Table 1.
Clinical profile and laboratory data.
Table 2.
Major clinical features and gene analyses in 11 patients with Alagille syndrome candidates for liver transplantation.
Fig 1.
Contrast-enhanced axial CT scans for hepatic parenchymal changes.
(A) Normal hepatic morphology in a 7-month-old girl with Alagille syndrome. (B, C) Macroscopic (B) and microscopic (C) specimens of the liver show cholestasis with paucity of interlobular bile duct, but parenchymal destruction is relatively mild (original magnification of microscopic image: x40). (D) Hepatic parenchymal changes with heterogeneous enhancement, fissure widening, left lobe hypertrophy, and periportal edema in a 5-month-old girl with biliary atresia. Note the presence of ascites and gastroesophageal varix. (E, F) Macroscopic (E) and microscopic (F) specimens of the liver show cholestasis and cirrhotic changes with marked parenchymal destruction (original magnification of microscopic image: x40).
Table 3.
CT imaging findings in patients with Alagille syndrome and end-stage biliary atresia.
Fig 2.
Contrast-enhanced axial CT scans for vascular changes.
(A) Normal hepatic vasculature in a 5-month-old boy with Alagille syndrome. Diameters of proximal right hepatic artery (arrowhead) and portal vein (thin arrow) are 1.3 mm and 5.0 mm, respectively. The ratio of the hepatic artery diameter to the portal vein diameter is 0.26. (B) Enlarged hepatic artery and small portal vein diameters in a 6-month-old girl with biliary atresia. Diameters of proximal right hepatic artery (arrowhead) and portal vein (thin arrow) are 2.4 mm and 2.6 mm, respectively. The ratio of the hepatic artery diameter to the portal vein diameter is 0.92.