Liver Diseases Flashcards
Wilson’s disease
Path: copper accumulation
Pt: brain (choreas), liver (cirrhosis), eyes (kaiser fleischer rings)
Low ceruloplasmin
wilson disease
heaptic steatosis
macrovesicular fatty change, reversible with alcohol cessation
NASH
non-alcoholic fatty liver disease metabolic syndrome: insulin resistance fatty infiltration of hepatocytes, cellular ballooning eventual necrosis and HCC High ALT > AST (L is for Lipids)
How do we get hepatic encephalopathy?
cirrhosis leads to portosystemic shunts, which decreases NH3 metabolism, leading to neutopsychiatric dysfuction
Budd-Chiari syndrome
thromcosis or compression of hepatic veins with congestion and necrosis. leads to congestive liver disease. nutmeg liver
a1-antitrypsin deficiency
this guy is supposed to break down elastase in the lungs but since it cant escape liver in disease state, we see elastase growing in the lungs like crazy, less elastic tissue, and more likely to develop emphysema
Diagnosing a1-antitrypsin deficiency
cirrhosis with PAS + globules (macrophages)
inheritance of wilsons disease and hemochromatosis
autosomal recessive
what normally happens to copper in our body?
excreted into bile by hepatocyte coppor transporting ATPase
Hemochromatosis disease
genetic mutation for increased Fe absorption leads to overload
hemochromatosis patient
presents with “bronze” diabetes, skin pigmentation, and cirrhosis
Treatment for hemochromatosis?
repeated phlebotomy
oral chelation therapy
Primary Sclerosing Cholangitis
MEN with pruritis, jaundice, aged 30-50 seen with IBD biopsy shows onion skin fibrosis of bile duct and beading extrahepatic need transplant
Primary biliary cholangitis
middle aged WOMEN with pruritis, jaundice
more subtle
autoimmune reaction causes lymphocytic inflitration, granulomas, and destruction of intrahepatic bile ducts
