Non-infectious hepatobiliary disease Flashcards
What are the three general patterns of liver disease?
Hepatocellular pattern
Hyperbilirubinemia
Cholestatic pattern
What are the three components of LFTs?
AST/ALT
Alk phos
GGT
What are the two tests that test the synthetic function of the liver?
INR
Albumin
35 yo man with arthralgias, myalgias, and subsequent jaundice = ?
Viral hepatitis
40 yo female with jaundice and severe, sudden RUQ pain and shaking chills = ?
Cholestatic disease
Palmar erythema can be seen in what?
Advanced cirrhosis
Ascites with jaundice usually indicates what?
Cirrhosis or malignancy with peritoneal spread
Virchow’s node or sister mary joseph nodule usually suggests what?
Metastatic malignancy
Liver that is TTP is seen in what?
EtOH liver
Hepatitis
Grossly enlarged and nodular liver suggests what?
malignancy
Hepatocellular disease is usually caused by what?
Alcohol
Hemachromatosis
What is the AST:ALT ratio for EtOH liver?
2:1
What is the cholestatic pattern of liver disease?
Elevated Alk Phos
Which gender is more susceptible to cirrhosis?
Women
What are the histological changes seen in alcoholic liver disease?
Macrovesicular fat accumulation
PMN infiltration
GGT is usually elevated by what amount in alcoholic liver disease?
x2
AST more than 300 indicates what?
Hep C on top of alcoholic cirrhosis
What is the cause of macrocytosis in liver disease?
folate/B12 deficiency, or myelotoxicity of EtOH
What is the cause of thrombocytopenia in alcoholic fatty liver?
Bone marrow suppression, splenic sequestration d/t portal HTN
What is fetor hepaticus?
Thiols pass through the lung from a failing liver, causing horrible breath
What are the two outcomes of alcoholic fatty liver?
alcoholic hepatitis or cirrhosis
What is the treatment for etoh liver disease?
abstinence and liver transplant
What is the cause of ascites in cirrhosis?
Portal HTN causing vasodilatation in the splanchnic system, causing an increase in the renin-angiotensin system, causing water retention
What dietary restriction should be undertaken with ascites?
NaCl restriction
What drug should be avoided in pts with cirrhotic liver and ascites? Why?
NSAIDs
Will shut down kidneys d/t need for prostaglandin
What is the serum ascites:albumin ratio in portal HTN?
> 1.1
What is the goal of treating ascitic fluid?
Reduce w/o intravascular volume depletion
What are the two drugs that are used to treat alcoholic liver disease with ascites?
Spironolactone and furosemide
What is a TIPS used in treating ascites?
Transjugular intrahepatic portosystemic shunt
What happens to the spleen in alcoholic cirrhosis?
Splenomegaly
What are the complications with alcoholic liver disease?
Hepatic encephalopathy
What are the precipitating causes of hepatic encephalopathy?
Hypovolemia GIB Hypoxia Infx hypoglycemia
Where does the ammonia come from in hepatic encephalopathy?
GI bacteria produce it , but liver cannot handle it
What is the treatment for hepatic encephalopathy? How does this work?
Lactulose changes the pH of the intestines, to kill of bacteria
What is spontaneous bacterial peritonitis (SBP) seen in alcoholic liver disease? What two bacteria usually cause this?
Ascitic fluid infection w/o a surgically treatable source–usually Klebsiella or E.Coli
Ascitic fluid with PMN over what number indicate the need for immediate abx? What else should be done?
250 cells/mm
Paracentesis
What protein level in ascitic fluid indicates high risk for the development of SBP?
Less than 1 g per dL
What is the abx of choice for spontaneous bacterial peritonitis?
Cephalosporin
What is the treatment for varices?
Band the varices
Beta-blockers (propanolol)
What is the treatment for variceal rupture?
stabilize them
Protect the airway
Treat the bleed
What is the cause of hepatorenal failure?
portal HTN reduces blood flow to the kidney
What is the discriminant function calculation?
4.6[PTT - PT] + (serum bili)
Over what score for the discriminant function indicates the need for treatment with prednisolone?
32
What causes the BUN to increase in alcoholic pts?
Increased reabsorption of nitrogen
What happens to alk phos in alcoholic cirrhosis?
Mildly elevated
What is the skin pigmentation with hemochromatosis? What cancer are these pts predisposed to?
Gray
hepatocellular CA
What is the pathophysiology with hemochromatosis?
