Disease relevance of Biliary Atresia

• Bone disease and low serum levels of 25-hydroxyvitamin D are prevalent in cholestatic syndromes such as primary biliary cirrhosis and biliary atresia [1].
• Elevation of IP-10 expression has been demonstrated in a number of human diseases, including chronic cirrhosis and biliary atresia [2].
• The most common indications for transplant were biliary atresia in children (56%) and hepatitis C in adults (40%); 35.6% of adults had hepatocellular carcinoma [3].
• The human Delta-like 1 homologue is implicated in the progression of liver fibrosis in biliary atresia [4].
• In 63 patients with malabsorption syndromes, 16 with congenital biliary atresia (BA) and 47 with cystic fibrosis (CF), axonal dystrophy in the gracile nucleus (ADG) was studied [5].

High impact information on Biliary Atresia

• Here, we address the hypothesis that production of IFN-gamma is a key pathogenic mechanism of disease using a mouse model of rotavirus-induced biliary atresia [6].
• These observations suggest that the carbohydrate-binding specificity of a virus can dramatically alter disease in the host and highlight the need for epidemiologic studies focusing on infection by sialic acid-binding reovirus strains as a possible contributor to the pathogenesis of neonatal biliary atresia [7].
• The second was the time-restricted expression of genes regulating biological networks previously unrecognized in biliary atresia, such as the complement components C3ar-1 and C1q-alpha/beta [8].
• No B19 DNA was detected in serum from patients with other types of FH or from 82 patients with biliary atresia [9].
• Increased urinary dipeptidyl peptidase IV activity in extrahepatic biliary atresia [10].

Chemical compound and disease context of Biliary Atresia

• Ursodeoxycholic acid in biliary atresia [11].
• Liver levels of vitamin A and cellular retinol-binding protein for patients with biliary atresia [12].
• One patient improved clinically and electrophysiologically after oral therapy with vitamin E. The findings in these patients were similar to those in others recently reported with vitamin E deficiency associated with biliary atresia [13].
• Levels of vitamin A, as retinol and retinol esters, and of cellular retinol-binding protein have been determined in liver samples from 6 normal adults and 11 children with biliary atresia, with and without vitamin A treatment [12].
• Recognition of the importance of persistent jaundice beyond 2 weeks of age in the infant or elevation of the conjugated bilirubin more than 2 mg/dl will allow the timely evaluation of neonates with possible biliary atresia prior to the development of irreversible hepatic damage [14].

Biological context of Biliary Atresia

• Pharmacokinetics of bupivacaine after continuous epidural infusion in infants with and without biliary atresia [15].
• In children with extrahepatic biliary atresia, impaired hydroxylation and defective intestinal absorption of cholecalciferol may lead to a deficiency of vitamin D and rickets [16].
• Therefore we have searched for a specific anti-Reo-3 antibody response in the sera of patients with biliary atresia or neonatal hepatitis and for Reo-3 antigens in their hepatobiliary tissues [17].
• The developing human biliary system at the porta hepatis level between 29 days and 8 weeks of gestation: a way to understanding biliary atresia. Part 1 [18].

Anatomical context of Biliary Atresia

• Hepatocytes and sinusoidal lining cells including Kupffer cells showed a pattern of intense intercellular adhesion molecule-1 and vascular cell adhesion molecule-1 expression in all specimens with active inflammation that could not differentiate the biliary atresia cases from the control group [19].
• The nontumor liver counter parts, cirrhotic liver tissues from children with biliary atresia, and other types of cancers, such as bile duct, colon, stomach, lung, prostate, and kidney, were all negative for RBMY expression [20].
• These results suggest that TGF-beta and PDGF play important roles in the fibrogenesis of biliary atresia, especially in its early stage, acting either by autocrine or paracrine mechanisms involving activated fibroblasts/myofibroblasts, bile duct cells, and macrophages [21].
• As DLK1 is a negative regulator of adipocyte differentiation and may control cell fate during differentiation, overexpression of DLK1 protein in HSCs in the early stage of biliary atresia suggests that DLK1 may be implicated in the transformation of HSCs from fat-storing cells to myofibroblasts and in fibrogenesis associated with biliary atresia [4].
• Erythrocyte membrane cholesterol and phospholipid determinations from these patients were compared with six infants with extrahepatic biliary atresia and target-shaped erythrocytes and with five normal adults [22].

Gene context of Biliary Atresia

• Our results imply that MMP-7 is a major MMP associated with the tissue remodeling during the progression of liver fibrosis in biliary atresia [23].
• Establishment of the enzyme-linked immunosorbent assay for connective tissue growth factor (CTGF) and its detection in the sera of biliary atresia [24].
• Promoter polymorphism of the CD14 endotoxin receptor gene is associated with biliary atresia and idiopathic neonatal cholestasis [25].
• A Delta-like 1 homologue (DLK1) gene was identified and this gene was up-regulated during the early stage, and down-regulated during the late stage, of biliary atresia, similar to the expression pattern of the procollagen alpha1(I) gene [4].
• To determine the proinflammatory cytokine profile in children with biliary atresia, we measured circulating levels of interleukin (IL)-1beta, IL-6, tumor necrosis factor-alpha and IL-8 [26].

Analytical, diagnostic and therapeutic context of Biliary Atresia

• Treatment with ursodeoxycholic acid renders children with biliary atresia suitable for liver transplantation [27].
• Indications for transplantation were hepatitis C cirrhosis (69%), hepatitis B with or without C cirrhosis (15%), viral hepatitis related hepatocellular carcinoma (12%), and biliary atresia (4%) [28].
• Radionuclide imaging with the newer technetium-99m-labeled hepatobiliary radiopharmaceuticals appears promising for the noninvasive diagnosis of biliary atresia [29].
• Twenty-three consecutive infants suspected of having biliary atresia (BA) were prospectively examined by using mangafodipir trisodium (Mn-DPDP)-enhanced magnetic resonance (MR) cholangiography [30].
• Blood concentrations of manganese are elevated in patients with biliary atresia who have undergone hepatic portoenterostomy [31].

References