Hello and welcome to the Zero to Finals podcast. My name is Tom and in this episode I'm going to be talking to you about primary biliris cirrhosis.

If you want to follow along with written notes on this topic, you can follow along at zero tofinals.com

slash primary biliris cirrhosis or in the gastroenterology section of the zero definals medicine book.

Let's get straight into it.

Let's start with some basic pathophysiology.

Primary bilari cirrhosis is a condition where the immune system attacks the small bile ducts in the liver.

And the first parts to be affected are the intra-loba ducts, also known as the canals of herring.

This causes obstruction of the outflow of bile, which is a process called colostasis,

so bile can't flow from the liver down the bile duct and into the gut.

The back pressure of the bile obstruction and the overall disease process ultimately leads to fibrosis, cirrhosis and liver failure.

Bial acids, bilirubin and cholesterol are normally excreted in the bile, through the bile ducts and

into the intestines. When there's an obstruction of the outflow of these chemicals, they build up in the

blood as they're not being excreted. The bile acids cause itching. The bilirubin causes that yellow

discoloration of the skin that we call jaundice.

And the raised cholesterol causes cholesterol deposits in the skin, which is what we call exanthalasma.

And remember that exanthomas are large nodular deposits in the skin or the tendons, which is the

same disease process where cholesterol is being deposited in the skin or the tendons, which is the same disease process where cholesterol's

being deposited in the skin because there's an excess amount in the blood. And the blood vessels

also get clogged up with cholesterol and this causes an increased risk of cardiovascular disease.

Bile acids are normally responsible for helping the gut to digest fats. So having a lack of

bile in the gut causes gastrointestinal disturbances, malabsorption of fats and greasy stools because

that fat is just passing straight through instead of being digested properly. Billy Rubin is normally responsible for the dark colour of the stools.

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