Fig. 56.1 Schematic illustration of biliary atresia (based on Japanese Association of Pediatric Surgeons classification) A more radical approach to the technique was pioneered in Sendai, Japan, during the 1950s…

Fig. 67.1 Ultrasonography (a) and colour Doppler (b) showing cavernomatous transformation of the portal vein in a 14-year-old boy with portal vein thrombosis. Liver MRI illustrating coronal (c), (d) and…

Fig. 68.1 Anatomy of the portal system In this system, the central vessel is the portal vein, which is formed by the union of the splenic vein (SV) and the…

Benign Hemangioma: focal, multiple, diffuse Mesenchymal hamartoma Hepatic adenoma Focal nodular hyperplasia Inflammatory myofibroblastic tumor (may be locally invasive) Malignant Primary Hepatoblastoma Hepatocellular carcinoma, fibrolamellar HCC Transitional tumors Embryonal sarcoma…

Cholestasis α1-antitrypsin deficiency Arginase deficiency (UCD) Bile acid metabolism defects, e.g. CTX Byler disease CDGs Cholesterol synthesis defects Citrin deficiency Galactosaemia LCHAD Mevalonic aciduria Niemann–Pick C (LSD) Peroxisomal defects Tyrosinemia…

Fig. 3.1 Common anatomical variants of EA/TEF anomalies. a EA with distal TEF, b isolated EA with no TEF, c H-type TEF, d proximal and distal TEF, e EA with…

Etiology of liver cirrhosis Metabolic liver disease α1-Antitrypsin deficiency Wilson disease Progressive familial intrahepatic cholestasis Glycogen storage diseases (especially types IV, VI, IX, and X) Lipid abnormalities (e.g., Gaucher disease)…

Small-intestinal enteropathy of unknown origin Intractable ulcerating enterocolitis of infancy Congenital enterocyte heparan sulfate deficiency Congenital intestinal integrin deficiency Congenital secretory diarrheas Congenital chloridorrhea Congenital Na-losing diarrhea Autoimmune enteropathy Diseases…

First author Location Age range Time period studied Incidence Henderson 2012 [6] Scotland < 16 years 2003–2008 2.1 Benchimol 2009 [7] Ontario, Canada < 18 years 1994–2005  1994 4.1 2005…

Fig. 31.1 Distribution of vessel involvement by large-vessel vasculitis, medium-vessel vasculitis and small-vessel vasculitis. Note that there is substantial overlap with respect to arterial involvement, and an important concept is…