Paucity of Intrahepatic Bile Ducts (Nonsyndromic)


Portal Tract
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.



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

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Portal Tract
Another 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.

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Higher Magnification
Higher magnification of the centrilobular region in nonsyndromic bile duct paucity shows cholestasis and giant cell transformation of hepatocytes, nonspecific features of many neonatal cholestatic disorders.



TERMINOLOGY


Synonyms




• Hypoplasia of intrahepatic bile ducts

• Intrahepatic biliary atresia


Definitions




• Heterogeneous group of disorders that cause bile duct paucity or ductopenia in patients without congenital abnormalities indicative of Alagille syndrome
image Ductopenia defined as significant decrease in number of interlobular bile ducts

– Ratio of number of interlobular bile ducts to number of portal tracts is < 0.4, with normal between 0.9 and 1.8

– Alternatively, ductopenia is diagnosed when > 50% of portal tracts lack bile ducts; normally, 80-100% of portal tracts contain ducts


ETIOLOGY/PATHOGENESIS


Developmental Anomaly




• Congenital disorders: Extrahepatic biliary atresia

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

• Chromosomal abnormalities: Turner syndrome, monosomy X, trisomies


Infectious Agents




• Intrauterine infections: CMV, rubella, syphilis


Idiopathic




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

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

• Diagnosis of exclusion


CLINICAL ISSUES


Epidemiology




• Incidence
image Varies in population studied but increased in regions where consanguineous marriage is common

• Age
image Generally considered disease of childhood as adults with ductopenia are classified in different categories

image Some patients with idiopathic adulthood ductopenia may represent late presentation of disorder


Presentation




• Jaundice and acholic stools

• Pruritus

• Hepatomegaly


Laboratory Tests




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

• Increased serum cholesterol may be seen

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Paucity of Intrahepatic Bile Ducts (Nonsyndromic)
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