Cirrhosis and alcoholic liver disease

Question 1

cirrhosis

Answer 1

diffuse hepatic process characterised by fibrosis and the conversion of normal liver architecture into structurally abnormal nodules
final histological pathway for a number of liver pathologies
80-90% of liver needs to be destroyed before clinical signs of liver disease are seen
fibrosis distorts hepatic vasculature which can lead to increased intrahepatic resistance, and portal HTN

Question 2

portal HTN

Answer 2

can lead to oesophageal varices as well as kidney hypoperfusion, water and salt retention and increased cardiac output

Question 3

causes of cirrhosis

Answer 3

common:
alcohol 
hep B
hep C (up to 20%)
NAFLD
NASH (10%)

less common:
haemochromatosis
primary biliary cirrhosis
biliary obstruction
autoimmune hepatitis
wilson's

Question 4

hereditary haemochromatosis

Answer 4

deficiency of the iron regulatory hormone hepcidin
autosomal recessive, variable penetrance
increased intestinal iron absorption leads to accumulation of iron in the tissues, particularly the liver, which may lead to organ damage
also affects the pancreas, joints, heart, skin and gonads

liver fibrosis, cirrhosis and HCC are the most serious complications

Question 5

haemochromatosis presentation

Answer 5

vague, non-specific initial symptoms: fatigue, weakness, arthropathy, abdo problems, ED, heart problems

Sx of advanced disease:
bronzing of the skin
hepatomegaly
arthropathy (esp 2nd & 3rd MCPs)

Question 6

haemochromatosis Tx

Answer 6

phlebotomy to remove 400-500ml blood weekly/bi-weekly

monitor serum ferritin, as raised levels (>1000ug/L) increase the risk of cirrhosis and are an indication for biopsy
if cirrhosis on biopsy, periodic screen for HCC

Question 7

encephalopathy

Answer 7

a spectrum of neuropsychiatric abnormalities in patients with liver failure, after the exclusion of other known brain disease

features include personality changes, intellectual impairment and reduced levels of consciousness

uncertain pathogenesis, but maybe be due to passage of neurotoxins to the brain

hepatic encephalopathy develops in up to 50% of patients with cirrhosis, and is a feature of decompensated cirrhosis

Question 8

covert hepatic encephalopathy

Answer 8

subclinical, less severe form
requires psychometric testing for diagnosis
significantly impacts QoL
ass/w. increased admissions and death

Question 9

common precipitants of hepatic encephalopathy

Answer 9

AKI
electrolyte imbalance
GI bleed
infection
constipation
sedatives
diuretics
high protein intake

Question 10

mild encephalopathy

Answer 10

may have normal memory, language and motor skills, but impairment of attention and decision making

Question 11

moderate encephalopathy

Answer 11

decreased short term memory and concentration
asterixis
fetor hepaticus
hyperventilation
hypothermia

Question 12

Wernicke-korsakoff syndrome

Answer 12

spectrum of disease resulting from thiamine deficiency, usually related to alcohol abuse

wernicke’s: confusion, ataxia, opthalmoplegia

korsakoff’s: late manifestation where wernicke’s encephalopathy has not been adequately treated

Question 13

pathogenesis of wernicke’s

Answer 13

chronic alcohol consumption can lead to thiamine deficiency by causing:
reduced nutritional thiamine
decreased absorption from the gut
impaired thiamine utilisation in the cells
(hence treat with pabrinex to give thiamine)

Question 14

thiamine

Answer 14

also known as vit B1
a cofactor required by enzymes in carbohydrate metabolism
reduced thiamine can interfere with numerous cellular functions
alcohol related neuronal loss has been documented in specific regions of the cerebral cortex, hypothalamus and cerebellum

Question 15

chronic subdural haematoma

Answer 15

can lead to wernicke’s, due to organic atrophy in the frontal and temporal lobes due to long term compression

Question 16

presentation of wernicke’s

Answer 16

alcohol misuse with confusion, N&V, fatigue, weakness or apathy should be considered high risk
unexplained hypotension or hypothermia would also heighten suspicion

Sx:
vision changes, double vision, abnormal eye movements, eyelid drooping
loss of muscle coordination
memory loss
hallucinations

signs:
polyneuropathy
decreased reflexes
gait and coordination abnormal
muscle weakness/atrophy
abnormal eye movements
hypotension
hypothermia
tachycardia
cachectic