Results also show that AQP1 expression in bile duct is higher than in interlobular bile ducts
, which may be related to the excretion of bile.
Liver biopsy was significant for severe hepatocellular cholestasis with ballooning and slight deficiency of interlobular bile ducts and cholangioles suggestive of ALGS.
(i) Diagnosis of Alagille Syndrome requires biopsy proven paucity of interlobular bile ducts (ii) Plus 3 of the following 5 characteristic features: (a) Chronic cholestasis (b) Congenital cardiac disease (c) Skeletal defects (d) Ocular anomalies (e) Characteristic facies Our patient had paucity of intrahepatic bile ducts on liver biopsy along with chronic cholestasis, congenital heart disease, and characteristic dysmorphic facies, meeting the classic criteria for ALGS.
Dommergues, "Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases," The Journal of Pediatrics, vol.
The histopathology revealed absence of interlobular bile ducts
with mild inflammatory infiltrate.
Injured bile ducts surrounded by a dense infiltrate of mononuclear cells, most of which are lymphocytes, these florid, asymmetric destructive lesions of interlobular bile ducts
are irregularly scattered throughout the portal triads and often seen only on large surgical biopsies of the liver in which adequate representation of small bile ducts occurs; inflammation is confined to the portal trials (Hirschfield, 2011).
Background: Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease characterized by destruction of the interlobular bile ducts
and a striking female predominance.
Diseases of the proximal pathways of the biliary system include (1) those that affect the interlobular bile ducts and (2) those that affect the bile canaliculi.
The smallest bile ducts, the interlobular bile ducts, which measure less than 100 im in diameter, are not visualized radiographically.
* Histologic evidence of nonsuppurative destructive cholangitis and destruction of interlobular bile ducts
They also documented progressive arterial thickening when the Kasai specimen was compared to the explanted liver in several cases, which was associated with disappearance of interlobular bile ducts. Arterial thickening has also been confirmed by comprehensive studies using imaging techniques.
Interlobular bile duct injury or inflammation has been reported in 31% to 94% of cases (77,87,88) and bile duct loss, in approximately 8% of cases, on initial biopsy samples.