HEPATOPANCREATOBILIARY

Polyps and Other Tumors of the Gastrointestinal Tract

Jul 12, 2016 by in HEPATOPANCREATOBILIARY Comments Off on Polyps and Other Tumors of the Gastrointestinal Tract

Adenomatous polyposis syndromes Familial adenomatous polyposis (FAP) MYH-associated polyposis (MAP) Turcots syndrome Lynch syndrome Hamartomatous polyps Solitary juvenile polyp Juvenile polyposis syndrome PTEN hamartoma tumor syndrome Peutz–Jeghers syndrome Inflammatory polyps…

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Familial Intrahepatic Cholestasis

Jul 12, 2016 by in HEPATOPANCREATOBILIARY Comments Off on Familial Intrahepatic Cholestasis

Disease name Aliases Gene Protein Features FIC1 deficiency Byler disease, PFIC1 ATP8B1 FIC1 Multisystem disorder Wide range of severity BSEP deficiency Byler syndrome, PFIC2 ABCB11 BSEP Liver only affected Spectrum…

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Vascular Disorders of the Liver

Jul 12, 2016 by in HEPATOPANCREATOBILIARY Comments Off on Vascular Disorders of the Liver

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…

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Liver Tumors in Children

Jul 12, 2016 by in HEPATOPANCREATOBILIARY Comments Off on 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…

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Inherited Metabolic Disorders and the Liver

Jul 12, 2016 by in HEPATOPANCREATOBILIARY Comments Off on 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…

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Complications of Cirrhosis in Children

Jul 12, 2016 by in HEPATOPANCREATOBILIARY Comments Off on 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)…

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