Hepatobiliary worksheet with answers-Paulson (exam 3) Flashcards
Primary sclerosing cholangitis is a progressive disease of the liver and _______, characterized by?
- gallbladder
- characterized by inflammation and scarring/fibrosis of intrahepatic and/or extrahepatic bile ducts
Primary sclerosing cholangitis:
-Sx?
- Patients may be asymptomatic, or complain of jaundice, pruritis, fatigue, and RUQ pain
- Hepatomegaly and splenomegaly may also be seen
Primary sclerosing cholangitis:
- Labworks shows a ______ pattern
- positive for which antibody?
- cholestatic pattern is seen, with **ALP usually predominantly elevated
- Positive P-ANCA (Perinuclear Anti-Neutrophil Cytoplasmic Antibody), elevated bilirubin, and elevated ALT/AST are also likely
Primary sclerosing cholangitis:
-Diagnosis is typically made by _____
**MRCP or ERCP–> Multifocal stricturing and dilation of intrahepatic and/or extrahepatic bile ducts can be seen
Primary sclerosing cholangitis is strongly associated with ______
- *inflammatory bowel disease, 90% with ulcerative colitis
- +/- Crohn disease
Primary sclerosing cholangitis:
Pts with PSC are at an increased risk for developing _______
cholangiocarcinoma (type of cancer)
Primary sclerosing cholangitis:
tx?
- The best definitive treatment is liver transplantation.
- Meds: UDCA(ursodeoxycholic acid) often used, though no meds have been shown to slow the disease process
Primary Biliary Cirrhosis is an autoimmune disease of _______
**intrahepatic small bile ducts
Primary Biliary Cirrhosis: pathophysiology
-T cells attack the intrahepatic small bile ducts and they ____
**they are gradually destructed and disappear–> leads to cholestasis–> cirrhosis/liver failure
Primary Biliary Cirrhosis:
-Most Pts are _____
**middle aged women
Primary Biliary Cirrhosis:
Are most patients symptomatic?
-Sx?
NO! Symptoms include fatigue, pruritis RUQ discomfort, jaundice, hepatomegaly.
Primary Biliary Cirrhosis:
- Labs?
- almost all Pts have positive _______ antibodies
- *cholestatic pattern. ALP and GGT usually very elevated
- **Almost all patients have positive antimitochondrial antibodies (AMA)
Primary Biliary Cirrhosis:
-1st line tx?
UDCA (ursodeoxycholic acid)
Autoimmune Hepatitis= a chronic disease with continuing _________
**hepatocellular inflammation and necrosis that tends to progress to cirrhosis
Autoimmune Hepatitis:
-Sx?
-Patients may be asymptomatic, OR may have Sx such as: hepatosplenomegaly, stigmata of chronic liver disease, jaundice, or acute liver failure.
Autoimmune Hepatitis:
- Labs?
- positive for which antibodies?
- Transaminases and bilirubin are elevated
- Smooth muscle antibodies and a positive ANA
Autoimmune Hepatitis:
-what establishes the diagnosis?
**Liver biopsy
Autoimmune Hepatitis:
-tx?
prednisone and azathioprine. (Then taper prednisone down)
Acute Liver Failure definition
The development of severe acute liver injury with encephalopathy and elevated INR (>1.5) in a patient without cirrhosis or preexisting liver disease.
Acute Liver Failure:
what is the MC cause?
-other common causes?
- acetaminophen toxicity= MC
- Other very common causes= drug reactions and viral hepatitis
Acute Liver Failure:
manifestations? (list 6) (pain in which quadrant?)
- encephalopathy (by definition)
- jaundice
- hepatomegaly
- RUQ tenderness
- renal failure
- thrombocytopenia
Acute Liver Failure: which 2 things often complicate acute liver failure?
-Adrenal insufficiency and cardiac injury often complicate acute liver failure
Acute Liver Failure: Labs? (what is elevated)
- PT/INR >1.5
- also ↑ ammonia
- elevated LFTs
Acute Liver Failure:
- tx for tylenol overdoses?
- It is KEY to prevent the development of _____
- acetylcysteine is used
- ***Prevention of the development of cerebral edema is key
Acute Liver Failure:
-definitive treatment?
*liver transplantation
NAFLD(nonalcoholic fatty liver disease) is subdivided into 2 categories:
- NAFL (nonalcoholic fatty liver)
- NASH (nonalcoholic steatohepatitis)
Difference b/w NAFL and NASH?
-In NAFL, hepatic steatosis is present WITHOUT any significant inflammation, but in NASH, hepatic steatosis is associated with inflammation.
NASH may be histologically indistinguishable from _______
**alcoholic steatohepatitis
NAFLD may progress to _______
cirrhosis.
NAFLD: major risk factors?
obesity, DM2, dyslipidemia, metabolic syndrome
NAFLD: Sx?
Most patients are asymptomatic. They might have fatigue, malaise, vague RUQ discomfort.
NAFLD: Labs show mild-moderate elevations of ____
mild-mod elevations of AST/ALT
NAFLD: definitive diagnosis made by ______
-tx?
-liver biopsy.
Treatment: Weight loss. This has been associated with histologic improvement.
-Patients should also avoid heavy alcohol consumption.
Hemochromatosis: definition
Excess iron absorption and deposition in parenchymal cells of the heart, liver, pancreas, adrenals, testes, pituitary, & kidneys.
Hemochromatosis: MC in which population?
Men (usually begin after age 40-50)
-Autosomal recessive.
Hemochromatosis: clinical Sx?
- weakness/lethargy
- skin hyperpigmentation
- arthralgia
- cardiac enlargement +/- heart failure
- diabetes
- ED/impotence
Hemochromatosis: Labs—> serum _____ is elevated with increased _____
-which test can eliminate the need for a liver bx?
- **Serum iron is elevated, with INCREASED transferrin saturation and ferritin
- **Genetic testing can eliminate the need for liver biopsy
Hemochromatosis: tx?
-**Therapeutic phlebotomy until iron stores are depleted, then begin maintenance (less frequently)
Hemochromatosis: tx if Pt CANT tolerate phlebotomy?
-what should these Pts avoid?
- chelation therapy is recommended
- These patients should avoid iron, vitamin C, and alcohol.
