chronic liver disease Flashcards
what is the definition of a chronic liver disease
one which can lead to cirrhosis
PBC
disease of the liver where interlobular bile ducts are damaged by chronic autoimmune granulomatous inflammation causing cholestasis which may lead to fibrosis, cirrhosis and portal hypertension
PBC epidemiology and classical presentation
women middle aged
classical patent is bus pass collecting women who is tired and itchy
PBC S/S
xanthomas
jaundice - appears later
itch without rash
fatigue
PBC Dx
2 of AMA, cholestastic LFT and liver biopsy
management PBC
Urseodeoxycholic acid
transplant if severe
autoimmune hepatitis
resulting inflammation the liver as being attacked by own immune cells
autoimmune hepatitis epidemiology
WOMEN >MEN
autoimmune hepatitis o/e
hepatomegaly, splenomegaly, jaundice
autoimmune hepatitis s/s
asymptomatic Malaise, fatigue, lethargy, nausea, abdo pain, anorexia. Gradual jaundice.
autoimmune hepatitis dx
elevated AST, ALT, IgG
autoimmune hepatitis treatment
corticosteroids - prednisolone
immune suppressant - azathioprine
autoimmune hepatitis histology
Interface Hepatitis w/marked piecemeal necrosis and lobular involvement, plasma cells
autoimmune hepatitis type 1
seen in 80%, usually occurs alongside other autoimmune disease
ASMA and ANA postiive
autoimmune hepatitis type2
children and young adults
more common in europe than in asia
which type of autoimmune hepatitis more commonly progresses to cirrhosis
2
LKM-1 positive in which type of autoimmune hepatitis
2
ASMA and ANA in type 2 and 3 autoimmune hepatitis
negative
anti-SLA in type 3
postive
PSC
disease of the bile ducts causing progressive cholestasis with bile duct inflammation and strictures.
PSC histology
beaded appearance - onion skinning fibrosis as fibrosis of cells around bile duct causes tightening in some areas and dilatation in others.
PSC s/s
jaundice and fibrosis
asymptomatic
itch
malabsorption
PSC associated with
IBD - UC (40%)
PSC dx
ERCP
PSC more common in
MALE
PSC treatment
maintain blood flow transplant
PSC complications
colorectal cancer post transplant
cholangiocarcinoma
describe the malabsorption PSC
due to dec amount of bile reaching the small intestine resulting in steatorrhoea and reduced amounts of at soluble vitamins (ADEK)
what can you develop post transplant
colorectal cancer
Haemochromatosis inheritance and genes
autosomal recessive
due to C282Y or H63D mutations in HFE gene - the HFE gene reduces the expression of DMT1 at the brush border of enterocytes
primary and secondary haemochromatosis
primary - AR condition
secondary - due to iron overload from blood transfusion, iron therapy or diet
Haemochromatosis complications pancreas and liver
PANCREAS
type 1 diabetes and malabsorption
LIVER
iron builds up which creates free radicals which cause cell damage and cirrhosis
Haemochromatosis complications skin
bronzed skin slate grey
Haemochromatosis complications heart
cardiomyopathy - arrhythmias
Haemochromatosis complications gonads
amenorrhoea and testicular atrophy 0 erections
Haemochromatosis investigations
blood test - inc transferring saturation and ferritin production liver MRI
biopsy
ECHO/ECG for cardiomyopathy
Haemochromatosis treatment
predispose to carcinoma
Venesection (phlebotomy)and blood letting.
out come depends on genetics and cofactors (alcohol)
wilsons disease inheritance
recessive
wilsons disease
loss of ceruloplasmin results in excess copper deposited in tissues copper accumulates first in the liver and then other organs e.g. brain
wilsons disease s/s
hepatic seen first - cirrhosis, chronic hepatitis, liver failure
neurology - weird movement, depression, labile emotions
eyes - kaiser-flesher rings
wilsons disease ix
urine - inc free copper
LFT
genetic
MRI
serum ceruloplasmin dec normally but can be falsely high or low
wilsons disease tx
lifelong copper chelating drugs
how do children in wilsons disease usually present
present with liver disease 0 cirrhosis chronic hepatitis or sub fulminant hepatic failure
how do adults with wilsons disease usually present
CNS signs - tremor, dysarthria, dysphagia
copper chelating drug
pencillamine
Budd Chiari classic presentation
young woman on oral contraceptive pill
Budd Chiari
Hepatic vein obstruction by thrombosis or tumour causes congestive ischaemia and hepatocyte damage.
Budd Chiari aetiology
pill
thrombophilia
leukaemia
obstructive cause e.g. tumour
Budd Chiari s/s
acute - jaundice, tender hepatomegaly
chronic - ascites
Budd Chiari ix
high protein ascitic fluid
US, CT, MRI to show occlusion
Budd Chiari tx
recanalisation or TIPS
thrombolysis
treat ascites
a1 - antitrypsin deficiency
excess tryptic activity that commonly affects the lungs and liver
a1 - antitrypsin deficiency effect on lungs
emphysema
a1 - antitrypsin deficiency effect on liver
hepatocellular cancer cirrhosis HCC
a1 - antitrypsin deficiency management
supportive
what is Methotrexate used in
rheumatoid arthritis and psoriasis
what can Methotrexate cause
progressive liver fibrosis, to treat stop the drug