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Gastrointestinal  ` gastrointestinal—PatHology  Gastrointestinal  ` gastrointestinal—PatHology        seCtion iii      393




                  Jaundice               Abnormal yellowing of the skin           HOT Liver—common causes of  bilirubin
                                          and/or sclera  A  due to bilirubin deposition.   level:
                   A
                                          Hyperbilirubinemia 2° to  production     Hemolysis
                                          or  clearance (impaired hepatic uptake,   Obstruction
                                          conjugation, excretion).                  Tumor
                                                                                    Liver disease





                   Conjugated (direct)   Biliary tract obstruction: gallstones, cholangiocarcinoma, pancreatic or liver cancer, liver fluke.
                    hyperbilirubinemia   Biliary tract disease:
                                             ƒ 1° sclerosing cholangitis
                                             ƒ 1° biliary cholangitis
                                         Excretion defect: Dubin-Johnson syndrome, Rotor syndrome.
                   Unconjugated          Hemolytic, physiologic (newborns), Crigler-Najjar, Gilbert syndrome.
                    (indirect)
                    hyperbilirubinemia
                   Mixed (direct         Hepatitis, cirrhosis.
                    and indirect)
                    hyperbilirubinemia



                  Physiologic            At birth, immature UDP-glucuronosyltransferase Ž unconjugated hyperbilirubinemia Ž jaundice/
                  neonatal jaundice       kernicterus (deposition of unconjugated, lipid-soluble bilirubin in the brain, particularly basal
                                          ganglia).
                                         Occurs after first 24 hours of life and usually resolves without treatment in 1–2 weeks.
                                         Treatment: phototherapy (non-UV) isomerizes unconjugated bilirubin to water-soluble form.



                  Biliary atresia        Most common reason for pediatric liver transplantation.
                                         Fibro-obliterative destruction of extrahepatic bile ducts Ž cholestasis.
                                         Often presents as a newborn with persistent jaundice after 2 weeks of life, darkening urine, acholic
                                          stools, hepatomegaly.
                                         Labs:  direct bilirubin and GGT.



































          FAS1_2019_09-Gastrointestinal.indd   393                                                                      11/7/19   4:42 PM
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