Neonatal hepatitis (NH) is inflammation of the liver during early infancy. In NH, bile does not flow properly from the liver to the intestines. Instead, it accumulates in the liver, causing inflammation and liver damage.
Infants with NH typically develop complications, such as malnutrition and delayed growth. Detecting the cause of NH early on and administering appropriate treatment helps improve outcomes.
This article discusses the causes, symptoms, diagnosis, treatment, and possible complications of NH.
NH is inflammation of the liver in an infant. It is an umbrella term for many types of neonatal liver disease.
According to the National Organization for Rare Disorders (NORD), other terms for NH include:
- neonatal cholestasis (NC)
- neonatal giant cell hepatitis (NGCH)
In NH, there is an impairment in bile production and flow. Bile is a digestive fluid that the liver makes and secretes to help with digestion. This impairment causes bile and other substances to back up in the liver, resulting in liver inflammation and damage.
Learn more about the function of the liver.
Biliary atresia (BA) is the most common identifiable cause of NH, occurring in 0.55 to 1.3 out of every 10,000 live births.
BA involves scarring of the bile ducts inside and outside the liver. This scarring prevents bile from flowing out of the liver and into the intestines, resulting in NH. Damage from BA-induced NH can eventually lead to a severe form of liver scarring or cirrhosis.
BA is the most common reason for liver transplantation in childhood.
Read more about biliary atresia.
Other causes of neonatal hepatitis
Other causes of NH include:
- Infections: Examples include:
- cytomegalovirus (CMV) and herpes viruses
- congenital syphilis
- echovirus
- Metabolic diseases: Examples include:
- cystic fibrosis (CF)
- respiratory chain defects
- fatty acid oxidation defects
- Genetic disorders: Examples include:
- alpha-1-antitrypsin deficiency
- progressive familial intrahepatic cholestasis (PFIC)
- Alagille syndrome
- Rare diseases: Other rare diseases can impair or affect liver function.
If doctors cannot identify the cause of NH, they will diagnose it as idiopathic NH (INH). “Idiopathic” means there is no identifiable cause.
Some infants with NH experience mild and transient disease that improves without treatment, while others experience severe disease along with medical complications.
Common signs and symptoms of liver disease may appear during the first few weeks of life. By 2 to 3 months of age, infants with NH will be noticeably underweight and show signs of delayed growth. Doctors call this “failure to thrive”.
Infants may also experience the following signs and symptoms:
- dark urine
- pale-colored stools
- itchy skin
- irritability
- an enlarged liver
- jaundice, which is yellowing of the skin and the whites of the eyes
According to the Childhood Liver Disease Research Network (CLDRN), infants with INH may experience jaundice as their only symptom. This typically develops during the first 2 weeks of life and resolves by the third month.
Severe cases of NH may cause additional symptoms, such as:
- easy bruising
- prolonged bleeding
- infection, or sepsis
- abdominal swelling due to an accumulation of fluid in the abdomen
To diagnose NH, doctors must first rule out other potential causes of liver disease in infancy. Infants with jaundice beyond their second week of life typically receive blood tests to determine the cause.
Doctors may also recommend an ultrasound scan to look for structural or functional issues with the liver and its surrounding ducts and blood vessels.
In some cases, doctors may request a liver biopsy to help rule out disorders affecting the inside of the liver. A liver biopsy involves using a needle and syringe to remove a small piece of liver tissue and then studying the tissue under a microscope.
If laboratory tests and liver biopsies fail to indicate the cause of NH, doctors will usually recommend genetic testing to look for certain gene mutations associated with liver disease.
As the CLDRN explains, doctors may diagnose INH if liver biopsies show enlarged liver cells, but additional lab tests fail to determine the cause.
Complications of NH may include:
All infants with NH are at risk of malabsorption, which means their bodies have difficulty absorbing nutrients from food. For this reason, they require special attention to their nutrition, vitamin levels, and growth. Doctors may recommend adding fats called medium-chain triglycerides to breast milk or to infant formula, as the intestine can absorb them without needing bile.
Many infants with NH will also require supplements of vitamins D, A, E, or K, along with a multivitamin formula.
The authors of a 2021 review highlight the need for infants with NH to have all their age-appropriate vaccines in their first year of life. A person can discuss the recommended vaccine schedules with the child’s doctor.
Infants likely to require a liver transplant should receive live vaccines at 6 months instead of 1 year because live vaccines are unsafe for people who have received organ transplants. Examples of live vaccines include the varicella vaccine and the measles, mumps, and rubella vaccine.
Doctors may also prescribe a medication called ursodeoxycholic acid (UDCA) to help with the following:
- promoting bile flow
- protecting the liver
- alleviating skin itching or “pruritus”
If UDCA is not effective, doctors may trial one of the following medications:
- rifampicin
- naltrexone
- cholestyramine
- sertraline
In some cases, doctors may also recommend surgery to treat NH. Other treatment options depend on the underlying cause of the condition.
The following groups can support parents and caregivers of infants with NH:
- Alagille Syndrome Alliance: an organization that supports families of people living with Alagille syndrome (AS) and provides access to educational resources about the condition
- Alpha-1 Foundation: an organization that promotes research into finding a cure for Alpha-1 and enables users to search for a local doctor who specializes in Alpha-1
- Children’s Liver Association for Support Services (CLASS): a volunteer group providing educational resources, financial assistance, and emotional support to families living with a child with liver disease or a liver transplant
- Progressive Familial Intrahepatic Cholestasis Advocacy and Resource Network (PFICARN): an organization offering educational materials, online support groups, and financial assistance for those affected by PFIC
The outlook for infants with NH depends on the cause. A
- The success rate of a hepatoportoenterostomy (HPE) procedure to drain bile is around 80% in infants who undergo the procedure before 45 days of age. In infants who have HPE after 90 days, the success rate is just 20%.
- Around 75% to 90% of infants whose jaundice resolves within 3 months of an HPE are still alive 10 years later without needing a liver transplant. For infants whose jaundice persists following an HPE and who do not receive a liver transplant, only 20% are alive 3 years later.
- Around 50% of children with BA require a liver transplant before the age of 2 years, while around 75% require a liver transplant by the age of 20 years.
- Among children who survive into their third decade of life, almost all have cirrhosis and associated complications, such as portal hypertension. This is the medical term for high blood pressure in the major vein that carries blood through the liver.
Other causes of NH have a different outlook. A pediatric gastroenterologist can give advice about a child’s specific diagnosis, treatment, and outlook.
Neonatal hepatitis is inflammation of the liver during early infancy. In NH, bile and other substances accumulate in the liver, leading to inflammation and damage.
Jaundice is a common and early symptom of NH. Other symptoms include dark urine, itchy skin, and delayed growth. Diagnosis may involve blood tests, ultrasound scans, and biopsies.
Treatment for NH involves dietary supplements and medication. In some cases, surgery may also be necessary. The outlook for NH depends on a child’s specific condition.
