GI – Liver: Cirrhosis, Cancer, PBC, PSC – PPT/Book – Flashcards
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Define liver cirrhosis.
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1. Slowly progressive. 2. Formation of fibrous and scar tissue that replaces hepatocytes. 3. Portal blood flow impaired.
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Most of the clinical features of liver cirrhosis develop as a result of what 3 things?
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1. Portal hypertension. 2. Hepatocellular dysfunction. 3. Altered cellular differentiation.
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3 most common causes of cirrhosis in industrialized nations.
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1. Nonalcoholic fatty liver disease. 2. Alcoholic liver disease. 3. Hepatitis C.
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True or False: Many people with cirrhosis at presentation have no readily identifiable cause.
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True.
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Pathophysiology of cirrhosis.
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1. Hepatocytes die and release inflammatory markers. 2. Inflammatory markers cause stellate cells to differentiate to fibroblasts. 3. The fibroblasts lay down collagen/connective tissue in interspace of diss, resulting in fibrosis. 4. Sinusioid is compressed and gets smaller. 5. Nodules of fibrous tissue with foci of regenerating hepatocytes forms with rearrangement of blood vessels. 6. Disruption of normal hepatic lobular architecture and vascular bed leads to portal hypertension and shunting.
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Fibrosis in the liver usually begins where?
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Around the portal area.
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True or False: The symptoms of liver cirrhosis are often nonspecific in the early stages and progress to a wide array of signs and symptoms in various systems.
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True.
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The diagnosis of liver cirrhosis can reliably be made by a combination of clinical, laboratory, and radiological findings in most cases. However, what is the gold standard?
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Liver biopsy. *However more often now doen to assess stage and severity of disease.
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MRI findings supportive of cirrhosis.
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1. Relative enlargement of left hepatic and caudate lobes as a result of right lobe atrophy. 2. Surface nodularity. 3. Portal hypertension features such as ascites, intra-abdominal varices, and splenomegaly.
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One of the many deficiencies in liver cirrhosisi will be deficient vitamin K-dependent coagulation factors. Which factors are these?
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II, VII, IX, X.
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Bulging flanks and shifting dullness suggest what?
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Ascites.
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Cirrhotic ascites is ultimately a result in what?
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An increase in total blood sodium and water.
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What imaging is used to screen for ascites? Confirm it?
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*Ultrasound to screen. *Parecentesis to confirm.
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What serum-asciteis albumin gradient [SAGG] level correlates with protal hypertension as the likely cause of fluid accumulation?
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Elevated SAGG >1.1 g/dL [calculated by serum albumin concentration - ascitic fluid albumin concentration].
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Ascites becomes clinically detectable with fluid accumulation greater than what?
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500 mL.
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Treatment of cirrhotic ascites if caused by portal hypertension.
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1. Diuretics to stimulate renal sodium loss. 2. Monitor diuresis closely for electrolyte disturbances and hypovolemia.
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Refractory ascites will occur in what percent of cirrhotic patients despite maximal diuretic therapy?
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10%. *Must be treated with paracentesis, colloid expansion with albumin, TIPS, and eventually transplant.
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2 scoring/classification systems for liver cirrhosis.
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1. Child-Turcotte-Pugh Classification 2. MELD score.
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The Child's score takes into acount what 5 things?
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1. Encephalopathy. 2. Ascites. 3. Bilirubin. 4. Albumin. 5. PT/INR.
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What are the Child classes for severity of Cirrhosis and what points correspond to each.
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Class A = 5-6 points, least severe. Class B = 7-9 points, moderately severe. Class C = 10 to 15 points, most severe. **Note, each criteria is scored 1 to 3 and then all scores are added.
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Which is used more often, the MELD score or Child's Score?
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MELD score.
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3 things used to calculate the MELD score.
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1. Bilirubin. 2. INR. 3. Creatinine concentration.
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What does the MELD score specifically predict? How is it used?
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*30 day mortality. *Used to stratify those on the liver transplant list.
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The MELD score ranges from 6 to 40. When are patients typically considered for liver transplantation?
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*15. *Average score at which patients undergo transplantation is age 20.
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What is the 5 year survival rate for liver transplants?
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70-80%, usually with good quality of life.
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What is the most common indication for liver transplant in the U.S.?
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Hepatitis C etiology.
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What is the most common type of liver cancer?
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Hepatocellular carcinoma.
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What is the cancer marker for hepatocellular carcinoma?
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Alpha-fetoprotein.
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Prognosis with hepatocellular disease.
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*With transplant/resection - 70% 5 year survival. *Widespread, multifocal = 5% 5 year survival rate.
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What is the most common benign tumor of the liver? What is it?
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Hepatomangioma: *Area of liver where the blood cells multiple and form a ball.
