Pediatric liver disease- What you need to know

Pediatric liver disease encompasses a range of conditions affecting the liver in children, from infancy through adolescence. The liver plays a vital role in metabolism, digestion, and detoxification, making any disruption in its function a matter of significant concern. There are two types of liver disease. Acute liver failure and Chronic liver failure. Acute liver failure occurs in children with no known prior liver disease. Chronic liver failure occurs when a long-lasting liver disease becomes much worse, either slowly or suddenly.

Causes of Pediatric Liver Disease:





  • Congenital Anomalies: Congenital liver anomalies, such as biliary atresia, Alagille syndrome, and congenital hepatic fibrosis, are among the primary causes of pediatric liver disease. These conditions often manifest early in life.
  • Metabolic Disorders: Inherited metabolic disorders like Wilson's disease, alpha-1 antitrypsin deficiency, and glycogen storage diseases can affect the liver's ability to process and store nutrients properly.
  • Infections: Viral hepatitis (such as Hepatitis B and C), cytomegalovirus, and Epstein-Barr virus can lead to liver inflammation and damage in children.
  • Autoimmune Hepatitis: An autoimmune response can target the liver cells, leading to inflammation and potential damage. This condition is more common in older children and adolescents.
  • Non-alcoholic Fatty Liver Disease (NAFLD): With the rising prevalence of childhood obesity, NAFLD has become a significant concern. It involves the accumulation of fat in the liver and can progress to more severe conditions like non-alcoholic steatohepatitis (NASH).

Symptoms:





Liver cirrhosis often causes no symptoms in the early stages. Symptoms start when there is portal hypertension or when the liver begins to fail, as scar tissue is replaced by healthy cells. Some common symptoms includes

  • Jaundice (yellowing of the skin and eyes)
  • Abdominal pain or swelling
  • Dark urine and Pale-coloured stool
  • Poor appetite and weight loss
  • Bruising or bleeding easily
  • Brain problems, such as confusion, irritability, unusual sleepiness(encephalopathy)
  • Fatigue
  • Itchy skin

Diagnosis

  • Blood Tests: Liver function tests, including measures of enzymes and bilirubin levels, help assess the liver's health and identify abnormalities..
  • Imaging Studies: Ultrasound, CT scans, and MRI are used to visualize the liver's structure and detect any abnormalities.
  • Liver Biopsy: A small sample of liver tissue may be obtained through a biopsy to examine under a microscope, providing information about the extent of damage and the underlying cause.
  • Genetic Testing: In cases of suspected genetic or metabolic disorders, genetic testing can help identify specific mutations contributing to the condition.

Treatment:

  • Medications: Depending on the cause of the liver disease, medications may be prescribed to manage symptoms, reduce inflammation, or address underlying issues. Chronic liver failure requires long-term care by a liver specialist who may prescribe various medications to treat or prevent.
  • Nutritional Support: Liver takes in food directly from the gut and processes it into energy and other proteins. An imbalance in this process can result in too much fat coming into the liver cells. Nutritional interventions are crucial, especially in cases of metabolic disorders. Specialised diets and supplements may be recommended.
  • Surgery: Children with acute liver failure, and children with chronic liver failure, must eventually have a liver transplant to survive. Surgical interventions, such as the Kasai procedure for biliary atresia or liver transplantation in severe cases, may be necessary.
  • Lifestyle Changes: For conditions like NAFLD, lifestyle modifications, including a healthy diet and regular exercise, are fundamental for managing and preventing progression.
  • Ongoing Monitoring: Regular follow-up visits and monitoring are essential to assess the effectiveness of treatment and identify any complications early on. Children with acute liver failure have to be admitted to the hospital for very close observation and support as this condition does get better on its own.

Conclusion:

Pediatric liver disease poses unique challenges due to the diversity of its causes and the age range of affected individuals. Early detection, accurate diagnosis, and timely intervention are crucial for improving outcomes. A multidisciplinary approach involving pediatricians, hepatologists, nutritionists, and other specialists is often necessary to provide comprehensive care for children with liver disease. As research continues to advance, the understanding of these conditions will evolve, potentially leading to improved diagnostic tools and treatment modalities in the future.