· Chronic liver diseases in children are heterogenous but converge in the common pathway of fibrosis. · Much of the literature on mechanisms of fibrogenesis focus on adults but pediatric physiology has documented differences. · Understanding of these distinctions are necessary to define, treat, and prevent fibrosis. · Current management of liver fibrosis relies heavily on liver biopsy. Multiple tools have shown high diagnostic performance in pediatric and adult populations. Large, multicenter studies are needed for validation. |
Purpose : To assess the usefulness of 99mTc-DISIDA scanning in the early evaluation of neonatal cholestasis and to verify the diagnostic value of this test in the differential diagnosis of biliary atresia. Methods : DISIDA scannings were performed and analyzed in 87 children(58 males and 29 females; age, 18-139 days, mean, 59.1 days) with neonatal cholestasis. Five groups according to the... |
Purpose : Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. Methods : From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and... |
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... |
Purpose : To evaluate the usefulness of magnetic resonance cholangiography(MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods : Fifty consecutive infants with cholestatic jaundice underwent single-shot MRC for 3 years. The radiologic diagnosis of non-biliary atresia with MRC was based on visualization of the common bile duct and common hepatic duct. The diagnosis of biliary atresia was... |
Purpose : Extrahepatic biliary atresia is the most common indication for liver transplantation in children, but the etiology of this disorders remains unknown. It would be very signficant to identify genes that are specifically expressed in pathologic liver tissue of biliary atresia and analyze the pattern of expression in those genes. Methods : We made dot blot panels consisting of 1,730 different EST(expressed sequence... |
Purpose : Neonatal hepatitis and congenital extrahepatic biliary atresia are two major causes of neonatal cholestasis. But the method of therapeutic trials used for each disease is essentially different. Nonetheless, it is very difficult to differentiate these diseases clinically and histologically. This study is aimed to find out major differences between the two by clinical characteristics and scoring of various... |
Purpose : The surgical results for congenital extrahepatic biliary atresia have been improved since Kasai first described hepatic portoenterostomy(Kasai operation) in 1959. This study was conducted to evaluate the postoperative prognosis of congenital extrahepatic biliary atresia after hepatic portoenterostomy. Methods : This clinical study was done by reviewing the medical records of 30 patients who have undergone hepatic portoenterostomy at the Severance Hospital, Yonsei... |
Together, neonatal hepatitis and biliary atresia make up approximately 70 to 80% of the causes of neonatal cholestasis. Biliary atresia must be differentiated from neonatal hepatitis as soon as possible in order to institute early surgical intervention. We performed this study to examine whether the measurement of the serum α-fetoprotein(AFP) and thyroxine-binding globulin(TBG) was useful for differentiating these two conditions.... |
Watson-Alagille syndrome is a kind of familial intrahepatic biliary atresia associated with charac- teristic face and cardiovascular anomaly. Growth and developmental delay, anomalies of the eyes such as posterior embryotoxon, deformities of the skeletal system are often the manifestations of this syndrome. We experienced three cases of Watson-Alagille syndrome recently. These patients showed charac- teristic faces and had peripheral pulmonary artery stenosis diagnosed by pulmonary... |
A clinical assessment of the 30 patients with extrahepatic biliary atresia who were admitted to the Department of Pediatrics and pediatric Surgery, Yonsei University College of Medicine from January, 1979 to January, 1987 was performed. In 21 patients who underwent hepatic portoenterostomy, the relationship between outcome and surgery was analyzed. Finally, collelations between lonterm prognosis and the histopathology of biopsied liver tissue, postoperative bile... |
Hepatobiliary scintigraphy with 99mTc-DISIDA was used to evaluate neonates with direct hyperbilirubinemia who were admitted to the department of Pediatrics, Maryknoll hospital, between May 1981 and Jun. 1983. 1)Total number of patients were 10, boys to girls ratio was 4:1, and age distribution ranging from 9 to 200 days of age. 2)Initial studies of the seven patients intrahepatic cholestasis showed definite... |
A clinical assessment was carried out in 37 cases with Extrahepatic biliary atresia (EHBA) and 35 cases with Neonatal hepatitis (NH), who were admitted to the Dept, of Pediatrics and Pediatric Surgery, Seoul National University Hospital, during the 30 months from January 1980 to June 1982. The following results were obtained; 1)By sex distribution, 40% of EHBA and 69% of NH... |
The following result WBS obtained through the clinical observation of 67 cases with neonatal hepatitis, 7 cases with extrahepaticbiliary atresia, 1 case with intrahepatic biliary atresia admitted at Department of Pediatrics, Yonsei University Hospital during the period of January, 1979 through October, 1982. The clinical & etiological study was performed by the Hepatitis B marker, Liver function test, Liver scan, pathologic findings by percutaneous liver... |