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Diseases

Acute-on-Chronic Liver Failure

When an individual with a pre-existing liver disease develops progressive jaundice, abdominal bloating and leg swelling followed quickly by bleeding or altered behavior, more often leading to kidney problem, then is called acute-on-chronic liver failure.

This distinct condition can happen in those with permanently scarred/cirrhotic liver or just with some scarring/fibrosis but not yet cirrhosis in the liver.

Traditionally, cirrhosis is either compensated or decompensated. Structurally when liver is cirrhotic but function is preserved, it is called compensated cirrhosis. When function starts gradually worsening due to progression of cirrhosis and destruction of parenchyma, then it’s called decompensated cirrhosis manifesting as jaundice, abdominal swelling and other complications of end stage liver disease. About 15% of patients with compensated cirrhosis develop clinical decompensation each year and about 35% will progress to another organ or multiple organ failure. Once cirrhosis transitions from compensated to decompensated stage, it is associated with approx. 2 years survival and evaluation for liver transplant is recommended when no other serious ailment is present and the overall general condition of the patient is fair.

The transition to decompensated cirrhosis is recognized by the presence of the following hallmark symptoms:

  • Abdominal swelling (ascites)
  • Swollen blood vessels in the upper gut causing bleeding (variceal hemorrhage)
  • Changes in mental disposition, disorientation, confusion, coma (hepatic encephalopathy)

When a compensated cirrhotic or non-cirrhotic with ongoing fibrosis, develops acute decompensation secondary to hepatic insult, it results in multiorgan failure commonly being the kidney. This distinct entity is called acute-on-chronic liver failure (ACLF). It is important to identify these patients in early stage before the onset of organ failure, because of potential of reversibility to previous compensated stage. Secondly, it is important to start intensive care management at the earliest and also list them for liver transplantation if needed, before they become too sick to transplant.   About 25-40% of patients admitted to any liver unit with a diagnosis of cirrhosis can be estimated to have ACLF.

Based on this, the World Gastroenterology Organization (WGO) proposed a classification of ACLF into three main groups:

  • Type A ACLF were those patients with underlying non-cirrhotic chronic liver disease who may present with signs and symptoms of acute or subacute liver failure, and viral hepatitis as the likely cause, especially in Asia and eastern countries where it is more prevalent
  • Type B ACLF were those patients with previous compensated cirrhosis
  • Type C ACLF were those patients with previous decompensated cirrhosis

As mentioned previously, if the cirrhosis is caused by a treatable cause (by chronic viral hepatitis, ongoing increased alcohol consumption, obesity and so on), then patients may have a transition from a decompensated to a compensated phase, which might not even need a liver transplant.  This is attributed to treating the cause and the unique power of regeneration of the liver. Hence ACLF is considered as a dynamic syndrome, ongoing process that may improve or worsen during hospitalization. So liver specialists use a scoring system to evaluate, treat, and monitor each case diagnosed with ACLF.

Risk Factors

Underlying chronic liver disease include:

  • Viral hepatitis B/C
  • Fatty liver disease, alcoholic, non-alcoholic [the more serious form called non-alcoholic steatohepatitis (NASH)]
  • Autoimmune hepatitis

Acute insults

  • Acute Hepatitis A/E/B
  • Alcohol binge
  • Bacterial Infections
  • Flare of Wilsons disease

In approximately 20%–45% of cases, the trigger remains unknown

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    Investigations

    Routine blood and liver tests

    • Prothrombin time (PT) test measures how long it takes for blood to clot. The PT is prolonged
    • International normalized ratio (INR), a calculation based on the PT test, is greater than 1.5
    • ACLF is mainly due to systemic inflammation and the inflammatory markers are increased:
    • White cell count
    • Plasma levels of C reactive protein (CRP)
    • Pro-inflammatory molecules such as interleukin (IL)-6, IL-1β, IL-8
    • Liver enzymes are elevated: Aspartate aminotransferase (AST)/serum glutamic-oxaloacetic transaminase (SGOT), alanine aminotransferase (ALT)/serum glutamic-pyruvic transaminase (SGPT), alkaline phosphatase (ALP)
    • Bilirubin is elevated
    • Test for the presence of hepatitis virus infections
    • Ammonia level is increased

     Imaging tests

    • Ultrasound may show features of underlying chronic liver disease
    • Computerized tomography (CT) scanning or magnetic resonance imaging (MRI) can further throw light on the chronicity of liver disease, cirrhosis, any associated portal vein thrombosis and so on.

    Treatment

    The treatment is based on organ support and treatment of associated complications.

    Medical Management

    The goal is to look for clues to the precipitating factors for ACLF:

    • If associated bacterial infections, gastrointestinal tract bleeding, alcoholism, and drug toxicity may be the likely causes, early identification and treatment of the trigger factor is essential
    • Patients with ACLF deteriorate rapidly and should be considered to be admitted to the ICU and should be preferably managed in a transplant center
    • Monitoring of all organ function should be done frequently and early treatment provided according to each specific organ in order to avoid a stage of multiple organ failure
    • Liver specialists use latest guidelines to manage such critically ill ACLF patients
    • Prevention of sepsis, management of organ failure, and early referral for transplant is crucial
    • Aggressive multidisciplinary approach can improve survival

    Surgical Management

    • Liver transplant surgery is a definitive way to treat ACLF. Assessment of the overall condition of the patient must be carried out to evaluate if liver transplant surgery can be carried out. The timing of transplantation is crucial in patients with ACLF, as these patients may provide a short window of opportunity because of the likely risk for developing multiorgan failure, which then becomes a contraindication for the liver transplant.
    • Liver support devices can be useful in patients with ACLF while awaiting a liver transplant. Death occurs in nearly half the patients with ACLF awaiting a liver transplant.

    Prevention of ACLF is as important as ACLF Management

    • Medical and paramedical professionals need to create awareness for the prevention strategy of viral hepatitis by timely vaccination
    • Identification and early treatment of fatty liver diseases: cessation of alcohol in alcoholic fatty liver disease, and preventive measures from NASH in non-alcoholic fatty liver disease