Biliary Cholangitis And Other Causes Of Cirrhosis Flashcards
Two categories of biliary cirrhosis
Intra hepatic: within the liver
Extrahepatic: outside the liver and benefits from biliary tract decompression
What are the four types of biliary cirrhosis causes
Primary biliary cholangitis
Autoimmune cholangitis
Primary sclerosing cholangitis
Idotpathic adulthood ductopenia
What is the general pathophysiology behind biliary cirrhosis
Cholate stasis, copper deposition, xanthamatous, biliary fibrosis, chronic portal inflammation
Who is more common to have primary biliary cholangitis
Females
What is the Pathophysiology behind primary biliary cholangitis cirrhosis
Primary lesion that causes a necrotizing inflammatory process of the GI tract. Leading to fibrosis and bile stasis. The fibrosis eventually develops into cirrhosis
S/s of primary Biliary cholangitis cirrhosis
Typically asymptomatic
Can develop out of proportion fatigue, puritis, jaundice, ascites, edema.
Hyperpigmentation, xanthalasma, xanthaomata, bone pain
Lab findings in primary biliary cholangitis
Elevated Alk phos, AST, ALT, IgM, bilirubin
Decreased thrombocytes, anemia, leukpenia
Primary biliary cholangitis diagnostics
Liver biopsy after AMA is negative
Primary biliary cholangitis treatment
Liver transplant
Pathophysiology behind Primary sclerosing cholangitis cirrhosis
Cause is unknown. Diffuse inflammation and fibrosis involving the entire biliary tree, resulting in chronic cholestasis.
Symptoms of primary sclerosing cholangitis
Fatigue, puritis, steatorrhea, vitamin deficiencies
Lab findings in primary sclerosing cholangitis
Elevated liver enzymes, Alk phos, PT
Decreased albumin
Diagnostics for primary sclerosing cholangitis
MRCP with possible ERCP : high stricturing and compression of biliary tree (poor prognostic)
Treatment for primary scleorsing cholangitis
Liver transplant
Treatment of autoimmune hepatitis
Does not respond to glucocorticoids or immunosupressants because the AIH is “burned out”
Can use immunosuppressants in cases of active inflammation
Diagnosis of autoimmune hepatitis
Liver biopsy: often does not show inflammation
Better to use ANA and ASMA markers
Pathophysiology behind NASH
Not fully understood
NASH diagnosis
Hepatic steatosis on biopsy
Absence of all other etiology
Negative for ETOH consumption
Treatment for NASH
No clear treatment
Life style support
May need transplant
Pathophysiology of cardiac cirrhosis
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
Symptoms of cardiac cirrhosis
CHF symptoms, large firm liver on exam
Elevated Alk phos, AST/ALT may be normal
Diagnosis of cardiac cirrhosis
Liver biopsy
Treatment of cardiac cirrhosis
Manage underlying heart disease
What is hemachromotosis diagnosis and treatment
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
What is Wilson’s disease, diagnostics, and treatment as it relates to cirrhosis
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
What is alpha 1 antitrypsin deficiency and treatment as it relates to liver cirrhosis
Disorder of abnormal folding of the alpha 1 antitrypsin protein, failing to secrete from liver
Transplant
