AnatomyOrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPhenomena and ProcessesNamed GroupsHealth Care
Biliary AtresiaCholestasisPortoenterostomy, HepaticCholestasis, IntrahepaticBile Ducts, ExtrahepaticCholestasis, ExtrahepaticBile DuctsFollicular AtresiaIntestinal AtresiaEsophageal AtresiaJaundice, NeonatalCholangitisJaundicePulmonary AtresiaBiliary TractLiverImino AcidsHepatitisBile Ducts, IntrahepaticChoanal AtresiaLiver TransplantationBilirubinTechnetium Tc 99m DisofeninInfant, NewbornCholedochal CystCholagogues and CholereticsLiver DiseasesUrsodeoxycholic AcidTricuspid AtresiaHepatic Duct, CommonBile Duct NeoplasmsBile Acids and SaltsTracheoesophageal FistulaLiver Function TestsBilePetromyzonHyperbilirubinemia1-NaphthylisothiocyanateSitus InversusCholangiographyAlagille SyndromeBiliary Tract DiseasesRotavirus InfectionsGallbladderRotavirusHypertension, PortalInfant, Newborn, DiseasesPortal SystemPostoperative ComplicationsSulbenicillinFlocculation TestsCholangiocarcinomaLiver CirrhosisLiving DonorsRetrospective StudiesTreatment OutcomeAnus, ImperforateJaundice, ObstructiveBile Canaliculi
Neonatal cholestasisObstructionCongenitalCholangitisCommon hepaCholelithiasisCirrhosisInfantsCholangiographyBilirubinPrimaryDisordersDiagnosisEtiologyEpithelial cellsFibrosisKasaiIntrahepatic biliaryPancreaticPorta hepatisDiseasesGallstonesPediatricManifestsDescriptorLiver diseaseOutcomesComplicationsMalnutrition2023Cholesterol saturationDuctsDisorderUDCA
Neonatal cholestasis6
Obstruction5
Congenital3
  • Patients with biliary atresia can be subdivided into 2 distinct groups: isolated biliary atresia (postnatal form) and associated situs inversus or polysplenia / asplenia with or without other congenital anomalies (fetal/embryonic form). (medscape.com)
  • The fetal/embryonic form of atresia is associated with other congenital anomalies. (medscape.com)
  • Congenital hepatic fibrosis results from a malformation of the ductal plate (the embryological precursor of the biliary system), secondary biliary strictures, and periportal fibrosis. (medscape.com)
Cholangitis4
  • Ursodiol and obeticholic acid are FDA-approved for the treatment of primary biliary cholangitis. (wikipedia.org)
  • UDCA is commonly used to treat patients with primary biliary cholangitis (formerly known as primary biliary cirrhosis), an immune-mediated cholestatic liver disease characterized by the destruction of intrahepatic bile ducts. (statpearls.com)
  • UDCA is the only drug approved by the U.S. Food and Drug Administration to treat primary biliary cholangitis. (statpearls.com)
  • Liver blood tests may, therefore, be appropriate if patients with autoimmune diseases develop symptoms that suggest liver disease, such as pruritus in primary biliary cholangitis. (medscape.co.uk)
Common hepa2
  • Type II: atresia of the common hepatic duct. (findmeacure.com)
  • The radiologic diagnosis of non-biliary atresia with MRC was based on visualization of the common bile duct and common hepatic duct. (e-cep.org)
Cholelithiasis3
  • UDCA has been used as medical therapy in gallstone disease (cholelithiasis) and for biliary sludge. (wikipedia.org)
  • UDCA may be given after bariatric surgery to prevent cholelithiasis, which commonly occurs due to the rapid weight loss producing biliary cholesterol oversaturation and also biliary dyskinesia secondary to hormonal changes. (wikipedia.org)
  • Solid crystalline precipitates in the BILIARY TRACT, usually formed in the GALLBLADDER, resulting in the condition of CHOLELITHIASIS. (lookformedical.com)
Cirrhosis6
  • previously known as primary biliary cirrhosis) where it can produce an improvement in biomarkers. (wikipedia.org)
  • If not surgically corrected, secondary biliary cirrhosis invariably results. (medscape.com)
  • As the biliary tract cannot transport bile to the intestine, bile is retained in the liver (known as stasis) and results in cirrhosis of the liver. (findmeacure.com)
  • Primary biliary cirrhosis is a rare, progressive, immuno-mediated hepatobiliary disease. (statpearls.com)
  • Cirrhosis may also occur in primary biliary cirrhosis . (wikidoc.org)
  • In the adult group the main etiology was represented by viral liver cirrhosis, whereas the glycogenosis and biliary atresia were the main indications for children. (deepdyve.com)
Infants8
  • Hepatobiliary scintiscanning is useful in evaluating infants with suspected biliary atresia. (medscape.com)
  • Although histopathologic features of biliary atresia have been extensively studied in surgical specimens from excised extrahepatic biliary systems of infants undergoing portoenterostomy, the pathogenesis of this disorder remains poorly understood. (medscape.com)
  • Cholestasis occurs in 1/2500 full-term infants. (msdmanuals.com)
  • Citrin deficiency can manifest in newborns or infants as neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), in older children as failure to thrive and dyslipidemia caused by citrin deficiency (FTTDCD), and in adults as recurrent hyperammonemia with neuropsychiatric symptoms in citrullinemia type II (CTLN2). (nih.gov)
  • Biliary atresia is a rare condition in newborn infants in which the common bile duct (that carry a liquid called bile from the liver to the gallbladder) between the liver and the small intestine is blocked or absent. (findmeacure.com)
  • The study population comprised infants aged 0-3 months with cholestasis who met the inclusion and exclusion criteria. (isainsmedis.id)
  • All infants less than 2 years old with cholestasis referred to the pediatric gastrointestinal ward were included in this study. (ac.ir)
  • Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE. (ucdenver.edu)
Cholangiography4
Bilirubin2
Primary2
Disorders3
  • These variants should not be confused with intrahepatic biliary hypoplasia, which comprises a group of distinct and surgically noncorrectable disorders. (medscape.com)
  • may result from extrahepatic or intrahepatic disorders, although some conditions overlap. (msdmanuals.com)
  • Some liver disorders such as biliary atresia, cholestasis, glycogen storage disease, cirrhotic diseases, etc. are also risk factors for HCC. (ccimindia.org)
Diagnosis2
Etiology2
Epithelial cells1
  • These "reactive" biliary epithelial cells in cholestasis, unlike normal condition, produce and secrete various cytokines such as CCL-2 or MCP-1, Tumor necrosis factor (TNF), Interleukin-6 (IL-6), TGF-beta, Endothelin (ET), and nitric oxide (NO). Among these, TGF-beta is the most important profibrogenic cytokine that can be seen in liver fibrosis in chronic cholestasis. (findmeacure.com)
Fibrosis3
  • Children younger than age one year have a history of low birth weight with growth restriction and transient intrahepatic cholestasis, hepatomegaly, diffuse fatty liver, and parenchymal cellular infiltration associated with hepatic fibrosis, variable liver dysfunction, hypoproteinemia, decreased coagulation factors, hemolytic anemia, and/or hypoglycemia. (nih.gov)
  • Biliary atresia is due to a progressive fibrosis or scarring of the bile ducts responsible for draining bile from the liver, which eventually leads to atresia or loss of the biliary system. (findmeacure.com)
  • However, there have been extensive studies about the pathogenesis and proper management of progressive liver fibrosis , which is arguably one of the most important aspects of biliary atresia patients. (findmeacure.com)
Kasai2
  • The discovery of metabolic liver diseases, bile acid synthesis defects, cryptogenic neonatal hepatitis syndromes, and the development of the Kasai portoenterostomy procedure for the treatment of biliary atresia, a condition up until then considered untreatable, were all factors that contributed to the foundation of the subspecialty of pediatric hepatology in the 1970s. (abdominalkey.com)
  • Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy. (ucdenver.edu)
Intrahepatic biliary1
  • The intrahepatic biliary tree functions to modify canalicular bile through secretory and reabsorptive processes in bile duct cholangiocytes. (jci.org)
Pancreatic1
  • Fiberoptic endoscopy designed for duodenal observation and cannulation of VATER'S AMPULLA, in order to visualize the pancreatic and biliary duct system by retrograde injection of contrast media. (lookformedical.com)
Porta hepatis1
  • 90% of patients) involves atresia of the right and left hepatic ducts to the level of the porta hepatis. (medscape.com)
Diseases2
Gallstones2
  • A clinical study in which patients with idiopathic acute pancreatitis attributed to microscopic gallstones or biliary sludge showed complete resolution of gallbladder microlithiasis after UDCA treatment, demonstrating its therapeutic effect. (statpearls.com)
  • Presence or formation of GALLSTONES in the BILIARY TRACT, usually in the gallbladder (CHOLECYSTOLITHIASIS) or the common bile duct (CHOLEDOCHOLITHIASIS). (lookformedical.com)
Pediatric3
  • Roux-en-Y cholangiojejunostomy is a standard procedure for biliary reconstruction in pediatric living donor liver transplantation (LDLT). (bvsalud.org)
  • However, there is uncertainty on whether the adult standard of Roux branch limb is suitable for pediatric LDLT and its impact on postoperative biliary complications (BC). (bvsalud.org)
  • This study aimed to explore the effect of the short Roux limb and standard limb on pediatric LDLT biliary reconstruction. (bvsalud.org)
Manifests1
Descriptor1
  • Biliary Atresia" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (ucdenver.edu)
Liver disease1
Outcomes3
  • Background: Outcomes in biliary atresia (BA) have been well-documented in large national cohorts from Europe, North America, and East Asia. (bvsalud.org)
  • Posttransplant management has also improved, including immunosuppression, earlier recognition of rejection and infection, and earlier recognition of vascular and biliary complications, all of which have contributed to improved outcomes. (musculoskeletalkey.com)
  • Malnutrition in Biliary Atresia: Assessment, Management, and Outcomes. (ucdenver.edu)
Complications1
  • The incidences of postoperative biliary tract complications between the 2 groups were compared retrospectively. (bvsalud.org)
Malnutrition1
20231
  • An observational analytical study was conducted cross-sectionally, using medical records of cholestasis patients treated at RSUP Prof. Dr. I.G.N.G Ngoerah, Denpasar, from January 2015 to May 2023. (isainsmedis.id)
Cholesterol saturation1
  • UDCA has been shown to decrease the biliary cholesterol saturation markedly and has found use as an alternative to cholecystectomy in patients with gallstone disease. (statpearls.com)
Ducts1
  • Infantile cholangiopathies that may lead to malabsorption of vitamin E include intrahepatic and extrahepatic biliary atresia, paucity of intrahepatic bile ducts, arteriohepatic dysplasia, and rubella-related embryopathy. (testcatalog.org)
Disorder2
  • Biliary atresia is a very rare disorder. (findmeacure.com)
  • Current data also supports UDCA treatment in intrahepatic cholestasis of pregnancy, a unique pregnancy-related disorder that can manifest during the late third trimester of pregnancy. (statpearls.com)
UDCA2