Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ Key Features +++ Essentials of Diagnosis ++ Elevated total and conjugated bilirubin Hepatomegaly and dark urine Patency of extrahepatic biliary tree +++ General Considerations ++ Characterized by impaired hepatocyte secretion of bile and patency of the extrahepatic biliary system A specific cause can be identified in about 60% of cases Infectious agents include Herpes simplex virus Varicella virus Enteroviruses (coxsackievirus and echovirus) Cytomegalovirus (CMV) Rubella virus Adenovirus Parvovirus Human herpesvirus type 6 (HHV-6) Hepatitis B virus (HBV) HIV Treponema pallidum Toxoplasma gondii +++ Clinical Findings +++ Symptoms and Signs ++ Clinical symptoms typically present in the first 2 weeks of life but may appear as late as age 2–3 months Infant may have jaundice, petechiae, or rash and generally appears ill Poor oral intake, poor sucking reflex, lethargy, hypotonia, and vomiting are frequent Stools may be normal to pale in color but are seldom acholic Dark urine stains the diaper Firm hepatomegaly is present Splenomegaly is variably present Unusual presentations include neonatal liver failure, hypoproteinemia, anasarca (nonhemolytic hydrops), and hemorrhagic disease of the newborn. +++ Differential Diagnosis ++ Galactosemia Hereditary fructose intolerance Tyrosinemia Concomitant bacteremia α1-Antitrypsin deficiency Cystic fibrosis Bile acid synthesis defects Progressive familial intrahepatic cholestasis Mitochondrial respiratory chain disorders Neonatal iron storage disease Alagille syndrome, arthrogryposis/renal dysfunction/cholestasis (ARC) syndrome or Zellweger syndrome Idiopathic neonatal hepatitis +++ Diagnosis +++ Laboratory Findings ++ Neutropenia, thrombocytopenia, and mild hemolysis are common Mixed hyperbilirubinemia, elevated aminotransferases with near-normal alkaline phosphatase, prolongation of clotting studies, mild acidosis, and elevated cord serum IgM suggest congenital infection Nasopharyngeal washings, urine, stool, serum, and cerebrospinal fluid (CSF) should be cultured for virus and tested for pathogen-specific nucleic acid Specific IgM antibody may be useful Serum bilirubin, alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase, and bile acids are typically elevated +++ Imaging ++ Long-bone radiographs can show "celery stalking" in the metaphyseal regions of the humeri, femurs, and tibias When indicated, computed tomography (CT) scans can identify intracranial calcifications (especially with CMV and toxoplasmosis). Hepatobiliary scintigraphy shows decreased hepatic clearance of the circulating isotope with intact excretion into the gut +++ Diagnostic Studies ++ Careful ophthalmologic examination may be useful for diagnosis of herpes simplex virus, CMV, toxoplasmosis, and rubella. Percutaneous liver biopsy shows Typical inclusions of CMV in hepatocytes or bile duct epithelial cells Presence of intranuclear acidophilic inclusions of herpes simplex or varicella-zoster virus Presence of adenovirus basophilic intranuclear inclusions Positive immunohistochemical stains for several viruses Viral cultures, immunohistochemical stains, or polymerase chain reaction (PCR) testing of biopsy material may be helpful +++ Treatment ++ Acyclovir for herpes simplex virus, varicella; ganciclovir for CMV; supportive/intravenous immunoglobulin for parvovirus; pyrimethamine and sulfadiazine ... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Download the Access App: iOS | Android Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.