Yellow deposits near the eyes. Usually indicates high cholesterol if there is no family history of it.
Next Test After Cholestatic Liver Enzymes
Abdominal ultrasound to look at the bile ducts for dilation.
*Note* ducts may not dilate in PSC.
Primary Sclerosing Cholangitis
A disease of the bile ducts that causes inflammation and obliterative fibrosis of the bile ducts inside and/or outside the liver. This impedes the flow of bile to the intestines and can ultimately lead to cirrhosis of the liver, liver failure, and other complications such as bile duct cancer, liver cancer, and colon cancer.
75% of those who have PSC have IBD.
Signs and Symptoms of PSC
Episodes of acute cholangitis
Steatorrhea (poor fat digestion)
Primary Biliary Cholangitis
An autoimmune disease of the liver. It is marked by the slow progressive destruction of the small bile ducts of the liver, with the intralobular ducts and intrahepatic ductules affected early in the disease. When these ducts are damaged, bile and other toxins build up in the liver and causes liver damage over time. This can lead to scarring, fibrosis, and cirrhosis.
Tested for with AMA
Used to test for PBC
Types of PSC
Large duct (95%) and small duct (5%)
Route for Liver Biopsy
Can go through the side into the liver, or can go through the jugular in the neck.
If the patient has low platelets or a high INR, you must go through the neck. Risks of a liver biopsy are bleeding, infection, and pain.
Ursodeoxycholic Acid (UDCA)
Is effective for PBC
May not be good for PSC, but may be as well...
Makes bile more water soluble so it flows out easier. Makes it harder to absorb fat too, so you need to put them on Vitamin ADEK.
Used to treat PBC. The drug reduces cholesterol absorption and is used to dissolve cholesterol gallstones in patients who want an alternative to surgery.
A medical condition as a result of severe infection and sepsis. It can cause multiple organ dysfunction and death.
Presents with hypotension, tachypnea, elevated or low WBC count, tachycardia, and altered body temperature.
Is a whole-body inflammatory response to an infection. Common signs and symptoms include fever, increased heart rate, increased breathing rate, and confusion.
Lab Signs of DIC
Liver tests normal
Blood pressure low/normal
Caused by a defect or absence of ADAMTS13.
vWF multimers cannot be cleaved, which results in increased platelet adhesion to endothelial injuries, particularly at ateriole-capullary junctions.
Complications of PSC
Gall bladder cancer (often removed)
Fat-soluble vitamin deficiencies
Metabolic bone disease