Paucity of Intrahepatic Bile Ducts (Nonsyndromic)



Paucity of Intrahepatic Bile Ducts (Nonsyndromic)


Joseph Misdraji, MD









A portal tract in an infant with nonsyndromic paucity of intrahepatic bile ducts shows arteries image and veins image but no bile ducts. The hepatocytes are compact and clustered.






The centrilobular region in nonsyndromic bile duct paucity shows cholestasis and giant cell transformation of hepatocytes image, nonspecific features of many neonatal cholestatic disorders.


TERMINOLOGY


Synonyms



  • Hypoplasia of intrahepatic bile ducts


  • Intrahepatic biliary atresia



ETIOLOGY/PATHOGENESIS


Developmental Anomaly



  • Congenital disorders: Extrahepatic biliary atresia


  • Metabolic disorders: α-1-antitrypsin deficiency, cystic fibrosis, progressive familial intrahepatic cholestasis (PFIC), peroxisomal disorders, disorders of bile acid synthesis, Niemann-Pick type C, arthrogryposis-renal dysfunction-cholestasis syndrome (ARC)


  • Chromosomal abnormalities: Turner syndrome, monosomy X, trisomies


Infectious Agents



  • Intrauterine infections: Cytomegalovirus (CMV), rubella, syphilis


Idiopathic



  • Accounts for variable percentage of total cases, depending on population studied


  • Idiopathic group has been diminishing as metabolic and genetic mechanisms of ductopenia are elucidated


  • Diagnosis of exclusion


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Varies according to population evaluated but increased in regions of world where consanguineous marriage is common


  • Age



    • Generally considered disease of childhood since adults with ductopenia are classified in different disease categories


    • Some patients with idiopathic adulthood ductopenia may represent late presentation of disorder that began in childhood


Presentation



  • Jaundice and acholic stools


  • Pruritus


  • Hepatomegaly


Laboratory Tests



  • Increased alkaline phosphatase, γ-glutamyl transpeptidase, and serum bilirubin


  • Increased serum cholesterol may be seen

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Paucity of Intrahepatic Bile Ducts (Nonsyndromic)
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