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Hepatic Failure-3. By Dr. A bdelaty S hawky Assistant Professor of Pathology. Cirrhosis. * Def : chronic diffuse irreversible liver disease characterized by loss of the normal hepatic architecture and replacement by regenerating nodules. * Gross features of cirrhotic liver:
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Hepatic Failure-3 By Dr. Abdelaty Shawky Assistant Professor of Pathology
* Def: chronic diffuse irreversible liver disease characterized by loss of the normal hepatic architecture and replacement by regenerating nodules. * Gross features of cirrhotic liver: a. Size: usually shrunken (except in biliary cirrhosis). b. Consistency: firm.
c. Outer surface & cut surface: nodular. According to the size of the nodules, cirrhosis is classified into: • Micronodular cirrhosis: the nodules are less than 3 mm. in diameter. • Macronodular cirrhosis: the nodules are more than 3 mm. in diameter (or poor prognosis). • Mixed micro & macronodular cirrhosis.
d. Color: sometimes indicate a specific etiology; yellow (nutritional cirrhosis), green (biliary cirrhosis), red (congestion) and dark brown (hemochromatosis).
* M/P: Loss of the normal hepatic architecture and replacement by regenerating nodules, which are surrounded by fibrous tissue septa.
1. post-hepatitic (viral) cirrhosis. • Follows chronic hepatitis. * N/E:shrunken liver with mixed micro & macronodular cirrhosis. * Prognosis: • Rapid and progressive course. • Great association with hepatocellular carcinoma.
2. Alcoholic (nutritional) cirrhosis. * N/E: the liver is shrunken, firm, and yellowish. It is micronodular, macronodular or mixed. * Prognosis: it regresses slowly over few years.
3. Biliary cirrhosis. • Two distinct types; A. Primary biliary cirrhosis (PBC). • Autoimmune disease affecting middle aged females characterized by increased AMA (auto-antibodies against mitochondrial enzymes). * N/E:the liver is enlarged, dark green in color (cholestasis) and of mixed type.
B. 2ry biliary cirrhosis. • Due to extra-hepatic biliary obstruction: * Causes: • Congenital biliary atresia. • Post–inflammatory stricture of the common bile or hepatic ducts. • Carcinoma of common bile duct, ampulla of vater or pancreatic head. • Biliary stones. • Pressure from outside by enlarged lymph nodes or tumors at the portahepatis
* Effects of cirrhosis: 1. Portal hypertension. 2. Liver cell failure. 3. Malignant transformation to hepatocellular carcinoma specially macronodular type.
* Def.elevation of the portal venous pressure (Normally = 7 m.mHg). * Causes: I. Pre-sinusoidal causes: 1. Massive Splenomegally. 2. Portal vein obstruction (thrombosis, pressure from outside). 3. Portal venular obstruction: (bilharziasis). II. Sinusoidal: cirrhosis. III. Post-sinusoidal: - Right sided heart failure and constrictive pericarditis.
* Effects of portal hypertension: • Ascitis: Intra-peritoneal accumulation of transudate. 2. Splenomegally: • May lead to hypersplenism (Splenomegally + pancytopenia). 3. Varices: esophageal varices, caput medusa, piles.
I. Benign tumors: 5%. A. Epithelial: • Liver cell adenoma. • Bile duct adenoma and cystadenoma. B. Mesenchymal:Cavernous hemangioma.
II. Malignant tumors: 95%. A. Primary: A. Epithelial: • Hepatocellular carcinoma (H.C.C) • Cholangiocarcinoma. B. Mesenchymal: • Angiosarcoma. • Lymphoma. C. Mixed: Hepatoblastoma. B. Metastatic tumors:Metastatic carcinomas, sarcomas & lymphomas.
* Definition: • Malignant epithelial tumor arising from hepatocytes. * Incidence: • Common, the most common primary malignant tumor of the liver.
* Predisposing factors: • Chronic hepatitis especially HBV. • Cirrhosis especially macronodular. • Aflatoxins contaminate badly stored cereals. • Azo dyes.
* Gross features: A mass;well defined borders, hard in consistency, yellow white and may be green (bile-stained) in color with areas of hemorrhage and necrosis.
* Spread: • Direct: within liver. • Lymphatic: to L.Ns at portahepatis. • Blood: intrahepatic through portal vein, and extrahepatic through hepatic veins.
* Clinical features: • Hepatomegaly. • Hemorrhagic ascitis. • Liver cell failure. • Elevated level of alpha fetoprotein (AFP). * Prognosis: • Poor, most patients die within one year from liver cell failure or metastasis.
Metastatic tumors of the liver * Incidence: The most common malignant tumor of the liver (more common than the 1ry malignant tumors). * Routes of spread to the liver: 1. via portal vein: from cancer stomach, intestine. 2. via hepatic artery: from cancer lung, and other systemic cancers that bypass the lung. 3. via lymphatics: from cancer lung and breast. 4. Directly: from cancer gall bladder, stomach, colon, kidney…
1. Jaundice:Hepatocelluar jaundice. 2. Hyporpteinaemia: due to decreased formation of plasma proteins. Contributes in generalized edema formation and ascitis. 3. Vitamin deficiency: Vit. A, K, B12, folic acid. 4. Coagulation defect: due deficiency of fibrinogen, factors V, VII, IX, X. 5. Anemia: due to repeated hemorrhage, hypersplenism, B12 and folic acid deficiency. 6. Hypoglycemia: due to defects in carbohydrate metabolism.
7. Hormone disturbances: • Increased serum aldosterone leads: to salt & water retention. • Increased estrogen leads to: • Gynaecomastia. • Hypogonadism (testicular atrophy and loss of libido in males). • Menstrual disturbances in females. • Palmer erythema (local vasodilation). • Spider angiomas of skin: Each angioma is a central, pulsating, dilated arteriole from which small vessels radiate 8. Ascitis: Due to salt & water retention, portal hypertension and hyporpteinaemia.
9. Hepatic encephalopathy: • Neurological disturbances (apathy, disorientation, asterixis i.e. flapping tremors and coma) due to toxic amoniawhich are formed in intestine and not detoxified in liver so pass directly to brain.
10. Foetorhepaticus:a characteristic bad (musty) odor smelled from the body and mouth of the patients due to formation of mercaptans by the action of gastrointestinal bacteria on the sulfur-containing amino acid methionine.
Hepatorenal syndrome refers to the appearance of renal failure in patients with severe chronic liver disease, in whom there are no intrinsic morphologic or functional causes for the renal failure. • Sodium retention, impaired free-water excretion, and decreased renal perfusion and glomerular filtration rate are the main renal functional abnormalities.