Pediatric Liver Disease

Overview

The liver is situated in the upper right quadrant of the abdominal cavity, beneath the diaphragm and on top of the stomach, right kidney, and intestines. It comprises two primary lobes, each of which has eight segments. A thousand lobules make up the segments.

The lobules are linked to tiny ducts, which join to bigger ducts to create the common hepatic duct. The bile generated by the liver cells is transported to the gallbladder and duodenum via the common hepatic duct (the first part of the small intestine).

Liver disease is defined as any injury or condition that restricts or impairs the function of the liver. It can be inherited or acquired (something that develops because of infection, unhealthy behaviors or habits, or exposure to certain toxins).

Children and adults are affected by over 100 different forms of liver disease. In reality, at least 30 million people in the United States have been diagnosed with some sort of liver disease.

 

Common Liver Disease Symptoms

See a doctor if your child develops any of the following symptoms, which could point to liver disease:

• Jaundice: Yellow coloring of the skin and whites of the eyes
• Abdominal pain or bloating
• Insomnia.
• Grey or pale-colored stools
• Blood in the stools or urine
• Loss of appetite
• Nausea
• Poor weight gain
• Portal hypertension and varices
• Pruritus (itching)
• Fatigue or loss of stamina
• Vomiting
• Vomiting of blood
• Yellow urine

After ingesting some chemicals, plants, or even some medications, a child's liver might become irritated. It can also occur as a result of a more chronic illness or abnormality, such as hepatitis or biliary atresia.

 

Common liver disease in Children

Acute liver Failure:

• What is Acute Liver Failure?

Acute liver failure is an uncommon and life-threatening condition. It generally happens all of a sudden and may be rather overpowering. You may always get help and support from the nurses in the hospital or through CLDF's Families Team. Because the early symptoms are typically identical to those of common children ailments, families and healthcare providers may miss it at first.

 

• What can cause acute liver failure?

Some possible causes include:

• Infections
• Metabolic diseases
• Autoimmune liver disease
• Blood disorders
• Medication (including alternative medicines)
• Paracetamol overdose (often accidental)
• Recreational drugs, especially ecstasy
• Poisonous substances
• Complications caused by another illness
• In many cases it is not possible to find a specific cause.

 

• What are the signs and symptoms of acute liver failure?

The signs and symptoms can vary but often include:

• General symptoms of feeling unwell.
• Abdominal pain.
• Nausea and vomiting.
• Weight loss.
• Jaundice.
• Poor blood clotting (coagulopathy) which may cause bruising and bleeding.
• Low blood sugar levels (hypoglycaemia).
• Fluid and salt imbalance including swelling e.g. abdominal swelling and lower leg swelling.
• Encephalopathy (changes within the brain’s functions) can cause symptoms ranging from a loss of concentration and irritability to drowsiness.

 

• What tests may be needed?

The tests will be different for each kid, and the clinical staff will go over them with you: 

• Blood tests: including liver function testing and blood clotting assays, may be performed. Your child will most likely need a series of blood tests over the course of many days to weeks. This is the most effective method of determining whether the liver is improving or deteriorating and arranging the next therapy.
• Urine tests.
• X-rays / scans.
• A liver biopsy: This may be a transjugular biopsy, which is safer for children whose blood isn't clotting properly. A portion of liver is extracted through a vein in the child's neck. In some cases skin and muscle biopsies are required.

 

• When is liver transplantation needed for children with acute liver failure?

Some children recover completely without the need for a liver transplant. Despite considerable injury, the liver has the potential to heal fast, and complete recovery is occasionally achievable. If your child's liver cannot heal on its own and physicians believe that a liver transplant is the best choice, he or she will be referred to the transplant coordinator.

They will register your child and offer you with further information on the transplant procedure. Supportive care and therapy will be provided indefinitely. Your child will have to wait for a matching liver in terms of blood group and size. Priority is given to children suffering from severe liver failure. Most children who receive a liver transplant will recover completely, and while they may require lifelong medication, the vast majority will be able to live a normal life.

 

Alagille Syndrome:

• What is Alagille syndrome?

Alagille syndrome is an uncommon genetic disorder. It can harm the liver, heart, kidneys, eyes, face, and bones, among other organs. One in every 30,000 live births is affected by Alagille syndrome.

 

• What causes Alagille syndrome?

Alagille is a hereditary condition. Genes are constructed from DNA and serve as instructions for the body. Different characteristics, such as hair and eye color, are determined by genes. They also regulate how various sections of our bodies develop and operate. A mutation (change) in a gene called JAG1 affects more than nine out of 10 patients with Alagille syndrome.

