• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.504-507
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• 2009

Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are chronic autoimmune liver diseases. Overlap syndrome is defined as a condition in which the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. Only a few cases of the overlap syndrome of AIH and PSC have been reported, especially in children. Moreover, PSC is known to be the most frequent liver disorder associated with inflammatory bowel diseases such as ulcerative colitis. We report one case of AIH-PSC overlap syndrome in a child who was diagnosed as having ulcerative colitis.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.473-481
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• 2002

Purpose : Ascending cholangitis is the most common complication after Kasai operations. The aim of this study is to evaluate the therapeutic efficacy of cefotaxime as an empirical antibiotic on ascending cholangitis after Kasai operations. Methods : Thirty-nine episodes of cholangitis in twenty-nine children who underwent Kasai operations at Seoul National University Children's Hospital from January 1991 to December 2000 were included in this study. Empirical cefotaxime treatments were divided into three groups : cefotaxime and amikacin treatment group(CA group), cefotaxime and gentamicin treatment group(CG group) and cefotaxime treatment group(C group). A diagnosis of cholangitis was made on the basis of unexplained fever(>$38^{\circ}C$) and either development of acholic stool or elevation of serum total bilirubin (>1.5 mg/dL). Therapeutic efficacy was judged by elimination of fever up to 72 hours, 120 hours, and 168 hours after antibiotic treatment. Results : There were therapeutic responses in 51%(20/39) up to 72 hours after antibiotic treatment : 54%(13/24) in CA group, 43%(3/7) in CG group and 50%(4/8) in C group. There were therapeutic responses in 69%(27/39) up to 120 hours, in 79%(31/39) up to 168 hours and in 82%(32/39) up to 2 weeks. There were no differences in therapeutic efficacy among the three regimens. In 12 of 39 episodes, the etiologic pathogens including Escherichia coli and enterococcus were cultured from the blood. Conclusion : Cefotaxime can be tried as an initial antibiotic in Korean children with ascending cholangitis after Kasai operation prior to the identification of microorganism on culture. However, further evaluation of pathogen and its resistant strain to cefotaxime should be done.

• Journal of Yeungnam Medical Science
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• v.3 no.1
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• pp.351-355
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• 1986

Sclerosing Cholangitis is an uncommon disease which involves either all or part of the extrahepatic biliary duct system and, occassionally, affects the intrahepatic biliary radicles. The disease has also been called "obliterative cholangitis" and "stenosing cholangitis", in reference to a progressive thickening of the bile duct walls encroaching upon the lumen. Several authors have proposed that the temr "primary sclerosing cholangitis" be reserved for cases in which there arew no associated diseases, and that all other cases be classified as "secondary sclerosing cholangitis". Many rigid criterias have been established for the diagnosis of primary sclerosing cholangitis. Recently the authors experienced one case of primary sclerosing cholangitis which was coincided with rigid criterias and was confirmed by operation with histologic examination. We present our case and review the literatures.

• Advances in pediatric surgery
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• v.18 no.1
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• pp.1-11
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• 2012

