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Liver Failure - Fulminant (quick onset) (Clinical presentation (Signs of…
Liver Failure - Fulminant (quick onset)
Need to know
Acute hepatic failure
Acute live injury with encephalopathy and deranged coagulation (INR > 1.5) in a patient with a previously normal liver
Acute-on-chronic hepatic failure
Liver failure as a result of decompensation of chronic liver disease
Hepatic failure occurs when the liver loses the ability to regenerate or repair, so that decompensation occurs
Marked by
Abnormal bleeding
Ascites
Hepatic encephalopathy
Confusion, coma, liver flap (asterisks - flapping tremor with wrist extended) and drowsiness
As the liver fails, nitrogenous waste e.g. ammonia builds up in the circulation and passes to the brain which can result in permanent brain damage as ammonia is neurotoxic
Astrocytes try to clear ammonia - excess glutamine in this process causes osmotic imbalance - cerebral oedema and so damage
Jaundice
Fulminant hepatic failure
Clinical syndrome resulting from massive necrosis of liver cells leading to severe impairment of liver function
Classification
Acute: encephalopathy with 8-28 days of jaundice onset
Subacute: within 5-26 weeks
Hyperacute: encephalopathy within 7 days of jaundice onset
There is a decreasing risk of cerebral oedema as the onset of encephalopathy is increasingly delayed
It is a rare but often life-threatening syndrome that is due to acute hepatitis from many causes
Paracetamol overdose is responsible for 50% of the cases in the UK
Histologically there is multiacinar necrosis involving a substantial part of the liver
Main Causes
Hepatocellular carcinoma
Drugs
Anti-depressant - amitriptyline
NSAIDs
Alcohol
Ecstasy or cocaine
Paracetamol - COMMON CAUSE
Antibiotics - Ciprofloxacin, doxycycline or erythromycin
Wilson's disease or Alpha-1 antitrypsin deficiency
Viruses
Cytomegalovirus
EBV
Hep A, B (and thus D), E but rarely C
Herpes simplex virus
Acute fatty liver of pregnancy
Clinical presentation
Fetor hepaticus - patient smells like pear drops
Cerebral oedema
Signs of hepatic encephalopathy
Grading
II - increasing drowsiness, confusion, slurred speech +/- liver flap, inappropriate behaviour/personality change
III - incoherent, restless, liver flap, stupor
I - altered mood/behaviour, sleep disturbance, dyspraxia
IV - coma
Signs of chronic liver disease suggest acute-on-chronic hepatic failure
Clubbing
Dupuytren's contracture
Bruising
Ascites is RARE
Small liver
Fever, vomiting and hypertension
Jaundiced patient
Neurological examination shows spasticity and hyper-reflexia; plantar responses remain flexor until late
Differential diagnosis
Cerebral infection - bacterial or viral
Drug or alcohol intoxication
Structural/space occupying lesions in the brain
Hypoglycaemia, electrolyte imbalance or hypoxia
Diagnosis
Imaging
Ultrasound will define liver size and may indicate underlying liver pathology
CXR
Electroencephalogram (EEG) useful in grading encephalopathy
Doppler ultrasound to see hepatic vein potency
Microbiology - to rule out infection
Urine culture
Ascitic tap - to check for pathogens
Blood culture
Blood
Low levels of coagulation factors and raising prothrombin time
Low glucose (since liver is glucose store, in form of glycogen)
High serum ALT & AST
Ammonia levels high
Hyperbilirubinaemia
Treatment
Mineral supplements e.g. calcium, potassium, phosphate etc.
Coagulopathy is managed with IV vitamin K, platelets, blood or fresh frozen plasma
Signs of raised intracranial pressure - give IV mannitol
Reduce haemorrhage risk by giving PPI e.g. lanzoprazole to reduce GI bleeds
Monitor glucose levels and administer IV glucose if necessary
Prophylaxis against bacterial and fungal infection
Treat cause e.g. paracetamol poisoning - give N-ACETYL-CYSTEINE
Liver transplant