Cirrhosis Flashcards
Cirrhosis =
A serious, irreversible disease caused by exposure to certain toxins that cause heptocellular injury and compromise liver function
Pathophysiology
Late stage of progressive hapatic fibrosis
Irreversible chronic injury of the hepatic parenchyma
Extensive fibrosis - distortion of the hepatic architecture
Formation of regenerative nodules
Common Causes
ETOH
Hepatitis B,D and C
Various drugs - acetaminophen, amiodarone, chemotherapeutic agents, antibiotics and carbon tetrachloride
Cause can be inherited or idiopathic
How much ETOH to cause cirrhosis?
Estimated that the development of cirrhosis requires, on average, the ingestion of 80 grams of ethanol daily for 10 to 20 years
Daily Intake of:
1 liter of wine,
8 beers, or
1/2 pint of hard liquor
Clinical Presentation
Patients present in a variety of ways Symptoms may be insidious or abrupt Fatigue is common Other nonspecific complaints: Weakness, malaise,pruritus, weight loss Hematemesis (15-25%) Abdominal pain Menstrual abnormalities, impotence. sterility, gynecomastia in men As disease progresses, anorexia is common
Clinical Findings
Signs assoc with clotting dysfxn:
Bruising
Hematemesis
Hematochezia
Other signs: Low-grade fever Anorexia Jaundice RUQ pain
Liver may be nodular, firm, enlarged or shrunken (late stages)
Ascites
Peripheral edema
Physical Findings
Spider angioma - vascular lesion consisting of central arterial surrounded by many smaller vessels. central arterial can be seen pulsating when compressed with glass slide Palmar erythema Gynecomastia Testicular atrophy Fetor Hepaticus - sweet pungent smell of breath Jaundice Asterixis Pigment gallstones Parotid gland enlargement Cruveilhier-Baumgarten murmur Hepatomegaly Splenomegaly Caput medusa
Physical Findings: Nail Changes
Muehrcke’s Nails - paired horizontal white bands separated by normal color (not specific for cirrhosis)
Terry’s Nails - proximal 2/3 of nailbed is white while distal 1/3 is red
Both thought to be r/t low serum albumin
Diagnostic Labs: Aminotransferases
Aminotransferases — Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) are usually moderately elevated. AST is more often elevated than ALT. However, normal aminotransferases do not preclude a diagnosis of cirrhosis. Most forms of chronic hepatitis other than alcohol have a ratio of AST/ALT less than one. However, as chronic hepatitis progresses to cirrhosis, the ratio of AST to ALT may reverse.
Alkaline phosphatase — Alkaline phosphatase is usually elevated but less than two to three times the upper normal limit. Higher levels may be seen in patients with primary sclerosing cholangitis and primary biliary cirrhosis
Diagnostic Labs:
Bilirubin
Albumin
Bilirubin — Bilirubin levels may be normal in well compensated cirrhosis. However, they rise as the cirrhosis progresses. In patients with primary biliary cirrhosis, a rising serum bilirubin portends a poor prognosis.
Albumin — Albumin is synthesized exclusively in the liver. Albumin levels fall as the synthetic function of the liver declines with worsening cirrhosis. Thus, serum albumin levels can be used to help grade the severity of cirrhosis. Hypoalbuminemia is not specific for liver disease since it may be seen in many other medical conditions such as congestive heart failure, the nephrotic syndrome, protein losing enteropathy, or malnutrition.
Diagnostic Labs: Prothrombin Time
Prothrombin time — The liver is involved in the synthesis of many of the proteins required for normal clotting. Thus, the prothrombin time reflects the degree of hepatic synthetic dysfunction. The prothrombin time increases as the ability of a cirrhotic liver to synthesize clotting factors diminishes.
Diagnostic Labs: Globulins
Globulins — Globulins tend to be increased in patients with cirrhosis. This may be secondary to shunting of bacterial antigens in portal venous blood away from the liver to lymphoid tissue which induces immunoglobulin production.Marked elevations of IgG may be a clue to the presence of autoimmune hepatitis. Increased levels of IgM are present in 90 to 95 percent of patients with primary biliary cirrhosis.