담관 낭종은 흔하지는 않지만, 외과적 절제가 필요한 주요 질환이다. 절제하지 않고 남겨두면 다른 질환으로의 이환이나 다양한 합병증에 의한 사망까지도 야기할 수 있기 때문에 적절한 수술적 치료가 필수적이다. 최근 수술에 따른 다양한 문제들이 계속 보고되고 있으며, 출생 전이나 건강 검진 시 발견되는 무증상의 담관 낭종의 수술 시기에 대해서도 아직 논란이 되고 있다. 저자들은 1995년부터 2009년까지 담관 낭종으로 수술 받은 환자 중 수술 받을 당시의 연령이 18세 이하인 32명 환자의 임상 양상과 수술 기록을 후향적으로 분석하여, 수술 성적에 영향을 주는 요인들을 알아보고자 하였다. 전체 32명 중 남자 10명, 여자 22명이었으며, 수술 당시의 평균 연령은 5.4세였다. 췌담관 합류이상은 9명(28.1%)이었으며, 평균 추적 관찰 기간은 34.6개월이었다. 술 전 증상을 호소한 환자는 30명(93.8%)이었으며, 복통(63.3%), 황달(40.0%), 구토(23.3%), 복부종물(16.7%), 발열(16.7%)등의 순서였다. 술 전 혈액 검사에서 AST/ALT의 상승이 18명(56.3%), 고빌리루빈혈증이 8명, 백혈구 증가증이 7명(21.9%)이었다. 술 후 합병증은 5명(15.6%)에서 나타났는데, 2명은 담관염이었고, 나머지 3명은 문합부 협착, 술 후 출혈, 장 폐쇄가 각각 1명 있었다. 술 후 평균 재원 기간은 12.2일 이었으며, 추적 관찰 기간 중 3명(9.0%)이 재입원 하였다. 수술 성적에 영향을 미치는 요인으로, 수술 당시의 나이가 많을수록 평균 수술 시간과 재원 기간이 증가하였다(p=0.004, p=0.028). 증상이 있었던 환자군에서 무증상 환자군보다 재원 기간이 더 길었다(p=0.001). 또한 췌담관 합류이상이 있었던 환자군에서 재입원율이 유의하게 높았다(p=0.005). 소아의 담관 낭종은 진단이 되면 증상 발현 전이라도 조기에 수술하는 것이 수술 시간 및 재원 기간을 단축시킬 수 있으며, 췌담관 합류이상이 동반된 경우에는 술 후에 재입원할 가능성이 높으므로 주의깊게 추적 관찰하는 것이 바람직할 것이다.

• The Korean Journal of Nuclear Medicine
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• v.19 no.2
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• pp.25-41
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• 1985

간흡충증은 한국 및 동남아세아에 널리 분포되어 있는 중요한 풍토병의 하나로서 진단은 전통적으로 분변검사에 의존하여 오고 있다. 최근 들어 담관내 간흡충 및 이의 합병증의 진단을 위해 ERCP 및 contrast cholangiogram등이 시도되었으나 실제에 이용하기에는 많은 제약이 있다 하겠다. 저자는 간흡충증에 있어서 최근 소개된 $^{99m}Tc-DISIDA$ hepatobiliary scintigraphy의 진단적 의의를 규명하고자 1982년부터 1983년까지 고신의대 부속 복음병원에서 검진한 간흡충증 환자 90명을 대상으로 하여 $^{99m}Tc-DISIDA$ hepatobiliary scintigraphy와 formalin-ether 원침법에 의한 분변검사를 시행하였다. $^{99m}Tc-DISIDA$ scintigram소견은 간내 담관 bile flow 및 총수담관 bile flow defect, 그리고 간세포기능의 정도에 따라 자료를 분석하였고 그외 합병증의 진단은 병록 기록, 수술 소견, ERCP 등에 의존하여 결론을 얻었다. $^{99m}Tc-DISIDA$ scintigraphy는 특이한 양상의 bile flow dynamics를 나타내었으며 간내 주담관의 intermittent irregular focal bile flow defect 및 tile flow stasis를 나타내고 말초담관의 bile flow defect는 경미하고 60분 내에 담관 bile flow activity의 완전한 배설을 나타내는 경우를 mild pattern, 간내 담관의 심한 irregular bile flow dynamics 및 간내담관의 심한 irregularity (담관내벽의 심한 불규칙성), 총수담관, 간내 주담관 및 말초 담관까지 심하게 irregular bile flow stasis를 나타내며 bile flow activity의 완전 베설이 $60\sim90$분사이 혹은 90분이상까지 인지된 경우를 moderate-severe pattern으로 분류하였다. 1) 분변검사상 간흡충증은 95검사중 70검사(환자 86명중 67명)에서 양성을 보여 분변 충난검사의 양성율은 73.7%였고 음성율은 26.3%였다. 2) $^{99m}Tc-DISIDA$ hepatobiliary scintigraphy는 90명중 70명에서 특이한 Cs-bile flow양성을 보였으며 양성율은 77.8%였으며 음성율은 22.2%였다. 3) $^{99m}Tc-DISIDA$ hepatobiliary scintigram양성율을 나타낸 환자 70명중 11명은 mild pattern, 59명은 moderate-severe pattern을 나타냈으며 그중 21명은 여러가지 간세포 기능 및 담관에 영향을 미치는 질환과 합병했지만 특이한 Cs-bile flow pattern을 dominent하게 나타내었으며 합병된 여러 질환들도 bile flow pattern상 인지 할 수 있었다. 4) $^{99m}Tc-DISIDA$ hepatobiliary scintigram 음성율을 나타낸 환자 20명중 8명은 만성간염, 5명은 간경변증, 3명은 재발성 농양성담관염(recurrent pyogenic cholangitis)과 간내담도의 stricture 및 담관담석증이 합병되었으며 scintigram상 합병증의 pattern을 나타냈고 4명에서는 low CBD obstruction을 나타내었으며 후에 CBD stone, CBD carcinoma, gall bladder Ca.의 porta hepatis 전이 및 clonorchis worms의 cluster에 의한 obstruction이 operation 및 ERCP로서 진단 되었다. 5) $^{99m}Tc-DISIDA$ hepatobiliary scintigraphy pattern은 현재의 자각증상과 관계된 dominent disease를 나타내었으며, 공간 점유병소도 multiple project images를 시행하므로서 쉽게 발견할 수 있었다. 이와 같이 간흡충증에 있어서 $^{99m}Tc-DISIDA$ hepatobiliary scintigram은 환자의 자각증상과 관계된 질환을 규명하는 데 필요한 정보를 얻었을 수 있었으며 간내담관의 damage정도를 규명하는데 필요한 procedure임이 판명되었다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.91-97
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• 2007

