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Alcohol-induced liver disease
Alpha 1-antitrypsin deficiency
Benign liver tumors
Cholestatic liver disease
Non-alcoholic fatty liver disease
Primary biliary cirrhosis
Primary sclerosing cholangitis
Pancreatic neuroendocrine neoplasms
Familial adenomatous polyposis
Juvenile polyposis syndrome
Small bowel ischemia and infarction
Protein losing enteropathy
Short bowel syndrome (NORD)
Small bowel bacterial overgrowth syndrome
Diverticulosis and diverticulitis
Irritable bowel syndrome
Cleft lip and palate
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Diffuse esophageal spasm
Eosinophilic esophagitis (NORD)
Gastroesophageal reflux disease (GERD)
Cyclic vomiting syndrome
Gastric dumping syndrome
Dental caries disease
Gingivitis and periodontitis
Temporomandibular joint dysfunction
Appendicitis: Pathology review
Cirrhosis: Pathology review
Colorectal polyps and cancer: Pathology review
Congenital gastrointestinal disorders: Pathology review
Diverticular disease: Pathology review
Esophageal disorders: Pathology review
Gallbladder disorders: Pathology review
Gastrointestinal bleeding: Pathology review
GERD, peptic ulcers, gastritis, and stomach cancer: Pathology review
Inflammatory bowel disease: Pathology review
Jaundice: Pathology review
Malabsorption syndromes: Pathology review
Neuroendocrine tumors of the gastrointestinal system: Pathology review
Pancreatitis: Pathology review
Viral hepatitis: Pathology review
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A patient perspective | Tilly Hale | TEDxNewcastle
Primary Biliary Cholangitis (Primary Biliary Cirrhosis)
Primary Biliary Cholangitis (Primary Biliary Cirrhosis) Diagnosis and Treatment
Primary biliary cholangitis
cystic fibrosis p. 58
labs/findings p. 729
biliary cirrhosis and p. 402
biliary cirrhosis and p. 403
in biliary cirrhosis p. 402
as granulomatous disease p. NaN
biliary cirrhosis p. 402
Primary biliary cholangitis and cirrhosis, or PBC, is an autoimmune disease where the T cells attack the cells that line the smaller-sized bile ducts in the liver.
As the cells are damaged or destroyed by immune attack, they start letting bile leak through their tight junctions and into the interstitial space, where it can get into the blood and the other liver cells.
This can cause inflammation and symptoms that are similar to cholestasis and obstructive jaundice, both of which there’s some disturbance in bile flow.
At first it presents as chronic inflammation, which is where it gets the name cholang-itis, for inflammation of the bile ducts, but advanced stages of the disease leads to cirrhosis as more cells are destroyed both from the autoimmune attack and infiltration of bile.
As with a lot of autoimmune diseases, the exact reason why T-cells start attacking these bile duct cells is unknown, and it’s likely related to genetic predisposition in combination with environmental triggers, and is associated with other autoimmune diseases like autoimmune hepatitis and Sjogren’s syndrome.
PBC tends to affect women at a much higher rate than men, about a 9:1 female to male ratio.
For some reason that has yet to be completely figured out, in almost all cases of PBC, your body creates antibodies to mitochondria, specifically a mitochondrial protein called PDC-E2; for that reason, one of the most notable markers in PBC are these antibodies to mitochondrial proteins (or AMAs) in the patient’s blood, as many as 95% of patients with PBC have AMAs in their blood.
Why though, are the bile duct cells specifically targeted? Don’t all cells have mitochondria? Shouldn’t these AMA’s be targeting all cells then?
One theory is based on a concept called molecular mimicry, where the antigens that bring about this autoimmune response are really similar to another protein, but different enough so that the immune system gets confused and thinks it’s a foreign version of that particular protein.
Here’s an example of two people’s cells, they both have this triangle protein, but they have different colored triangles, each person’s immune knows that these are their own proteins, their own self-proteins and that they shouldn’t attack, but they have antibodies for proteins that are very similar, but definitely different from self, so say they have antibodies for triangles, just different colored triangles, so like person A has red, yellow, and blue, just not green, and person B has green, yellow, and blue, and not red. So both these people’s immune systems are like yep, these are all self so we’re good.
Primary biliary cirrhosis (PBC), also referred to as primary biliary cholangitis, is a chronic autoimmune disease that affects the liver. It is characterized by the progressive destruction of the small bile ducts in the liver, which leads to inflammation, fibrosis, and ultimately cirrhosis (scarring) of the liver. Symptoms may not appear until the later stages of the disease but can include fatigue, itching, jaundice, and abdominal pain. Treatment involves drugs like ursodeoxycholic acid (UDCA) and cholestyramine, which slow the progression of the disease and improve symptoms. In advanced cases, liver transplantation may be necessary.
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