 

• How is Alagille syndrome diagnosed?

If your child has some of the features of Alagille syndrome there are a number of tests which can be carried out to find out if Alagille syndrome is the cause.

These tests include:

• Liver biopsy – to see whether there are fewer bile ducts than normal
• Blood tests – to check whether the liver is working properly
• Heart tests – to check for any heart abnormality or murmur
• Ultrasound of the abdomen
• Eye examination – to check for eye abnormalities
• X-ray – to look for changes in the bones of the spine

Diagnosis might be challenging in newborns since the disorder can resemble other types of liver disease, such as biliary atresia.

The mutation that causes Alagille syndrome is known as'sporadic' in around six out of ten patients. This signifies that the gene was not passed down from parents and is present for unknown reasons. The gene that causes Alagille syndrome is handed down from parent to kid in four out of ten instances.

 

• How is Alagille syndrome treated?

There is no cure for Alagille syndrome, although there are therapies that help alleviate the symptoms. The primary therapies address the liver issues that arise as a result of the reduced number of bile ducts in and around the liver. The following are some of the most frequent approaches to treat Alagille syndrome. They include taking vitamin supplements and drugs, evaluating diet, and, in rare situations, undergoing a liver transplant or other operations.

 

alpha-1 antitrypsin deficiency

• What is alpha-1 antitrypsin deficiency?

In the liver, people with alpha-1 antitrypsin deficiency create a slightly different version of alpha-1 antitrypsin protein. This aberrant alpha-1 antitrypsin accumulates in the liver. This implies that it is not transferred to other regions of the body, therefore people normally have low levels of alpha-1 antitrypsin in their blood, but this is not always the case. 

This can result in lung and liver damage. One out of every 10 children with alpha-1 antitrypsin deficiency has liver issues. Many persons with the syndrome will develop liver scarring, although the vast majority will not develop liver disease.

 

• What are the signs and symptoms of alpha-1 antitrypsin deficiency?

1. Failure to gain weight or weight loss.
2. Constant tiredness or lethargy.
3. Deepening or reappearance of jaundice.
4. A swollen abdomen. This may be due to an enlarged spleen or an abnormal collection of fluid. Enlargement of the liver and the spleen can also be signs of alpha-1. Sometimes the baby’s tummy may be obviously bigger than usual or it may only be noticed when a doctor examines your baby.
5. Recurrent nose bleeds.
6. Vomiting blood.
7. Blood in the stool. This may be black or red.
8. Swollen ankles, especially in the evenings.

 

• Are there any cures for alpha-1 antitrypsin deficiency?

There is no particular therapy or cure for alpha-1 antitrypsin insufficiency at the time. Regardless, the medical personnel caring for your kid will monitor and manage any symptoms that arise as a result of it. It may be important to alter your child's diet. A dietician will handle this. Here are a few things to keep in mind:

• Babies often do better with a specific milk formula than with regular milk or breast milk alone. The particular milks are easier to absorb. A specialized dietician can advise you on how to increase your baby's calorie intake by adding extra milk feeds to their diet.
• Older children may require additional calories, which can be provided in the form of high-calorie beverages.
• Dietary changes are frequently required since newborns and children with damaged livers produce less bile. As a result, they frequently do not digest all of the food they consume. They may lose weight or struggle to acquire weight.
• Extra vitamins, particularly vitamin K, may be given to children to help their blood clot normally.

 

Autoimmune liver diseases 

• What are Autoimmune liver diseases?

They are a set of disorders in which the body's immune system targets organ systems. Rheumatoid arthritis and inflammatory bowel disease are two examples of autoimmune disorders. Normally, the immune system detects and eliminates germs and viruses from the body. Autoimmune disorders are caused by the immune system producing specialized proteins called autoantibodies that target a certain organ and cause autoimmune disease. It is unknown what causes the immune system to operate in this manner. It is suggested that there might be a variety of causes, including:

• A problem with the immune system.
• An individual’s genes.
• Environmental factors.

 

• What are the symptoms of autoimmune liver disease?

The most common symptoms are:

• Tiredness and generally feeling unwell.
• Loss of appetite.