Increased intestinal Fe absorption d/t genetic defect in hepcidin
What are the ssx of hemochromatosis (4)
Hepatomegaly
Gray hyperpigmentation
Arthropathy
Cardiac involvement
What are the joints that are commonly affected in hemochromatosis?
MCPs
What are the labs used to diagnose hemochromatosis?
High serum Fe, ferritin, and transferrin
Transferrin saturation greater than what percent is seen in hemochromatosis?
45%
What is the treatment for hemochromatosis?
Phlebotomy
Symptomatic hemochromatosis is how many times more symptomatic in men than women?
10x
What are the histological characteristics of NAFLD?
Similar to EtOH liver, but without h/o alcohol
What is the LFT enzymatic ratio with NAFLD?
ALT >AST (opposite of alcoholic liver disease)
What is NAFLD associated with?
DM
Fat
Metabolic syndrome
HTN
How do you diagnose NAFLD?
Fatty infiltration on imaging
Liver biopsy
Stigma of chronic liver disease
What is the treatment for NAFLD?
Weight loss
Control DM
Avoid EtOH
What is type I autoimmune hepatitis?
Elevated transaminases with absence of other causes, and associated with other autoimmune disease
What is the lab that is very useful for diagnosing autoimmune hepatitis?
Serum protein electrophoresis (more than 80-% of pts have hypergammaglobulinemia)
What are the Abx seen in autoimmune hepatitis? (5)
ANA Anti-dsDNA **SMA** ANCA anti-actin
How do you confirm the diagnosis of autoimmune hepatitis?
Liver biopsy
What is the treatment for autoimmune hepatitis? Is there a cure?
Prednisone and azathioprine
No cure, but very good prognosis
What is the cause of Wilson’s disease?
Impaired excretion of Cu into bile and build up
What is the genetic cause of Wilson’s disease?
AR
What is the cause of arthritis seen in Wilson’s disease?
Chondrocalcinosis
When are pts diagnosed with Wilson’s disease, usually?
5-25 yo
What are the ceruloplasmin levels in Wilson’s disease?
Low
How do you confirm the diagnosis of Wilson’s disease?
Liver biopsy
What is the treatment for Wilson’s disease?
Chelation with penicillamine or trientine/Zn salts
Is there a cure for Wilson’s disease?
yes–liver transplant
What is the pathophysiology of Alpha-1-antitrypsin deficiency?
Pathologic polymerization of variant AAT molecules that accumulate in hepatocytes
How do you diagnose alpha-1-antitrypsin deficiency?
Direct serum measurement of APR
Genotype testing
What is the treatment for alpha-1-antitrypsin deficiency?
Liver transplant
What are the two causes of conjugated bili elevation?
Decreased excretion
Backward leakage
In whom is Gilbert syndrome common?
Young, white males
What are the bilirubin findings with Gilbert syndrome?
Unconjugated is less than 6 mg/dL, but high
What are the 4 sources of Alk phos in the body? How do you determine if it is from the liver?
Liver
Bone
Prego (baby)
Gut
GGT will be elevated if it is from the liver.
AMA ab is diagnostic for what biliary disease?
Primary biliary cirrhosis
What is intrahepatic cholestasis? What diseases is this associated with?
not dilated bile duct, but slow bile movement
Primary sclerosing cholangitis
Primary biliary cirrhosis
What is primary sclerosing cholangitis?
Progressive disease with inflammaiton, fibrosis, and sclerosis of intra and extra hepatic bile ducts
What disease is primary sclerosing cholangitis associated with?
UC
What cancer can primary sclerosing cholangitis are susceptible to?
Cholangiocarcinoma
What ab is elevated in Primary sclerosis? Which is NOT elevated?
Elevated = pANCA NOT = AMA
What mineral is element is elevated in the liver of patients with primary sclerosing cholangitis?
Cu
Beaded appearance to the bile duct = what disease?
primary sclerosing cholangitis
What are the things that are needed to diagnose Gilbert syndrome?
Unconjugated hyperbilirubinemia
+ no evidence of hemolysis
What is the characteristic finding of a liver biopsy with primary sclerosing cholangitis?
Onion skin appearance
In whom is primary biliary cirrhosis common?
Middle aged women
What are the diagnostic labs for primary biliary cirrhosis?
Elevated alk phos
AMA +
Does the AMA antibody found in primary biliary cirrhosis correlate with the disease severity?
No