Cholestasis of the newborn

Cholestasis is a violation of excretion of bilirubin, which leads to an increase in the level of direct bilirubin and jaundice. There are many causes of cholestasis, which are revealed in laboratory studies, liver and biliary tract scanning, and sometimes liver biopsy and surgery. Treatment of cholestasis depends on the cause.

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Causes of newborn cholestasis

Cholestasis can occur as a result of extrahepatic or intrahepatic disorders or when combined. The most frequent extrahepatic cause is atresia of bile ducts. A large number of intrahepatic disorders is known, which are united by the collective term "neonatal hepatitis syndrome".

Atresia of the bile ducts is an obstruction of the bile ducts due to progressive sclerosis of extrahepatic bile ducts. In most cases, atresia of the bile ducts develops several weeks after birth, probably after the inflammatory process and the cicatricial changes of extrahepatic (and sometimes intrahepatic) bile ducts. This condition is rare in preterm infants or in children immediately after birth. The cause of the inflammatory response is unknown, but it is believed that there are infectious causes.

The syndrome of neonatal hepatitis (giant cell hepatitis) is an inflammatory process in the liver of a newborn. A large number of metabolic, infectious and genetic causes are known; in some cases the disease is idiopathic. Metabolic diseases include alpha1 antitrypsin deficiency, cystic fibrosis, neonatal hemochromatosis, respiratory chain defects and fatty acid oxidation. Infectious causes include congenital syphilis, ECHO viruses, certain herpesviruses (herpes simplex virus, cytomegalovirus); hepatitis viruses are less likely to cause. Less common genetic defects, such as Allagil's syndrome and progressive familial intrahepatic cholestasis, are also known.

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Pathophysiology of newborn cholestasis

With cholestasis, the primary cause is insufficient excretion of bilirubin, which leads to an increase in conjugated bilirubin in the blood and a decrease in bile acids in the digestive tract. As a result of low bile acid content, the malabsorption of fats and fat-soluble vitamins (A, D, E, K) develops in the digestive tract, which leads to hypovitaminosis, malnutrition, and growth retardation.

Symptoms of newborn cholestasis

Symptoms of cholestasis are detected during the first two weeks of life. In children, jaundice is observed and often dark urine (conjugated bilirubin), achiolic stool, hepatomegaly. If cholestasis continues, itching constantly develops, as well as symptoms of deficiency of fat-soluble vitamins; the growth curve may decrease. If the causative disease leads to the development of fibrosis and cirrhosis of the liver, portal hypertension can develop with subsequent ascites and gastrointestinal bleeding from the varicose veins of the esophagus.

Diagnosis of newborn cholestasis

Any child with jaundice after two weeks of life should be screened for cholestasis with measurement of total and direct bilirubin, hepatic enzymes and other functional hepatic tests, including albumin, RT and PTT levels. Cholestasis is detected by increasing total and direct bilirubin; when the diagnosis of cholestasis is confirmed, further research is needed to determine its cause. This examination includes tests to identify infectious agents (eg, toxoplasmosis, rubella, cytomegalovirus, herpesvirus, IM, hepatitis B and C viruses) and metabolic disorders, including urine tests for organic acids, blood sera for amino acids, alpha1 antitrypsin, blood test for diagnosis cystic fibrosis, urine for the determination of reducing substances, tests for galactosemia. Liver scanning should also be done; the removal of contrast in the intestine excludes atresia of the bile ducts, however, insufficient excretion can be observed both in the atresia of the bile ducts and in severe hepatitis of newborns. Ultrasound of the abdominal cavity can help in assessing the size of the liver and visualization of the gallbladder and common bile duct, but these data are nonspecific.

If a diagnosis has not been made, a liver biopsy is usually performed relatively early. For patients with atresia of the bile ducts, a typical increase in portal triads, proliferation of bile ducts, increased fibrosis. Hepatitis of a newborn is characterized by a disruption in the structure of lobules with multinucleated giant cells. Sometimes the diagnosis remains unclear, and then surgical intervention with operational cholangiography is required.

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Treatment of newborn cholestasis

The initial treatment for newborn cholestasis is conservative and consists of providing adequate nutrition with the introduction of vitamins A, D, E, and K. In children with artificial feeding, mixtures with a high content of medium chain triglycerides should be used, since they are better absorbed in conditions of lack of bile acids. Requires the introduction of a sufficient number of calories; children may need more than 130 kcal / (kg x day). In children who have a small amount of bile secretion, the administration of ursodeoxycholic acid at a dose of 10-15 mg / kg once or twice a day can reduce itching.

There is no special treatment for hepatitis of newborns. Children with presumed atresia of bile ducts require a surgical examination with intraoperative cholangiography. If the diagnosis is confirmed, portoenterostomy is performed by Kasai. Ideally, this should be done during the first two weeks of life. After this period, the forecast significantly worsens. After the operation, many patients have serious chronic problems, including persistent cholestasis, recurrent ascending cholangitis, delay in weight gain. Even with optimal treatment, many children develop cirrhosis and require a liver transplant.

What prognosis is the cholestasis of the newborn?

Atresia of the bile ducts progresses and, in the absence of treatment, leads to hepatic insufficiency, cirrhosis with portal hypertension for several months and the death of a child up to a year. Newborn cholestasis associated with neonatal hepatitis syndrome (especially idiopathic) is usually slowly resolved, however, damage to liver tissue can occur and lead to death.