B5-025 Liver Disorders I Flashcards
the dual blood supply of the liver comes from
2
- portal vein
- hepatic vein
macrophages that live in the sinusoids of the liver
Kupffer cells
lined with simple cuboidal epithelium
“string of pearls”
bile duct
blood in zone one is […] in oxygen
rich
blood in zone 3 is […] in oxygen
low
there is a more dense distribution of cytochrome p450 enzymes in zone […]
3
large cells with centrally located nucleus and prominate nucleolus
hepatocytes
hepatocytes are connected to their neighbor hepatocytes through a
bile cannaliculus
space between hepatocytes and sinusoids
space of Disse
stellate cells live in the
space of Disse
what do stellate cells do in their quiescient state?
store fat-soluble vitamins
ADEK
in a normal adult state, the cell plates should be […] cells thick
how many cells
1
2 cells max (regenerative state)
3+ indicates neoplastic process
feathery degeneration indicates an accumulation of
bile
steatosis can be due to
3
obesity, alcohol, diabetes
accumulation of iron in a canalicular distribution
hemochromatosis
accumulation of copper
Cu not excreted in bile
Wilson’s disease
accumulation of misfolded protein in the RER
a1 antitrypsin deficiency
old age/”wear and tear” pigment seen in hepatocytes
lipofuscin
- clumped cytokeratin filaments
- ballooned degeration
Mallory hylanine
steatohepatitis, alcohol use
- hyperpigmentation of the skin
- jaundice
- diabetes
genetic hemochromatosis
“bronze diabetes”
what lab values would you expect to be elevated in hemochromatosis?
4
- AST/ALT
- serum iron
- transferrin
- serum ferritin
treatment for hereditary hemochromatosis
weekly phlebotomy and deferoxamine
inheritance pattern of hereditary hemochromatosis
bonus points if you name the gene
autosomal recessive inheritance of HFE gene
HFE regulates
hepcidin
controls how much iron is absorbed in intestine
- caused by inappropriately high intestinal iron absorption
- results in excess body iron stores
hereditary hemochromatosis
why do most cases of hereditary hemochromatosis present after age 40?
- takes a long time to accumulate
- cirrhosis occurs when the body iron exceeds 30-40 g around age 40
risk factors for hereditary hemochromatosis
- male gender
- hepatitis C
- alcohol
PAS-diatase resistant globules
a1 antitrypsin deficiency
Piz allele
alpha 1 antitrypsin deficiency
present with cirrhosis of the liver
and early onset empysema, chronic bronchitis, asthma, etc
alpha 1 antitrypsin deficiency
all patients with PiZZ genotype accumulate […] in hepatocytes
alpha 1 antitrypsin deficiency
- resting tremor
- brownish ring around cornea
wilson’s disease
what labs would you expect to be abnormal in Wilson’s disease?
- decreased ceruloplasmin
- elevated urine copper
mutation in ATPase leading to defective conjugation of copper in ceruloplasmin
Wilson’s disease