5.2 - Intrahepatic Biliary Tract Disease Flashcards
1
Q
What is and what causes Secondary Biliary Cirrhosis?
A
- Arises from uncorrected obstruction of the extrahepatic biliary tree leading to prolonged cholestasis (bile stasis) what causes inflammation, which in turn leads to per-portal fibrosis and eventually cirrhosis.
- Most common causes: cholelithiasis (gallstones), malignancies of biliary tree or pancreatic head, strictures from previous surgical procedures; biliary atresia, cystic fibrosis
2
Q
What is primary biliary cirrhosis?
A
An autoimmune destructive disorder of the intrahepatic biliary tree leading to portal inflammation and progressing over decades to cirrhosis; occurs primarily in middle-age women.
3
Q
What are the labs/diagnostic tests associated with primary biliary cirrhosis?
A
- Abnormal liver function tests, including alkaline phosphatase, mild elevation of ALT and AST and present/increased immunoglobulins
- In patients WITHOUT cirrhosis, the degree of elevation of alkaline phosphatase is strongly related to the severity of ductopenia (a reduction in the number of bile ducts in the liver) and inflammation.
- Hyperbilirubinemia also reflects the severity of ductopenia and biliary necrosis.
- Histologic evidence of non-suppurative destructive cholangitis and destruction of interlobular bile ducts.
4
Q
How do you manage patients with primary biliary cirrhosis?
A
- Only approved treatment is ursodiol (ursodeoxycholic acid) 13-15mg/kg/day
- May lead to need for liver transplantation.
5
Q
What is/causes primary sclerosing cholangitis?
A
- Chronic cholestatic liver disease distinguished by inflammation and fibrosis of BOTH intrahepatic and extrahepatic bile ducts leading to multifocal bile duct strictures.
- Patients with PSC typically also have ulcerative colitis or Crohn’s disease (approx. 70%).
- Likely autoimmune-mediated and progressive disorder usually leading to cirrhosis
6
Q
What lab/diagnostic tests are used to diagnose Primary Sclerosing Cholangitis?
A
- Elevated cholestatic profile: elevated alkaline phosphatase and AST/ALT
- Cholangiography: either performed by MRI cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP); PSC changes include multifocal bile duct strictures and segmental dilations
- ERCP is Gold Standard for diagnosis PSC.
- Patients with PSC are at increased risk for developing cholangiocarcinoma (7-9%)
7
Q
How do you manage patient’s with Primary Sclerosing Cholangitis?
A
- Endoscopic management of strictures to relieve biliary obstruction is major treatment for PSC
- Patients who are experiencing symptoms, such as pruritus, cholangitis or jaundice and worsening liver function are candidates for endoscopic management.
- Only long-term effective treatment for PSC is liver transplantation.
8
Q
What is/causes Fulminant Liver Failure/Acute Liver Failure (ALF)?
A
- Massive acute liver destruction associated with encephalopathy and elevated PT (>1.5) within 6 months of initial diagnosis and occurswhen > than 80-90% of hepatic function is lost
- Caused by hepatic necrosis attributable to drug or toxin injury, viral hepatitis or autoimmune damage, veno-occlusive disease, Wilson disease, sepsis, heat stroke
- Acute kidney injury complicated ALF in approx. 30-70% of patients
Mortality rate approximately 80%
9
Q
What is important to note when conducting a history on a patient in acute liver failure?
A
- Timing of symptom onset
- History of alcohol use
- History of prior episodes of jaundice
- Medication use
- Risk factors for intentional drug overdose- history of depression or prior suicide attempts
- Toxin exposure, including occupational toxin exposures or wild mushroom ingestion
- Risk factors for acute viral hepatitis, including travel to endemic areas, IV drug use, occupational exposure, sexual exposure, chronic HBV infection, immunosuppression
- Risk factors for hepatic ischemia, including hypotension, cardiac failure, hypercoagulable disorder, oral contraceptive use or malignancy
- Family history of liver disease, such as Wilson disease.
10
Q
What are the subjective symptoms associated with acute liver failure?
A
- Weakness/fatigue/mental status changes
- Jaundice/Pruritus
- Anorexia/Weight loss
- Abdominal discomfort – RUQ or generalized distention from ascites
- Nausea and vomiting
11
Q
What labs should be ordered to diagnose acute liver failure?
A
- ABG
- Lactate
- toxicology screen
- Acetaminophen level
- viral hepatitis serology’s
- ceruloplasmin
- autoimmune markers
- HIV serology
- amylase & lipase (elevated)
- PT/INR (> 1.5)
- aminotransferase levels ( often markedly elevated)
- bilirubin level ( elevated)
- CBC/platelet count ( low- thrombocytopenic)
- ammonia level (elevated)
- Cr/BUN (frequently elevated)
- albumin (low)
- Blood type and screen
12
Q
What diagnostic tests should be ordered to diagnose acute liver failure?
A
- CT abdomen (? Use of contrast if AKI is present) – identifies evidence of malignant infiltration, hepatomegaly, ascites, hepatic vein occlusion, portal vein occlusion
- U/S abdomen – with Doppler imaging preferred; initial diagnostic exam (inexpensive, non-invasive) useful in identifying portal hypertension, hepatic steatosis, hepatic congestion, underlying cirrhosis, malignant infiltration
- CT or MRI head – identifies cerebral edema
- CXR – identifies pulmonary edema/infection
- Consider Liver biopsy if labs and imaging tests fail to identify an etiology- may help with diagnosis of malignant infiltration, autoimmune hepatitis, Wilson disease, hepatitis due to herpes simplex virus and acute fatty liver of pregnancy; due to coagulopathy, the biopsy is performed via a Trans jugular approach instead of a percutaneous approach
13
Q
In general, how do you manage a patient with fulminant liver failure/acute liver failure?
A
- Patients should be hospitalized, preferably in ICU
- Hepatology consult asap
- Transplant center should be contacted and transfer arranged early in the evaluation process, since patient’s condition can deteriorate rapidly.
- Liver transplantation has the BEST OUTCOME in fulminant liver disease
14
Q
How do you manage a patient in acute liver failure due to acetaminophen toxicity?
A
- If ingestion within 4 hours, give activated charcoal (1 gm/kg orally)
- Begin N-acetylcysteine in any suspected or possible acetaminophen toxicity (140mg/kg PO or NGT, diluted in 5% solution, followed by 70mg/kg every 4 hours x 17 doses
15
Q
How do you manage a patient in acute liver failure due to viral hepatitis?
A
- Supportive care for viral hepatitis A and E; no virus specific treatment is available
- Acute hepatitis B should be treated with one of the hepatitis B antiviral agents
19
Q
What are the 4 clinical grades of encephalopathy?
A
Grade 1: (prodrome)
Grade 2:
Grade 3: Severe Encephalopathy
Grade 4: Coma
21
Q
What is chronic liver disease?
A
- Cirrhosis represents a late stage of progressive hepatic fibrosis characterized by distortion of the hepatocytes and formation of regenerative nodules
- Irreversible in late stages
- In earlier stages, specific treatments are aimed at the underlying cause so as to improve or reverse cirrhosis.
- Can lead to multi-organ failure
