chronic liver disease Flashcards
(41 cards)
PBC affects
women more
PBC pathophysiology
T-lymphocyte mediated attack on bile duct epithelial cells
what antibodies are found in PBC
Antimitochondrial antibodies
PBC symptoms
Pruritus, with or without jaundice
hepatosplenomegaly and xanthelasma
=
PBC signs
a raised serum alkaline phosphatase or autoantibodies
steatorrhoea and malabsorption of fat-soluble vitamins
what is the abnormality in the liver biochemistry in PBC
ALP
what do you look for in the blood for PBC
Serum AMA
treatment of PBC
lifelong ursodeoxycholic acid
what is ursodeoxycholic acid
natural bile acid
how can you help with pruritus in PBC
cholestyramine
PBC prognosis
jaundice = die in 5 years asymptomatic = normal life expectancy
Hereditary haemochromatosis inheritance
autosomal recessive
what is Hereditary haemochromatosis
iron deposition in organs leading to fibrosis
in Hereditary haemochromatosis what is the mutation
HFE
what is HFEs normal job
protein in the small intestine allowing absorption of iron
what is raised in Hereditary haemochromatosis
serum iron
management of Hereditary haemochromatosis
removing blood twice a week
big complication with Hereditary haemochromatosis
liver cirrhosis
wilsons inheritance
recessive
what happens in wilsons
decreased secretion of copper into the biliary system
and reduced incorporation of copper = accumulation
in wilsons where does copper accumulate
liver
basal ganglia
cornea
renal tubules
affects of wilsons
liver = cirrhosis
basal ganglia = Parkinsonism/dementia
cornea
renal tubules
signs of wilsons
ring in the cornea
diagnosing wilsons
low total serum copper and caeruloplasmin
urinary copper