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What is the most common benign, non-vascular liver tumor? What is it?
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Fibronodular hyperplasia: *Localized area of hyperplasia with cells becoming more numerous. *Secondary to vascular injury. *Central vein with fibrosis around it = central scar.
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What is an adenoma of the liver? What is it associated with?
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*Enlarged liver cells that are highly vascular [don't biopsy]. *Associated with estrogen and BCP.
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Primary biliary cirrhosis is associated with [intrahepatic/extrahepatic/both] bile duct involvement
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Intrahepatic. *Destruction of small and medium bile ducts.
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Primary biliary cirrhosis is more common in [females/males].
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Females.
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3 symptoms of PBC secondary to destruction of small bile ducts.
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1. Xanghoma from cholesterol back up. 2. Juandice from bilirubin back up. 3. Itching from bile salt back up.
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What is the rule of 90's for PBC?
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90% female. 90% have isolated elevation of alk. phos. 90% have positive Anti-mitochondrial ab [AMA].
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What is the onset of PBC?
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Insidious between age of 40 and 60.
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A patient with PBC is usually [asymptomatic/symptomatic] at presentation.
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Asymptomatic. *Isolated elevation of alk phos might be only clue before the work-up.
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True or False: There is no familial link for PBC.
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False. Like many autoimmune diseases there is an increase risk among first degree relatives.
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4 risk factors for PBC.
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1. Family history. 2. Smoking. 3. Hormone Replacement Therapy.
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Which other autoimmune disorders are highly correlated with PBC?
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1. Celiac. 2. CREST. 3. Sjogren's. 4. Autoimmune thyroid disease. 5. Calcinosis. 6. Raynauds. 7. Esophageal dysmotility. 8. Sclerdactyly. 9. Telangiectasia.
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What is the hallmark serology finding for PBC?
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Positive AMA.
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Elevated bilirubin is a[n] [early/late] finding of PBC.
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Late.
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Diagnosis of PBC is confirmed how?
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Liver biopsy.
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PBC can lead to what 2 things?
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1. Progressive cholestasis. 2. Cirrhosis.
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4 stagese of PBC.
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1. Asymptomatic. 2. Symptomatic. 3. Cirrhotic stage. 4. Liver failure.
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Treatment of PBC
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1. Suppression of underlying pathogenic process with ursodeoxycholic acid. 2. Management of symptoms and complications [naloxone for itching]. 3. Hepatoma surveillance. 4. Might also consider steroids and colchicine.
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What is the only drug for PBC that has been shown to slow progression, increase survivial rate, and prolong time to transplant?
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Ursodeoxycholic Acid.
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Primary Sclerosing cholangitis affects [extrahepatic/intrahepatic/both] ducts.
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Intra and extrahepatic ducts.
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What is primary sclerosis cholangitis?
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Segmental inflammation leading to fibrosis of bile ducts.
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PSC most commonly occurs in what demographic?
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Men younger than 45.
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About 70% of PSC patients also have which other autoimmune disorder?
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Ulcerative colitis [which usually occurs first]
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How is the diagnosis of PSC made?
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MRCP or ERCP.
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Which autoimmune marker is elevated in PSC?
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PANCA antibody.
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What is the characteristic pattern fibrosis in PSC?
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*Circumferential and segmental. *Leads to alternating areas of stricture and non-stricture, giving it a beaded appearance.
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How can the histologic appearance of PSC be described?
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Onion skinning around the bile ducts.
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What is the usual time to death from diagnosis in both PSC and PBC?
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About 12 years.
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Most patients with PSC eventually progress to what?
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End stage liver disease.
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3 complications of PSC.
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1. Cholestasis with biliary cirrhosis. 2. Recurrent cholangitis. 3. Colangiocarcinoma [30-40%].
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What are lab abnormalities in PSC?
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1. Elevated alk phos. 2. AST/ALT mildly elevated. 3. pANCA [sensitive but not specific].
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Symptoms of PSC.
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Similar to PBC: 1. Fatigue. 2. Pruritis. 3. Jaundice. *Most are asymptomatic at presentation.
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True or False: No treatment for PSC slows disease.
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True.
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Why is surgery avoided in PSC?
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Because it is segmental. [similar to Chron's].
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Cholangitis associated with PSC can be treated how?
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Antibiotics and ERCP with stricture dilation.
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What can be given to PSC patietns to help bind bile salts?
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Cholestyramine.
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This drug is used in PBC, but can also improve lab values in PSC.
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ursodeoxycholic acid.
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What is congestive hepatomegaly.
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Liver filled with blood.
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What is steatotic hepatomegaly.
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Liver filled with fat.
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What are the symptoms of hepatomegaly?
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Diffuse, dull discomfort in the RUQ secondary to stretching of the liver capsule.
