Fulminant Hepatic Failure

A middle-aged gentleman with no past history of liver disease presented to Aster Hospital, Mankhool with a three-day history of fever, myalgia, nausea and high colored urine. He was detected to have an abnormal LFT and was referred to our hospital for further evaluation. There was no history of alcohol intake or medication use. He had returned from Georgia about 10 days back. At admission patient was fully conscious, afebrile and hemodynamically stable. He had mild icterus. Abdomen was soft and non-tender. There was no organomegaly. Examination of the chest, CVS and CNS was normal. Initial investigations showed conjugated hyperbilirubinemia, markedly elevated AST and ALT (5000 IU/ml) and deranged PT/INR. USG abdomen showed a normal liver with no biliary radicle dilation and patent hepatic and portal veins. A diagnosis of acute hepatitis was made and patient was investigated for hepatitis viruses (hepatitis A, B, E) and other infections such as Malaria, Enteric fever, and Rickettsia. Serological markers for all these etiologies were negative. Patient had a rapid deterioration after the first 24 hours. He developed mental confusion, tachypnea, tachycardia, hypotension, metabolic acidosis with elevated serum lactate, and hypoglycemia. There was worsening of liver function tests and INR. These were all features of fulminant hepatic failure. Patient was aggressively managed with N-acetylcysteine (NAC) infusion, broad-spectrum antibiotics, vasopressors, concentrated dextrose infusion and measures were taken to reduce the intracranial pressure in the form of elective intubation, mechanical ventilation, IV Mannitol and 3% normal saline. Invasive hemodynamic monitoring was done using arterial and central lines. Renal replacement therapy was considered in view of worsening metabolic acidosis and renal insufficiency. Since the patient satisfied criteria for fulminant hepatic failure, he was a candidate for urgent Liver transplant. Since the patient was extremely critical and unstable, meticulous planning was done for a safe transfer and patient was shifted to Cleveland Clinic Abu Dhabi. Plan was to stabilize and then airlift the patient to a transplant center in the UK.

Fulminant Hepatic failure (FHF) or Acute Liver Failure (ALF) is a life-threatening multisystem illness resulting from severe hepatic injury due to varied causes. Paracetamol overdose is the most common cause in the developed world. In Asia and middle east ALF is usually is related to viral etiologies (Hepatitis A, B and E), seronegative hepatitis, dengue virus and variety of prescription and herbal drugs. Upto 40% of cases may not have a defined etiology. Autoimmune liver disease and rarely hepatic venous outflow obstruction can also present as FHF. Early recognition of severe hepatitis and early signs of liver failure should prompt opinion from an expert in liver disease and urgent transfer of the patient to a specialized ICU and transplant center should be considered. Patients with FHF can deteriorate rapidly over 24-48 hours and become hemodynamically unstable, develop raised intracranial pressure due to cerebral edema and go into multi-organ failure. While urgent liver transplantation is life saving in such patients, the role of good intensive care cannot be underscored. Intensive care plays a pivotal role by providing organ support thereby buying time for optimization prior to liver transplantation or hepatic regeneration leading to recovery. The non-transplant survival in FHF has improved considerably with advances in intensive care and even in the absence of transplant, upto 40% patients can recover with good intensive care.

Management of FHF should always be done in a multidisciplinary manner with intensivists specifically trained in Liver Critical care, Hepatologists, and Transplant Surgeons. The critical care team and intensive care unit at Aster Hospital Mankhool is geared to take care of patients with Fulminant Hepatic Failure and other forms of advanced liver diseases and works in coordination with the Aster Integrated Liver Care and Transplant Program based at Aster Medcity Kochi and Aster CMI Hospital, Bangalore.

Take home points:

  1. Suspect severe acute hepatitis in any patient with a short history and serum bilirubin > 10 mg/dl and AST and ALT > 2000. Ideally all such patients should be hospitalized and opinion should be taken from a Hepatologist.
  2. Prothrombin time and INR should be monitored daily in patients with severe hepatitis and any value of INR more than 1.5 should be taken seriously. Prothrombin time and INR in liver failure do not get corrected with Vitamin K. FFP should also not be used to correct deranged coagulation in these patients.
  3. Patient should be monitored for development of early signs of raised intracranial pressure such as headache, vomiting, and somnolence. These signs together with an INR>1.5 are an indicator of development of FHF. Such patients should be transferred to an ICU and urgent counseling for liver transplantation and shifting to a transplant center should be considered.
  4. Transfer between centers either by road or air is extremely critical. Specific measures should be taken to prevent episodes of raised ICP or hemodynamic instability during transfer. Expert critical care personnel should monitor the transfer process.

 

Dr. Kaiser Raja

Consultant in Hepatology and Liver Transplantation 

Dr. Alai Taggu

Specialist Physician & HOD, Critical Care Medicine

Dr. Amal Premchandra Upadhyay

Consultant Gastroenterologist

Aster Hospital, Mankhool