Biliary Cholangitis And Other Causes Of Cirrhosis

Question 1

Two categories of biliary cirrhosis

Answer 1

Intra hepatic: within the liver
Extrahepatic: outside the liver and benefits from biliary tract decompression

Question 2

What are the four types of biliary cirrhosis causes

Answer 2

Primary biliary cholangitis
Autoimmune cholangitis
Primary sclerosing cholangitis
Idotpathic adulthood ductopenia

Question 3

What is the general pathophysiology behind biliary cirrhosis

Answer 3

Cholate stasis, copper deposition, xanthamatous, biliary fibrosis, chronic portal inflammation

Question 4

Who is more common to have primary biliary cholangitis

Answer 4

Females

Question 5

What is the Pathophysiology behind primary biliary cholangitis cirrhosis

Answer 5

Primary lesion that causes a necrotizing inflammatory process of the GI tract. Leading to fibrosis and bile stasis. The fibrosis eventually develops into cirrhosis

Question 6

S/s of primary Biliary cholangitis cirrhosis

Answer 6

Typically asymptomatic
Can develop out of proportion fatigue, puritis, jaundice, ascites, edema.
Hyperpigmentation, xanthalasma, xanthaomata, bone pain

Question 7

Lab findings in primary biliary cholangitis

Answer 7

Elevated Alk phos, AST, ALT, IgM, bilirubin
Decreased thrombocytes, anemia, leukpenia

Question 8

Primary biliary cholangitis diagnostics

Answer 8

Liver biopsy after AMA is negative

Question 9

Primary biliary cholangitis treatment

Answer 9

Liver transplant

Question 10

Pathophysiology behind Primary sclerosing cholangitis cirrhosis

Answer 10

Cause is unknown. Diffuse inflammation and fibrosis involving the entire biliary tree, resulting in chronic cholestasis.

Question 11

Symptoms of primary sclerosing cholangitis

Answer 11

Fatigue, puritis, steatorrhea, vitamin deficiencies

Question 12

Lab findings in primary sclerosing cholangitis

Answer 12

Elevated liver enzymes, Alk phos, PT
Decreased albumin

Question 13

Diagnostics for primary sclerosing cholangitis

Answer 13

MRCP with possible ERCP : high stricturing and compression of biliary tree (poor prognostic)

Question 14

Treatment for primary scleorsing cholangitis

Answer 14

Liver transplant

Question 15

Treatment of autoimmune hepatitis

Answer 15

Does not respond to glucocorticoids or immunosupressants because the AIH is “burned out”
Can use immunosuppressants in cases of active inflammation

Question 16

Diagnosis of autoimmune hepatitis

Answer 16

Liver biopsy: often does not show inflammation
Better to use ANA and ASMA markers

Question 17

Pathophysiology behind NASH

Answer 17

Not fully understood

Question 18

NASH diagnosis

Answer 18

Hepatic steatosis on biopsy
Absence of all other etiology
Negative for ETOH consumption

Question 19

Treatment for NASH

Answer 19

No clear treatment
Life style support
May need transplant

Question 20

Pathophysiology of cardiac cirrhosis

Answer 20

Right ventricle increased congestion pressure, increasing pressure in IVC, increasing pressure in hepatic veins, liver congestion/swelling, decreased blood flow and oxygenation to liver tissue, ischemia leading to necrosis and fibrosis

Question 21

Symptoms of cardiac cirrhosis

Answer 21

CHF symptoms, large firm liver on exam
Elevated Alk phos, AST/ALT may be normal

Question 22

Diagnosis of cardiac cirrhosis

Answer 22

Liver biopsy

Question 23

Treatment of cardiac cirrhosis

Answer 23

Manage underlying heart disease

Question 24

What is hemachromotosis diagnosis and treatment

Answer 24

Inherited disorder of iron metabolism, leading to depositions in the liver, which leads to fibrosis/cancer/ cirrhosis
Diagnostics: serum iron, elevated transferrin and ferritin, HFE mutation
Treatment: phlebotomy

Question 25

What is Wilson’s disease, diagnostics, and treatment as it relates to cirrhosis

Answer 25

Inherited copper disorder with failure to excrete leading to accumulation in the liver
Typically young adults
24 hr urine copper, Liver biopsy, kaiser flesher corneal rings
Cooper chelation drugs : Penicillamine, trientine, dimercaprol

Question 26

What is alpha 1 antitrypsin deficiency and treatment as it relates to liver cirrhosis

Answer 26

Disorder of abnormal folding of the alpha 1 antitrypsin protein, failing to secrete from liver
Transplant