Young female,
Hepatits E related ALF.
GCS 3-4/15, fixed pupils
Minimal vent settings
No CV support
Good renal function
Intact brain stem
EEG: diffuse encepahlopathy
CT: cerebral edema
No cerebral herniation
No signs of infection
wrt the young patient with acute liver failure from hepatitis E. Is her liver function worsening or improving? This is critical here as the liver transplant can only be of any possible benefit if her liver function is deteriorating. If her liver function is improving then the liver transplant will not be of any benefit at all. Her neurological state is precarious and that has to be a factor also in the decision making. This is a difficult decision as there is no certainty of a successful outcome.
Sincerely
Deb Verran
Sydney, Australia
Dear Verran,
Thank you very much. Her bilirubin is down from 28 to 18, INR down from 11 to 4. However her ammonia is up from 300 to 500 and lactate is static at 10. GCS not improving and she has focal fits.
There are some reported cases of good response to Ribavirin in doses from 400-800 mg/day in severe acute hepatitis E. Why to not prove this therapy in this urgent indication ?
INR level of 11 is already an indication alone for liver transplantation which do not require any further evaluation.
Fixed pupils, brain edema and mechanical ventilation are poor prognosis factors.
That precisely is the question. Should transplant be performed with such poor prognostic factors?
Have you tried to prove Ribavirin in this patient?. This may be a good non-dangerous option
Aditionally you can use extracorporeal liver support systems such as MARS, Prometheus, plasmapheresis...
Living donor liver transplantation is life saving procedure like these acute stuation. You should explain all risks of procedure and the clinical progress to her parents.
Would you recommend ribavirin with such high bilirubin and coagulopathy since its associated with hyperbilirubinemia hemolysis and thrombocytopenia. Thanks
She is status post spontaneous abortion of a 6 months old fetus. Dr Ince we don't have MARS unfortunately. Trying to arrange a donor.
This lady aborted a fetus due to infection with hepatitis E, which has bad outcomes in pregnancy. However the outcomes improve if the fetus is delivered which is the case with this lucky lady. Further her bilirubin and INR has improved. Since her renal parameters are normal, inj. Mannitol can be given to reduce brain edema and improve her GCS. Ammonia lowering agents can be tried like L-Ornithine-L-Aspartate. However my experience is that such patients take pretty long time for improvement. Also since she is having seizures, a gynaecology consult may be done to rule out two dreaded complication -- eclampsia and acute fatty liver of pregnancy both of which have far poorer outcomes
Thank you Ray. She underwent LDLT finally. POD 1 stable hemodynamically but low GCS despite being off sedation. Let's see how it goes.
Good success!. I hope everything would go very well. My best regards on it.
Excellent. Pray full recovery of your patient. Best regards for ur success.
In any case like this with acute liver failure, whatever the etiology is, you can give IV N-acetylcystiene in the same dose like paracetamol toxicity. It will be very beneficial.
It can save her from transplantation, prevent renal and electrolytes complications and improves brain perfusion.
How is going the evolution of this patient after the liver transplantation?
Post transplant she never shown any improvement in GCS. She was stable hemodynamically and graft function was very good. She was declared brain dead POD 5 after a set of neurological tests, EEGs and CT scan unfortunately.
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