Portal Hypertension in Children
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…
Liver Tumors in Children
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…
Inherited Metabolic Disorders and the Liver
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…
Congenital Problems of the Gastrointestinal Tract
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…
Complications of Cirrhosis in Children
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)…
Ulcerative Colitis
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…
Microvillus Inclusion Disease and Tufting Enteropathy
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…
Vasculitides Including Henoch–Schönlein Purpura
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…
Nonalcoholic Fatty Liver Disease
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…
Autoimmune Liver Disease
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…
