A  26  year  old  female  presents  to the  Emergency  Department  having  been found at home confused and jaundiced by her GP.

Her GCS is E3V5M5

She has a temperature of 38 ˚C, BP 90/60, HR 90 and SpO2  94% on 4 litres/min O2.

Her plasma biochemistry is as follows:

Test

Value

Normal Range

Sodium

137 mmol/L

(135 – 145)

Potassium

4.1 mmol/L

(3.5 – 5.0)

Total bilirubin*

200 micromol/L

(0 – 25)

AST*

4000 U/L

(<40)

GGT*

500 U/L

(<40)

ALT*

3000 U/L

(<40)

Urea

4.2 mmol/L

(4 – 6)

Coagulation profile:

Test

Value

Normal Range

INR*

2.6

(0.8 – 1.2)

a)  Based on the above information, what do you think this woman is suffering from?

b)  List 5 important aetiologies which could result in this presentation.

c)  List 4 important complications (apart from respiratory failure) she is at risk of developing.

d)  List 4 reasons why this woman might progress  to developing  respiratory failure.

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College Answer

a)  Based on the above information, what do you think this woman is suffering from?

Acute liver failure (without more detail it is hard to say, hyperacute, acute, subacute or chronic)

b)  List 5 important aetiologies which could result in this presentation.

•     Sepsis
•    Viral hepatitis – Hep B/C/D, CMV/EBV
•    Drug induced
•     Poisoning
•    Miscellaneous  (wilson’s disease, acute fatty liver of pregnancy, ischaemic necrosis, Budd-Chiari, complications of hepatic surgery)
•     Idiopathic
•    Pregnancy related

c)  List 4 important complications (apart from respiratory failure) she is at risk of developing.

•    Cerebral oedema and herniation
•     Coagulopathy
•    GI bleed
•     Sepsis
•    Renal failure

d)  List 4 reasons why this woman might progress  to developing  respiratory failure.

•    Impaired ventilation because of coma
•    Pleural effusions
•    ARDS
•    Intra-pulmonary shunts
•    Aspiration pneumonia
•    Sepsis- pulmonary or extrapulmonary

Discussion

a)  Based on the above information, what do you think this woman is suffering from?

With the transaminases in their thousands, a raised bilirubin, coagulopathy and fever, one might assume that this lady has some sort of acute hepatitis. As the college rightly points out, "acute hepatic failure" is the only description one can make without any further history.

b)  List 5 important aetiologies which could result in this presentation.

  • Vascular causes, eg. acute hepatic artery thrombosis or ischaemic hepatic injury
  • Infectious causes, eg. acute viral hepatitis or liver abscess
  • Neoplastic causes, eg. decompensation due to hepatocellular carcinoma
  • Drug-induced hepatitis eg. paracetamol overdose
  • Exacerbation of congenital disorder, eg. Wilson's disease
  • Autummune hepatitis, eg. cryptogenic cirrhosis or idiopathic autoimmune hepatitis (IAIH)
  • Traumatic hepatic injury

c)  List 4 important complications (apart from respiratory failure) she is at risk of developing.

There is a brilliant article on this subject which outlines all the possible and impossible complications of acute hepatic failure. I will summarise their Panel 1:

Complications of acute liver failure

  1. Unprotected airway due to obtundation
    Potential for aspiration due to nausea and vomiting
  2. Acute lung injury and ARDS (due to SIRS)
  3. Systemic inflammatory response.
    Vasodilated shock with hyperdynamic circulation
  4. Hyperammonaemic encephaloparthy
    Increased intracranial pressure (cerebral oedema)
  5. Decreased lactate clearance and metabolic acidosis
  6. Renal failure (hepatorenal syndome)
  7. Hypercatabolic state
    Hypoglycaemia
  8. Coagulopathy
    Thrombocytopenia
    Bone marrow suppression
  9. Increased susceptibility to sepsis
    Decreased complement synthesis

d)  List 4 reasons why this woman might progress  to developing  respiratory failure.

This answer calls for differentials of respiratory failure in a semiconscious shocked woman with fever.

  • ARDS due to SIRS
  • Aspiration pneumonia
  • Decreased respiratory drive due to decreased level of consciousness
  • Pleural effusions due to hypoalbuminaemia / ascites
  • Intrapulmonary shunts - the so-called "hepatopulmonary syndrome" (very interesting to read about)

References

Bernal, William, et al. "Acute liver failure." The Lancet 376.9736 (2010): 190-201.

 

Fallon, Michael B., and Gary A. Abrams. "Pulmonary dysfunction in chronic liver disease." Hepatology 32.4 (2000): 859-865.