Liver Failure In Kids: The Causes, Early Signs, And Its Prevention

Table of contents:

• Fatty Liver Disease and its types

• Progression of fatty liver to cirrhosis or liver failure

• Causes of Liver Failure in Children

• Symptoms of Liver Failure in Children

• Diagnosis of Liver Failure and Preventive Measures

• Treatment of Liver Failure in Children

• When to see a Doctor

• Take home points

• FAQs on Liver Failure in Children

Introduction

Liver failure happens when the liver is unable to perform its function. There are a lot of functions of the liver ranging from filtering blood, detoxifying harmful substances, producing bile, storing nutrients, regulating blood sugar levels, and aiding in digestion, among other important functions.

Liver failure is a gradual process, starting with liver inflammation or hepatitis. Over time, chronic inflammation leads to fibrosis or scarring, which affects liver function. As the fibrosis progresses, it can lead to cirrhosis, which severely impairs liver function and can eventually result in liver failure.

Pediatric acute liver failure (PALF) can be defined as:

• Biochemical evidence of acute liver injury in a child with no known evidence of chronic liver disease along with at least one of the following

• INR > 1.5, not corrected with vitamin K supplementation, with encephalopathy

• INR > 2.0, not corrected with vitamin K supplementation, without encephalopathy.

Fatty Liver Disease and its types

Normal liver contains a small amount of fat. When the amount of fat reaches 5-10% of liver weight it is called steatosis (Fatty Liver Disease).

In children, there are two types of Fatty liver disease which can cause complications.

1. Simple fatty liver disease, or nonalcoholic fatty liver(NAFLD): When there is an excess amount of fat present in a child's Liver but no inflammation or damage is seen. It usually doesn’t lead to many complications.

2. Nonalcoholic steatohepatitis (NASH): When fat deposition in the Liver leads to inflammation or damage then it is called Nonalcoholic steatohepatitis (NASH). About 20-25% of children having NAFLD progress to NASH form while others remain as Simple fatty liver disease without any inflammation or damage to the liver.

Progression of fatty liver to cirrhosis or liver failure

Liver failure is a gradual process. Liver failure occurs when end-stage liver disease(cirrhosis) progresses and ultimately every physiological function of the Liver gets deranged. 

The Progression of Fatty Liver Disease to Liver Failure:

1. Fatty Liver Disease: Fat content of the Liver increases, this can be because of obesity, insulin resistance, high levels of triglycerides in the blood, excessive alcohol consumption, certain medications, viral hepatitis, and rapid weight loss. Usually, inflammation is not seen at this stage and if proper measures are taken then the liver can get back to a normal state.

2. Non-alcoholic steatohepatitis (NASH): When fat present in the Liver starts causing inflammation and Liver damage. This happens because the causative factor of fatty Liver Disease is left untreated. If left untreated then this can progress to the next stage, i.e Fibrosis.

3. Fibrosis: Fibrosis is the development of scar tissue. As inflammation progresses it leads to fibrosis of the Liver. The liver becomes stiff and less functional because of Fibrosis.

4. Cirrhosis: Cirrhosis is a late-stage Liver Disease. The liver becomes severely scarred, and liver function is significantly compromised. Cirrhosis is irreversible, but treatment can slow down its progression.

5. Liver failure: Liver failure is the last stage in which the liver completely stops functioning. It is a life-threatening condition.

Causes of Liver Failure in Children

There are several possible causes of liver failure in children, including:

• Inherited metabolic disorders such as alpha-1 antitrypsin deficiency.

• Viral infections such as hepatitis A, B, or C

• Autoimmune diseases such as autoimmune hepatitis or primary sclerosing cholangitis

• Drug or toxin-induced liver injury

• Biliary atresia is a congenital condition in which the bile ducts are absent or blocked.

• Acute fatty liver pregnancy is a rare complication that can occur during pregnancy.

• Reye's syndrome: Reye's syndrome (RS) is a rare disorder that affects all organs of the body but is most harmful to the brain and the liver—causing an acute increase of pressure within the brain and, often, massive accumulations of fat in the liver and other organs. If children with viral infection are given Aspirin, it can also lead to RS.