Diagnostic Labs: Serum Sodium
Serum sodium — Hyponatremia is common in patients with cirrhosis with ascites and is related to an inability to excrete free water. This results primarily from high levels of anti-diuretic hormone secretion. Hyponatremia often becomes severe as cirrhosis progresses to end-stage liver disease
Hematologic Abnormalities
Patients with cirrhosis commonly have a number of hematologic abnormalities, including disorders of coagulation and varying degrees of cytopenia. Thrombocytopenia is the most common first hematologic abnormality while leukopenia and anemia develop later in the disease course
Anemia—Anemia is usually multifactorial in origin; acute and chronic gastrointestinal blood loss, folate deficiency, direct toxicity due to alcohol, hypersplenism, bone marrow suppression (as in hepatitis-associated aplastic anemia), the anemia of chronic disease (inflammation), and hemolysis may all contribute.
Thrombocytopenia—Thrombocytopenia is mainly caused by portal hypertension with attendant congestive splenomegaly. An enlarged spleen can result in temporary sequestration of up to 90 percent of the circulating platelet mass. However, this uncommonly results in platelet counts less than 50,000/mL, and, unless complicated by coexisting coagulopathy, is rarely a clinical problem. Decreased thrombopoietin levels may also contribute to thrombocytopenia.
Leukopenia and neutropenia—Leukopenia and neutropenia are due to hypersplenism with splenic margination.
DD
Primary Biliary Cirrhosis Secondary Biliary Cirrhosis Cardiac Failure Hemochromatosis Wilson’s Disease Uremia Nephrotic Syndrome Pericarditis Blood Dyscrasias Biliary Disease Hepatitis Thrombosis Tumor Alpha 1-antitrypsin deficiency Nonalcoholic steatohepatitis (NASH) Primary sclerosing cholangitis Parasitic infection Pancreatitis Common bile duct obstruction
Radiographic Dx Tests
Radiographic Findings:
not used as a primary diagnostic modality. Major utility of radiography in the evaluation of the cirrhotic patient is in its ability to detect complications of cirrhosis
Ultrasonography:
used to confirm liver size, assess portal circulation, determine presence of ascites or tumor
Bx
Confirmed Dx test to confirm cirrhosis and determine cause of liver dsfxn
Sensitivity 80-100%
Not necessary if clinical and lab data strongly suggest presence of cirrhosis
Other dx tests
CT not routinely used
MRI role in dx is unclear
Abdominal paracentesis
Abdominal fluid analysis
(to identify bacterial peritonitis or peritoneal carcinomatosis) indicated in the presence of ascites
Upper endoscopy
is recommended to diagnose esophageal varices
Prognosis Tool
Child-Turcotte-Pugh (CTP) score
The current CTP scoring system is based upon five parameters: serum bilirubin, serum albumin, prothrombin time, ascites and encephalopathy. The sum of the points for each of these five parameters gives the total score. Patients with chronic liver disease are placed in one of three classes (A, B, or C)
a good predictor of outcome in patients with complications of portal hypertension
based upon subjective parameters such as the degree of ascites and encephalopathy
may be altered substantially by medical intervention
The Child-Pugh classification system correlates with survival; one-year survival rates for patients with Child’s A, B, and C cirrhosis are approximately 100, 80, and 45 percent, respectively.
Tx
The major goals of treating patients with cirrhosis include:
Slowing or reversing the progression of liver disease
Preventing superimposed insults to the liver
Preventing and treating the complications
Determining the appropriateness and optimal timing for liver transplantation
Interferon Therapy
Lamivudine
slows the progression of cirrhosis in patients with chronic hepatitis C virus infection, and may also decrease fibrosis and the risk of hepatocellular carcinoma
Treatment of patients with chronic hepatitis B with lamivudinemay result in significant improvement in liver function and histology and reduces the risk of hepatocellular carcinoma.
Preventing Superimposed Insults to Liver
Eliminate ETOH and hepatotoxic drugs like NSAIDs
Vaccinations
can prevent superimposed insult to a liver that may have little functional reserve
Liver Transplantation
Liver transplantation is indicated as treatment for select patients with irreversible, progressive chronic liver disease
Management
Complex and requires coordinated efforts by specialists
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