Other symptoms are:

• Nausea (feeling sick) or being sick.
• Abdominal pain.
• Jaundice with dark urine and pale stools (poo).
• Muscle pain.
• Weight loss.
• Itching.
• Fever.
• Nose bleeds, bleeding gums, and bruising easily.
• Amenorrhoea (delayed starting of periods or they stop once started)
• Diarrhoea (bowel symptoms are more common in autoimmune sclerosing cholangitis)

Later symptoms which can appear as the condition progresses include:

• A swollen abdomen (ascites).
• Swelling, especially in the lower legs (oedema).
• Irritability and confusion.

 

• How is autoimmune liver disease diagnosed?

If a child or young person is displaying the symptoms of autoimmune liver disease, there are a number of tests which are used to confirm a diagnosis:

• Blood tests.
• Liver biopsy.
• Ultrasound scan.
• MRI-MRCP scan.
• Upper and lower gastrointestinal endoscopy if there are bowel symptoms.

• How is autoimmune liver disease treated?

Prednisolone:

Prednisolone is a steroid that is commonly used as the first therapy. 

When therapy begins, high dosages of steroids are necessary. Depending on the results of the blood tests and the severity of the symptoms, the dose will be gradually lowered. The general goal of treatment is to use as little medicine as possible to address the condition.

Side effects of steroids include:

• Being more likely to pick up an infection
• Increased appetite and weight gain
• Acne
• A risk of bone weakness
• Behavioural changes
• Slower growth
• Diabetes in some individuals

 

Azathioprine

Azathioprine is another medication that can be used to assist the steroid work or when the steroid has major negative effects. It, like prednisolone, is a drug that suppresses the immune system's reaction.

If prednisolone and azathioprine do not effectively treat the condition, further medications are available. If these alternatives are required, your medical team will discuss them with you.

 

Other medications

Ursodeoxycholic acid (also known as urso) is another medication that is commonly administered to children with ASC. The goal is to increase bile flow through the bile ducts. If colitis (inflammation of the large intestine) is present, many medications, including mesalazine, may be administered. If these are required, your healthcare team will discuss them with you.

 

• How is autoimmune liver disease monitored?

Those with autoimmune hepatitis or autoimmune sclerosing cholangitis require regular blood tests. Monitoring the condition is really important. Regular blood tests are taken to measure AST (aspartate aminotransferase) and ALT (alanine aminotransferase). These are enzymes which are normally present in the liver and their levels can be used to monitor the health of the liver. When first diagnosed, testing may be done weekly to see how quickly the level of steroids can be reduced. As the condition stabilises, blood tests will be needed less often (every 3-6 months).

 

Cystic Fibrosis and Liver Disease

• What happens to the liver in cystic fibrosis?

The small bile ducts are where the liver's damage begins. The bile generated by the liver cells into these ducts becomes stickier than normal, resulting in duct obstruction (this very occasionally causes jaundice). The surrounding liver tissue is eventually injured and scarred, a condition known as biliary fibrosis. This is similar to the underlying mechanism that occurs in the lungs. Fibrosis increases over time and finally spreads throughout the liver. This causes the liver to harden and blood flow through it to become more difficult.

 

• How is a diagnosis made?

Most children and young people with cystic fibrosis have regular check-ups which include:

Physical examination: Signs of liver disease may be found during a routine examination. These include:

1. Enlargement of the liver (hepatomegaly).
2. Enlargement of the spleen (splenomegaly) or both spleen and liver (hepatosplenomegaly).
3. This is rare and usually only seen when liver disease is very advanced.
4. Visible veins on the abdominal wall which suggests poor blood flow through the liver (portal hypertension).

 

Blood tests: can be useful in monitoring liver function, although they may stay normal despite the advancement of liver disease. Learn more about liver exams.

 

Abdominal ultrasonography: it reveals the size and texture of the liver, the blood flow through it, and any evident abnormalities of the bile ducts, such as enlargement and the presence of stones or obstructions. It also allows for precise measurement of the spleen and gallbladder. If a liver disease is detected after the foregoing tests, a liver biopsy may be indicated. This may aid in determining the amount of the liver's damage.

 

• What are the main effects of liver disease in children with cystic fibrosis?

1. Portal hypertension (increased blood vessel pressure caused by scarring of the liver).
2. Enlarged spleen
3. Disturbance of the normal working of the liver. This may include poor growth, reduced absorption of dietary fat soluble vitamins A, D, E and K, ascites, and an increased tendency to bruise or bleed

 

Gilbert’s syndrome

• What causes Gilbert’s syndrome?

When both parents pass on a defective gene, the ailment is inherited. In reality, the gene responsible for Gilbert's syndrome is widespread in the UK population (affecting about 4 in every 100 people). Many people have no symptoms of the illness. Parents are frequently infected with the illness but may not exhibit any symptoms of jaundice. The affected gene is known as UGT1A1.