• Hemochromatosis: A disorder that causes excess iron deposition and, in turn, multiple organ dysfunction. The liver is among one the organs in which the deposition of iron takes place.

• Wilson disease: A rare, autosomal recessive disorder caused by abnormal copper accumulation in the body particularly involving the brain, liver, and cornea.

Symptoms of Liver Failure in Children

As the liver performs many functions in our body, any disease of the liver leads to a variety of symptoms. Liver cirrhosis symptoms itself often cause no symptoms early in the disease process. Symptoms start when there is portal hypertension and/or the liver begins to fail, as scar tissue replaces healthy cells.

The severity of symptoms depends upon the extent of liver damage.

A person with Liver cirrhosis can experience:

• Fatigue

• Weakness

• Abdominal swelling 

• Loss of appetite and weight (include these 4 in early symptoms)

As the disease progress and more scarring occur, the flow of bile is obstructed, and the Person will experience:

• Jaundice (yellowing of skin, sclera and mucous membrane)

• Dark urine, because of bile pigment in urine.

• Bleeds and bruises can occur easily and take longer time to heal

Later symptoms include,

• Reddened palms (palmar erythema)

• Loss of body hair

• Enlarged liver and spleen(hepatosplenomegaly)

• The appearance of thin, purplish-red, spidery-looking blood vessels on the skin, especially around the navel (spider angiomata)

• Water retention and swelling in the legs and abdomen (oedema)

• Vomiting blood(hematemesis) 

• Itching

• Abdominal infections

• Forgetfulness or confusion

• Tremors

• Inability to fully process drugs

• Enlarged, twisted, thin-walled blood vessels called varices in the oesophagus (oesophageal varies) and/or stomach that can rupture and lead to life-threatening bleeding.

Diagnosis of Liver Failure and Preventive Measures

Liver Failure can be difficult to diagnose in the early stages, as other diseases can present with similar symptoms.

Steps taken for diagnosing Liver Failure are:

• Bilirubin levels

• Enzyme levels(specifically ALT, AST and ALP)

• Coagulation profile

• Signs of encephalopathy 

• CT scan or MRI

• Liver biopsy

Treatment of Liver Failure in Children

Treatment of Liver Failure is determined based on whether the onset was acute or chronic. Close collaboration between pediatric hepatologists, intensivists, transplant surgeons, neurologists, nephrologists, and/or metabolic disease specialists is crucial.

• Children with acute liver failure are hospitalized. 

• Treating specific causes.

• IV fluids, and medications to resolve abdominal pain.

• In case of Chronic liver failure dietary and lifestyle changes are required.

• Most children with liver failure need Liver transplant.

Prevention is always better than cure, we can prevent liver failure in children in the following ways:

• Getting vaccinated for hepatitis B

• Consult a Physician before taking OTC (over-the-counter) drugs like paracetamol.

• Screening for metabolic syndromes, autoimmune diseases, Wilson disease and hemochromatosis. 

When to See a Doctor

Liver failure in children is a medical emergency, it needs medical attention and intensive treatment. If your child is experiencing symptoms such as jaundice, abdominal pain, fluid in the abdomen, vomiting, confusion, or difficulty breathing. You should immediately reach a Doctor.

Take home points

• Liver failure happens when the liver is unable to perform its function. Liver failure is a gradual process, starting with liver inflammation or hepatitis.

• Liver failure occurs when end-stage liver disease(cirrhosis) progresses and ultimately every physiological function of the Liver gets deranged

• Symptoms of liver failure include Fatigue, Weakness, Abdominal swelling, Loss of appetite and weight, Jaundice, Dark urine, Bleeds and bruises can occur easily and takes a longer time to heal.

• The progression of liver disease happens from fatty liver disease to nonalcoholic steatohepatitis to cirrhosis to liver failure.

• Causes of liver failure in children could be congenital disorders like hemochromatosis, and Wilson disease or it could be metabolic, autoimmune or acquired as in the case of drug toxicity (paracetamol poisoning).

• Treatment of Liver failure in Children usually needs emergency medical attention and hospital admission.

References:

• Acute Liver Failure in Children

• Management of Acute Liver Failure: A Pediatric Perspective

• Aspirin and Reye Syndrome