PMC9386286_01
(Top) Liver biopsy shows: (a) preserved architecture with mild portal inflammation (HE, 4x). (b) fibroedematous portal tracts (Masson trichrome, 4x). (c) CK7 immunostaining displays interlobular biliary ducts and diffuse abnormal staining of periportal hepatocytes (CK7, 4x). (d) MDR3 is normally expressed at the canalicular pole of the hepatocytes (MDR3, 20x). (Bottom) (e) Increased inflammation (HE, 4x) and (f) fibrosis with fibrous septae (Masson trichrome, 4x). (g) The extrahepatic biliary duct shows a thick fibrotic wall. Small siero-mucous glands are present within the fibrous wall. No neoplastic cells are evident (HE, 10x). (h) CK7 shows residual epithelial of the main lumen (CK7, 10x). HE, hematoxylin and eosin; CK7, cytokeratin 7; MDR3, multidrug resistance protein 3.
fped-10-969081-g0001.jpg
PMC9386286_02_fped-10-969081-g0001.jpg
F1
At the definitive histologic exam, the liver was characterized by cholangitis and biliary fibrosis (Figures 1e,f). The removed extrahepatic biliary tree displayed a fibrotic thickened wall with only a slim lumen (Figures 1g,h); the gallbladder, normally shaped, showed chronic cholecystitis.
(a) Magnetic resonance cholangiopancreatography. (b) Percutaneous transhepatic cholangiography. Dilation of intrahepatic ducts and of the two hepatic ducts with regular profiles, abruptly stopping at the confluence level, without appreciation of the choledochus and the gallbladder.
fped-10-969081-g0002.jpg
PMC9386286_02_fped-10-969081-g0002.jpg
F2
Percutaneous transhepatic cholangiography (PTC) confirmed stenosis of the confluence of the right and left hepatic ducts and failure to visualize the extrahepatic biliary tree below the stenosis (Figure 2b).
PMC9386286_02
(Top) Liver biopsy shows: (a) preserved architecture with mild portal inflammation (HE, 4x). (b) fibroedematous portal tracts (Masson trichrome, 4x). (c) CK7 immunostaining displays interlobular biliary ducts and diffuse abnormal staining of periportal hepatocytes (CK7, 4x). (d) MDR3 is normally expressed at the canalicular pole of the hepatocytes (MDR3, 20x). (Bottom) (e) Increased inflammation (HE, 4x) and (f) fibrosis with fibrous septae (Masson trichrome, 4x). (g) The extrahepatic biliary duct shows a thick fibrotic wall. Small siero-mucous glands are present within the fibrous wall. No neoplastic cells are evident (HE, 10x). (h) CK7 shows residual epithelial of the main lumen (CK7, 10x). HE, hematoxylin and eosin; CK7, cytokeratin 7; MDR3, multidrug resistance protein 3.
fped-10-969081-g0001.jpg
PMC9386286_02_fped-10-969081-g0001.jpg
F1
At the definitive histologic exam, the liver was characterized by cholangitis and biliary fibrosis (Figures 1e,f). The removed extrahepatic biliary tree displayed a fibrotic thickened wall with only a slim lumen (Figures 1g,h); the gallbladder, normally shaped, showed chronic cholecystitis.
(a) Magnetic resonance cholangiopancreatography. (b) Percutaneous transhepatic cholangiography. Dilation of intrahepatic ducts and of the two hepatic ducts with regular profiles, abruptly stopping at the confluence level, without appreciation of the choledochus and the gallbladder.
fped-10-969081-g0002.jpg
PMC9386286_02_fped-10-969081-g0002.jpg
F2
Percutaneous transhepatic cholangiography (PTC) confirmed stenosis of the confluence of the right and left hepatic ducts and failure to visualize the extrahepatic biliary tree below the stenosis (Figure 2b).
Separation of the 1.020-1.063 g/ml plasma fraction by fast protein liquid chromatography (FPLC). The 1.020-1.063 g/ml density fraction, corresponding to lipoprotein X and LDL, was separated by FPLC and analyzed for phospholipid content. Insert shows cathodic migration of Lp X stained with filipin at agarose gel electrophoresis. Black line indicates origin.
fped-10-969081-g0003.jpg
PMC9386286_02_fped-10-969081-g0003.jpg
F3
Protein Liquid Chromatography, as previously described (Figure 3). Presence of LpX was additionally confirmed by electrophoresis using Sebia agarose gels, followed by