The Overlap syndrome is characterized by a combination of the major hepatobiliary autoimmune diseases such as autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis. It is frequently accompanied by inflammatory bowel disease. Chronic lymphoplasmacellular osteomyelitis is characterized by recurrent episodes of bacterial osteomyelitis and is associated with autoimmune diseases (especially inflammatory bowel disease). We report the case of a girl who was diagnosed with ulcerative colitis and autoimmune hepatitis at 4 years of age and with the overlap syndrome with primary sclerosing cholangitis at 6 years. At 9 years, she was diagnosed with chronic lymphoplasmacellular osteomyelitis.

• Parasites, Hosts and Diseases
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• v.32 no.4
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• pp.223-230
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• 1994

Clonorchiasis is an important parasitic disease of humans in Korea. The present study intended to compare sonographic findings with histopathological changes in experimental clonorchiasis. Eighteen New Zealand white rabbits were infected with metacercariae of Cknorchis sinensis, and examined 4, 10, and 22 weeks post-infection (PI). Four infected rabbits were treated with praziquantel 10 weeks PI and were examined 12 weeks after treatment. Sonography revealed mild to severe dilatation of the intrahepatic ducts (IHDD) and slightly increased periductal echoes in 12 out of 14 rabbits at 4 weeks PI, and all of the animals after 10 and 22 weeks PI and 12 weeks after treatment. The histopathological lesions were duct dilatation, mucosal hyperplasia, and periductal fibrosis, which progressed from 4 weeks to 22 weeks PI and even in treated rabbits. The dilated intrahepatic ducts over 1 mm diameter were detected by sonography. The present results indicate that sonographic findings are well correlated with histopathological lesions in rabbit clonorchiasis except for early phase of light burden of infection. The sonography has a limitation in discriminating residual sequelae of the ducts after praziquantel treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.120-124
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• 2001

Sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation and obliterative fibrosis of the bile ducts, leading to biliary cirrhosis and ultimately to liver failure. In children, sclerosing cholangitis is known to be associated with Langerhans cell histiocytosis, as well as with congenital immunodeficiencies and cystic fibrosis. Secondary sclerosing cholangitis is suspected in Langerhans cell histiocytosis with chronic cholestasis, liver dysfunction and portal hypertension. Unlike primary sclerosing cholangitis, the cholangitis associated Langerhans cell histiocytosis is destructive in nature and progresses more rapidly to biliary cirrhosis, therefore uniformly the prognosis is poor. In this setting, liver transplantation should be considered early in children with sclerosing cholangitis complicating Langerhans cell histiocytosis before end-stage liver failure and variceal bleeding. We experienced a case of secondary sclerosing cholangitis in Langerhans cell histiocytosis in a 2-year-old boy. We report this case with brief review of the related literatures.

• Parasites, Hosts and Diseases
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• v.31 no.1
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• pp.13-20
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• 1993

The histological change of the billary mucosa in clonorchiasis is characterized as adenomatous hyperplasia, and cross-sectioned mucosa looks like intestinal mucosa. In addition to the glandular hyperplasla, the metaplasia of mucin secreting cells is also known. The present study investigated the presence of intestinal secretion from the biliary mucosal cells of rabbits and rats with Clonorchis sinensis infection. The rabbit was infected with 300 and the rat was infected with 100 metacercariae of C. sinensif. A part of the animals were followed up after praziquantel treatment. The rabbit livers were prepared for histochemistry to observe any endocrine secretion and the bile duct mucosa of the mice was processed far the activity of brush border membrane (BBM)-bound enzymes of the small intestine. Immunohistochemistry with the polyclonal antibodies and biotin-streptavidin-peroxidase staining kit showed no positive cells for gastrin and secretin, but a few cells were positive for serotonin. The proliferated billiw mucosa of the mice revealed no activity of dissaccharidases and aminopeptidase. Only alkaline phosphatase activity was found both in the control and the infected. The hyperplastic billary mucosal cells showed no gastrointestinal secretory functions. The serotonin secreting cells may be one of the inflammatory cells.

• Applied Microscopy
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• v.39 no.2
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• pp.117-124
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• 2009

Clonorchis sinensis is the most important widely distributed parasite of the human bile duct in East Asia and the most prevalent parasitic helminth in Korea. The prevalence rate of human clonorchiasis has remained at about 2.9% in Korea. C. sinensis induces dilatation of the duct, hyperplasia of the mucosa, metaplasia or neoplasia of the mucosal epithelium, periductal inflammation and fibrosis, and thickening of the ductal wall. Fibroblast are the most common cells in connective tissue and are responsible for the synthesis of extracellular matrix components. The fibrosis associated with chronic inflammation and injury may also contribute to cholangiocarcinoma pathogenesis, particularly through an increase in extracellular matrix components, which participate in the regulation of bile duct differentiation during development. In this study, ultrastructural changes, the distribution of lectin receptors and actin protein in cultured SD rat bile duct fibroblast after infection of C. sinensis were observed. Experimental group had been divided into four groups: normal bile duct fibroblast cultured in basal media (G1); C. sinensis infected bile duct fibroblast cultured in basal media (G2); normal bile duct fibroblast cultured in basal media containing excretory-secretory product (ESP) (G1-1); C. sinensis infected bile duct fibroblast cultured in basal media containing ESP (G2-1). Overall, once a host is infected by C. sinensis, it affects the host to the extent that sialic acid of ductal fibroblast is increased. Number of cytoplasmic process of SD rat bile duct fibroblast was increased. Actin protein and sialic acid were located in cell surface. Fibroblast induced by C. sinensis was not recovered to normal fibroblast. The cytoplasm bulk and cytoplasmic process were increased whereas the growth rate of the fibroblast of infected SD rat was reduced rather than that of normal fibroblast. In result, it inhibits fibroblast proliferation and increases actin protein on fibroblast cytoplasm, and so causes fibroblast metamorphosis and cellular mutation.