In Gilbert's syndrome, a small mutation in the gene causes less of the enzyme (protein) that usually removes bilirubin from the body to be produced. This causes a rise in bilirubin levels in the blood. It is then detected in the skin and the whites of the eyes, resulting in a faint yellow discoloration known as jaundice. The illness appears to afflict boys more frequently than girls and is frequently identified in adolescence.

 

• What are the symptoms of Gilbert’s syndrome?

Occasional jaundice is the most common symptom. Sometimes this may be accompanied by other symptoms such as:

• Vague abdominal discomfort
• Generally feeling tired. This may be made worse by infection, over-exertion, stress, or periods of dieting/fasting
• There may have been jaundice as a baby
• There may be other members of the family with Gilbert’s syndrome
• Episodes of jaundice can be triggered by:
• Being dehydrated
• Fasting
• Not getting enough sleep
• Having an infection
• Being stressed
• Physical exertion
• Having surgery
• Menstruation (periods)

 

• How is Gilbert’s syndrome diagnosed?

1. Using a complete medical history, including any family history of liver illness.
2. Clinical evaluation and testing (including blood tests). In most situations, Gilbert's syndrome would be diagnosed if liver function tests were normal except for a greater unconjugated bilirubin level than normal.
3. If the diagnosis of Gilbert's syndrome is not obvious, the gene can be examined.

 

Hepatitis B

• What is Hepatitis B?

The term hepatitis refers to liver inflammation. The liver is a vital organ in the body that performs several functions. If the liver becomes inflamed or injured, it may not function correctly.

 

• How did my child get hepatitis B?

Hepatitis B is transmitted when blood from an infected individual enters the body of another. Even if a person is asymptomatic, the virus can be transmitted.

It cannot be transmitted by casual touch, such as sharing utensils or drinks, snuggling, hand holding, or breastfeeding (unless nipples are cracked or bleeding). Hepatitis B cannot be transmitted by casual kissing, although there is a danger if the infected person's lips has cold sores, ulcers, bleeding gums, wounds, or abrasions.

Hepatitis B can be passed on from mother to child or from person to person.

 

• How can I prevent my child passing the infection to other people?

Ensure your child knows what they need to do:

• Apply a waterproof dressing to any cuts or wounds.
• If you've cut yourself or had a nosebleed, don't let anyone touch your blood.
• Do not share your toothbrushes with anybody else.
• You should clean up any blood spills you may have.
• Share no razors or hair clippers.
• Do not share any drug-injecting, snorting, or smoking equipment, such as needles, syringes, spoons, water, filters, straws, notes, or crack pipes.

 

• What treatment is available?

There is currently no treatment for hepatitis B, although more research is being conducted. Treatment aims to manage the hepatitis B virus by lowering viral levels in the blood and increasing immune system response. This reduces the chance of the infection spreading and protects the liver cells.

 

Hepatitis C

• What is Hepatitis C?

There are several strains of the hepatitis C virus. Each group is referred to as a genotype. There are subgroups within each category. It's critical to understand your genotype since various genotypes respond differently to treatments. The following genotypes are more frequent in the United Kingdom:

• Genotype 1 (1a, 1b).
• Genotype 2 (2a, 2b).
• Genotype 3 (3a, 3b).

 

• Treatment for Hepatitis C

Over the last few years, there have been numerous advancements in hepatitis C treatment. Many patients today have a treatable and curable condition. Some of the most recent medicines are not yet accessible in children, although it is hoped that they will be very soon. Severe liver damage is uncommon in children and adolescents, but it can become a concern when an individual enters adulthood. Some people may be able to spontaneously eliminate the virus, although this is less probable in children who were infected at birth or when they were very young.

 

Conclusion

Your liver is your body's biggest organ. It aids in the digestion of food, the storage of energy, and the removal of toxins from the body.There are many kinds of liver diseases:

• Diseases caused by viruses, such as hepatitis A, hepatitis B, and hepatitis C
• Diseases caused by drugs, poisons . Examples include fatty liver disease and cirrhosis.
• Inherited diseases, alpha-1 antitrypsin

Swelling of the belly and legs, bruising easily, changes in the color of your feces and urine, and jaundice, or yellowing of the skin and eyes, are all symptoms of liver disease. There are occasions when there are no symptoms. Imaging scans and liver function tests can detect liver damage and aid in the diagnosis of liver illnesses.