Slide 1

. . Prof Lerine Bahy Eldin. Head of pediatric hepatology unit.

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. . A condition in which little or no bile is secreted or the flow of bile into the.

. . . Conjugated hyperbilirubinemia. . Conjugated bilirubin >.

. . . . •. . Bilirubin. . metabolism. . . Biliverdin Ind Bilirubin.

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. . . Levels of bile stasis. . Hepatocyte. . :. .

. . . Progressive familial intrahepatic. . cholestasis.

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. . . Levels of bile stasis. . Hepatocyte. . :. .

. . Dubin – Johnson syndrome. . .

. . . Bile canaliculus. . :. . hepatocyte swelling eg.

. . . Alagille Syndrome. . .

. . . Peculiar facies. . .

. . . Butterfly vertebrae. . .

. . Posterior embryotoxon. . .

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. . . Bile canaliculus. . :. . hepatocyte swelling eg.

. . . Extrahepatic bil system. . :. . eg EHBA,. .

. . EHBA. Definition. Biliary atresia is a condition in which the normal.

. . Types. • The fetal-embryonic form appears in the first 2 weeks.

. . Classification: Three main types of biliary atresia are surgicaly defined: 1 -In type I, the common bile duct is obliterated while the proximal bile.

. . porta hepatis lla porta hepatis gallbladder cystic duct common hepatic duct common bile duct gallbladder cystic duct common hepatic duct common bile duct bowel 11b porta hepatis bowel porta hepatis gallbladder cystic duct bowel common hepatic duct common bile duct gallbladder cystic duct bowel intra-hepatic ducts common hepatic duct common bile duct.

. . lla 11b. .

. . Clinically. Regardless of etiology, the clinical presentation of neonatal cholestasis is remarkably similar in most infants Typical symptoms include variable degrees of jaundice, dark urine, and light stools.

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. . BA is deceiving ?. In the case of biliary atresia, most infants are full-term, In most cases, acholic stools are not noted at birth but develop over the first few weeks of life and is persistent. Appetite, growth, and weight gain are always normal.

. . infants with biliary atresia typically show only.

. . Transaminases level are not markedly elevated ALP and GGT are markedly elevated Serum albumin and INR are usually normal until late stages.

. . US. . .

. . The triangular cord sign (TCS) is a specific ultrasonographic finding,.

. . GB. . . .

. . Biliary atresia can be accurately. diagnosed by ultrasonography using the findings of TCS combined with.

. . Do not search for IHBD. .

. . The presence of. triangular cord in the right and left hepatic.

. . HIDA scan. hepatobiliary iminodiacetic acid. scan.

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. . . Liver biopsy. . .

. . Liver biopsy. . .

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. . This surgery is called a Kasai procedure after the Japanese surgeon who developed the surgery, Dr. Morio Kasai or called hepatoportoenterostomy This procedure is not usually curative, but ideally does buy time until the child can achieve growth and undergo liver transplantation.

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. . After kasai. Antibiotics Steroids Choleretics Fat soluble vitamins.

. . . other causes of cholestasis. . Metabolic. .

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. . . other causes of cholestasis. . Metabolic. .