1. hereditary hemochromatosis
   1. periportal iron deposition
      1. heavy in periportal tracts, lighter toward central venule
   2. micronodular cirrhosis
   3. secondary hemochromatosis: ALSO see iron in Kupffer cells and macrophages
   4. on prussian blue, iron is blue
2. alpha-1 anti trypsin
   1. round to oval cytoplasmic inclusions in periportal hepatocytes
   2. immunostain shows presence of alpha-1 antitrypsin
3. PBC stages
   1. stage 1: duct membrane lesion, granulomatous reaction
   2. jigsaw liver indicates biliary cirrhosis
4. PSC pathology
   1. onion skin fibrosis
   2. degeneration of bile duct epithelium with PRESERVATION of basement membrane
   3. abnormally shaped liver on gross exam
5. fibrous occlusion of small hepatic venules
6. path
   1. centrilobular congestion
   2. CAUDATE LOBE HYPERTROPHY
      1. doesn't atrophy because has a different outflow tract
7. pathology
   1. gross
   2. adenocarcinoma
8. Syllabus
9. metabolic liver diseases
   1. hereditary hemochromatosis
      1. excessive iron deposition
      2. homozygous recessive
      3. AGE 40, males present before females
      4. MICRONODULAR CIRRHOSIS
      5. DIABETES MELLITUS (iron deposition in pancreas)
      6. SKIN PIGMENTATION (bronze skin)
      7. 200x increased risk of HCC
      8. pathology
   2. cystic fibrosis
   3. wilson's disease
      1. excessive copper accumulation
      2. AR
      3. defective biliary copper excretion
      4. variable onset
      5. Keyser-Fleischer ring in iris
      6. pathology
         1. copper in periportal hepatocytes
         2. hepatic necrosis
         3. atrophy of brain tissue and cavitation of basal ganglia
   4. alpha-1 anti-trypsin
      1. most common pediatric liver disease
      2. AR
      3. pathology
10. alcoholic liver disease
    1. hepatic steatosis
       1. macro- or microvesicular steatosis, but reversible
       2. accumulation of collagen over time that encapsulates hepatocytes
    2. alcoholic hepatitis
       1. ballooning degeneration
       2. mallory bodies: cytokeratin filaments (not diagnostic)
       3. neutrophilic reaction
       4. fibrosis
       5. micronodular cirrhosis
11. benign tumors and tumor-like lesions
    1. hepatic adenoma
       1. solitary
       2. well-encapsulated
       3. portal tracts or central venules absent, but see arteries and veins traversing tumor
       4. associated with ORAL CONTRACEPTIVES
    2. focal nodular hyperplasia
    3. metastatic tumor
    4. HCC
       1. caused by Hep B, C, alcohol, contraceptives
       2. anabolic steroids can cause
       3. NASH predisposes
       4. variable histology
    5. cholangiocarcinoma
       1. arises from biliary epithelim
       2. type of adenocarcinoma
       3. hilar tumor (klatskin)
    6. angiosarcoma
12. cystic diseases of liver
13. biliary tract disease
    1. primary biliary cirrhosis
       1. immune-mediated attack on pyruvate dehydrogenase of bile duct cells
       2. chronic progressive
       3. destruction of bile duct basement membrane
       4. lymphocytic infiltration
       5. middle-aged women
       6. AMA+
       7. increased GGT and bilirubin
       8. pruritis, xanthelasma, osteoporosis
       9. LARGE bile ducts
    2. primary sclerosing cholangitis
       1. "onion skin fibrosis"
       2. mostly MALE
       3. SMALL bile ducts
       4. pathology
    3. secondary sclerosing cholangitis
       1. mechanical problem leading to cholangitis and biliary cirrhosis
14. vascular disorders
    1. budd-chiari syndrome (LIVER OUTFLOW OBSTUCTION)
       1. hypercoagulation
       2. vascular injury (trauma, vasculitis
       3. stasis or mass lesions
       4. IBD, MULTIPLE MYELOMA
    2. veno-occlusive disease
       1. alkaloid ingestion
       2. after bone marrow transplant
       3. hepatic radiation
       4. from CHF, sickle cell disease
    3. CHF (NUTMEG apperance)
    4. shock (ischemic necrosis of CENTRILOBULAR hepatocytes)