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)…
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…
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…
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…
Fig. 66.1 Factors associated with the pathogenesis of NAFLD. PNLA pectin lyase, APOC apolipoprotein C, TNF tumor necrosis factor, KLF Kruppel-like factor, PUFA polyunsaturated fatty acids, NAFLD nonalcoholic fatty liver…
Fig. 63.1 Histological features of autoimmune hepatitis. a Portal and periportal lymphocyte and plasma cell infiltrate, extending to and disrupting the parenchymal limiting plate (interface hepatitis). Swollen hepatocytes, pyknotic necroses…
Fig. 57.1 Biliary development and the ductal plate malformation (modified from [1]). a At 14 weeks of gestation, hepatoblasts in contact with portal vein mesenchyme adopt a biliary epithelial fate….
Fig. 48.1 Air enema showing intussusception up to the rectum Spontaneous reduction is another possible outcome and reportedly occurs in almost 20 % of intussusceptions [4]. Etiology The pathogenesis of intussusception…
