in Korea revealed that 98 cases(62%) were in Seoul, 17 cases(10.8%) in Gyeongsangbuk-do, 12 cases(7.6%) in Kyongki-do, 8 cases(5.1%) in Chungchongnam-do, each 5 cases(3.2%) in Gyeongsangnam-do and Chungchongbuk-do, 6 cases(3.8%) in other areas. 4) In the species of isolated mycobacteria other than tuberculosis, M. avium-intracellulare was found in 104 cases(65.2%), M. fortuitum in 20 cases(12.7%), M. chelonae in 15 cases(9.5%), M. gordonae in 7 cases(4.4%), M. terrae in 5 cases(3.2%), M. scrofulaceum in 3 cases(1.9%), M. kansasii and M. szulgai in each 2 cases(1.3%), and M. avium-intracellulare coexisting with M. terrae in 1 case(0.6%). 5) In pre-existing pulmonary diseases, pulmonary tuberculosis was 113 cases(71.5%), bronchiectasis 6 cases(3.8%), chronic bronchitis 10 cases(6.3%), and pulmonary fibrosis 6 cases(3.8%). The timing of diagnosis as having pulmonary tuberculosis was within 1 year in 7 cases(6.2%), 2~5 years ago in 32 cases(28.3%), 6~10 years ago in 29 cases(25.7%), 11~15 years ago in 16 cases(14.2%), 16~20 years ago in 15 cases (13.3%), and 20 years ago in 14 cases(12.4%). Duration of anti-tuberculous treatment was within 3 months in 6 cases(5.3%), 4~6 months in 17 cases(15%), 7~9 months in 16 cases(14.2%), 10~12 months in 11 cases(9.7%), 1~2 years in 21 cases(18.6%), and over 2 years in 8 cases(7.1%). The results of treatment were cure in 44 cases(27.9%) and failure in 25 cases(15.8%). 6) Associated extra-pulmonary diseases were chronic liver disease coexisting with chronic renal failure in 1 case(0.6%), diabetes mellitus in 9 cases(5.7%), cardiovascular diseases in 2 cases(1.3%), long-term therapy with steroid in 2 cases(1.3%) and chronic liver disease, chronic renal failure, colitis and pneumoconiosis in each 1 case(0.6%). 7) The clinical presentations of mycobacterial diseases other than tuberculosis were 86 cases (54.4%) of chronic pulmonary infections, 1 case(0.6%) of cervical or other site lymphadenitis, 3 cases(1.9%) of endobronchial tuberculosis, and 1 case(0.6%) of intestinal tuberculosis. 8) The symptoms of patients were cough(62%), sputum(61.4%), dyspnea(30.4%), hemoptysis or blood-tinged sputum(20.9%), weight loss(13.3%), fever(6.3%), and others(4.4%). 9) Smear negative with culture negative cases were 24 cases(15.2%) in first examination, 27 cases(17.1%) in second one, 22 cases(13.9%) in third one, and 17 cases(10.8%) in fourth one. Smear negative with culture positive cases were 59 cases(37.3%) in first examination, 36 cases (22.8%) in second one, 24 cases(15.2%) in third one, and 23 cases(14.6%) in fourth one. Smear positive with culture negative cases were 1 case(0.6%) in first examination, 4 cases(2.5%) in second one, 1 case (0.6%) in third one, and 2 cases(1.3%) in fourth one. Smear positive with culture positive cases were 48 cases(30.4%) in first examination, 34 cases(21.5%) in second one, 34 cases(21.5%) in third one, and 22 cases(13.9%) in fourth one. 10) The specimens isolated mycobacteria other than tuberculosis were sputum in 143 cases (90.5%), sputum and bronchial washing in 4 cases(2.5%), bronchial washing in 1 case(0.6%). 11) Drug resistance against all species of mycobacteria other than tuberculosis were that INH was 62%, EMB 55.7%, RMP 52.5%, PZA 34.8%, OFX 29.1%, SM 36.7%, KM 27.2%, TUM 24.1%, CS 23.4%, TH 34.2%, and PAS 44.9%. Drug resistance against M. avium-intracellulare were that INH was 62.5%, EMB 59.6%, RMP 51.9%, PZA 29.8%, OFX 33.7%, SM 30.8%, KM 20.2%, TUM 17.3%, CS 14.4%, TH 31.7%, and PAS 38.5%. Drug resistance against M. chelonae were that INH was 66.7%, EMB 66.7%, RMP 66.7%, PZA 40%, OFX 26.7%, SM 66.7%, KM 53.3%, TUM 53.3%, CS 60%, TH 53.3%, and PAS 66.7%. Drug resistance against M. fortuitum were that INH was 65%, EMB 55%, RMP 65%, PZA 50%, OFX 25%, SM 55%, KM 45%, TUM 55%, CS 65%, TH 45%, and PAS 60%. 12) The activities of disease on chest roentgenogram showed that no active disease was 7 cases(4.4%), mild 20 cases(12.7%), moderate 67 cases(42.4%), and severe 47 cases(29.8%). Cavities were found in 43 cases(27.2%) and pleurisy in 18 cases(11.4%). 13) Treatment of mycobacterial diseases other than tuberculosis was done in 129 cases(81.7%). In cases treated with the first line anti-tuberculous drugs, combination chemotherapy including INH and RMP was done in 86 cases(66.7%), INH or RMP in 30 cases(23.3%), and not including INH and RMP in 9 cases(7%). In 65 cases treated with the second line anti-tuberculous drugs, combination chemotherapy including below 2 drugs were in 2 cases(3.1%), 3 drugs in 15 cases(23.1%), 4 drugs in 20 cases(30.8%), 5 drugs in 9 cases(13.8%), and over 6 drugs in 19 cases (29.2%). The results of treatment were improvement in 36 cases(27.9%), no interval changes in 65 cases(50.4%), aggravation in 4 cases(3.1%), and death in 4 cases(3.1%). In improved 36 cases, 34 cases(94.4%) attained negative conversion of mycobacteria other than tuberculosis on cultures. The timing in attaining negative conversion on cultures was within 1 month in 2 cases(1.3%), within 3 months in 11 cases(7%), within 6 months in 14 eases(8.9%), within 1 year in 2 cases(1.3%) and over 1 year in 1 case(0.6%). Conclusion: Clinical, laboratory and radiological findings of mycobacterial diseases other than tuberculosis were summarized. This collected datas will assist in the more detection of mycobacterial diseases other than tuberculosis in Korea in near future.