hepatic failure-3

Hepatic Failure-3By

Dr. Abdelaty Shawky Assistant Professor of Pathology

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CIRRHOSIS

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* 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.

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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.

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d. Color: sometimes indicate a specific etiology; yellow (nutritional cirrhosis), green (biliary cirrhosis), red (congestion) and dark brown (hemochromatosis).

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Liver cirrhosis

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* M/P:

Loss of the normal hepatic architecture and

replacement by regenerating nodules, which are

surrounded by fibrous tissue septa.

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Cirrhosis

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* Etiological classification of cirrhosis:

B. Acquired cirrhosis A. congenital cirrhosis

1. Post-hepatitic (viral).2. Alcoholic.3. Biliary cirrhosis.4. Cryptogenic.

1. Congenital syphilis.2. Hemochromatosis.3. Glycogen storage disease.4. Wilson disease.5. α1 antitrypsin deficiency.

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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.

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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.

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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.

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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 porta hepatis

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* Effects of cirrhosis:

1. Portal hypertension.2. Liver cell failure.3. Malignant transformation to hepatocellular carcinoma specially macronodular type.

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PORTAL HYPERTENSION

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* 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.

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* Effects of portal hypertension:

1. Ascitis: Intra-peritoneal accumulation of

transudate.

2. Splenomegally:

• May lead to hypersplenism (Splenomegally +

pancytopenia).

3. Varices: esophageal varices, caput medusa, piles.

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Splenomegaly

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TUMORS OF THE LIVER

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I. BENIGN TUMORS: 5%.

A. Epithelial: • Liver cell adenoma.• Bile duct adenoma and cystadenoma.B. Mesenchymal: Cavernous hemangioma.

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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.

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HEPATOCELLULAR CARCINOMA (H.C.C)

(HEPATOMA)

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* Definition:• Malignant epithelial tumor arising from

hepatocytes.

* Incidence:• Common, the most common primary malignant

tumor of the liver.

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* Predisposing factors:• Chronic hepatitis especially HBV.• Cirrhosis especially macronodular.• Aflatoxins contaminate badly stored cereals.• Azo dyes.

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* 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.

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H.C.C

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* Spread:

1. Direct: within liver.

2. Lymphatic: to L.Ns at porta hepatis.

3. Blood: intrahepatic through portal vein, and

extrahepatic through hepatic veins.

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* Clinical features:• Hepatomegaly.• Hemorrhagic ascitis.• Liver cell failure.• Elevated level of alpha feto protein (AFP).

* Prognosis:• Poor, most patients die within one year from

liver cell failure or metastasis.

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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…

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Metastatic carcinoma to liver

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Clinical manifestations of liver cell failure

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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.

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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.

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9. Hepatic encephalopathy:

• Neurological disturbances (apathy,

disorientation, asterixis i.e. flapping tremors and

coma) due to toxic amonia which are formed in

intestine and not detoxified in liver so pass

directly to brain.

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10. Foetor hepaticus: 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.

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Hepatorenal syndrome

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• 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.

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Thanks