(精选)pathology-of-liver-biliary-tract-and-pancreas教学课件.pptVIP

Hemochromatosis = increased uptake of iron in intestine ? depositions of hemosiderin liver + pancreas + skin + heart gene HFE, relationship to receptor for tranferin chocolate brown cirrhosis pancreas fibrosis ? bronze diabetes mellitus skin pigmentations (bronze) cardiomyopathy Wilson disease  = hepatolenticular degeneration disorder of copper metabolism defect at level of ceruloplasmin ? accumulation: liver + brain + eye cirrhosis basal ganglia (neurologic symptoms) Kayser-Fleischer green brown ring Liver cirrhosis - complications 1. Portal hypertension ( 10 mmHg) - portosystemic shunts - rectum – hemorrhoids - cardioesophageal junction – e. varices - abdominal wall – caput medusae - ascites - splenomegaly - GIT congestion 2. Hemorrhagic diathesis 3. Progressive liver failure 4. Hepatocellular carcinoma Portal hypertension 1. prehepatic - portal vein thrombosis / stenosis 2. intrahepatic - cirrhosis - massive steatosis - nodular hyperplasia - granulomatous diseases (TBC, sarcoidosis) 3.posthepatic - severe right-sided cardiac failure - constrictive pericarditis - Budd-Chiari syndrome Neoplasms I. Primary benign tumors 1. cavernous hemangioma - mesenchymal, most common - red-blue soft nodule, subcapsular location !!! percutaneous needle biopsy – bleeding !!! Neoplasms 2. hepatocellular adenoma - women - oral contraceptives - men - anabolics - well circumscribed pale nodule, several cm - Mi: cords of hepatocyte-like cells, NO portal tracts - rupture (pregnancy – estrogens) - bleeding 3. cholangioadenoma, cholangiohamartoma (von Meyenburg complex) - small multiple subcapsular nodules - Mi: tubular structures (fibrous stroma) Neoplasms II. primary malignant tumors: 1. hepatocellular carcinoma - Africa, Asia (China), Japan - M : F … 3-8 : 1, 60-70 years - chronic hepatitis (HBV, HCV) + cirrhosis + dietary factors - clinically: alpha-fetoprotein - unifocal x multifocal x diffusely infiltrative, yellow-white - invasion of vascular channels