Neonatal cholestasis

Neonatal cholestasis refers to elevated levels of conjugated bilirubin identified in newborn infants within the first few months of life. Conjugated hyperbilirubinemia is clinically defined as >20% of total serum bilirubin or conjugated bilirubin concentration greater than 1.0 mg/dL regardless of total serum bilirubin concentration. The differential diagnosis for neonatal cholestasis can vary extensively. However, the underlying disease pathology is caused by improper transport and/or defects in excretion of bile from hepatocytes leading to an accumulation of conjugated bilirubin in the body.

Neonatal cholestasis can present in newborn infants within the first few months of life. While neonatal cholestasis can present from a number of pathologic causes, 35 40% of neonatal cholestasis cases are caused by biliary atresia. This is one of the most common causes for neonatal cholestasis. Metabolic and genetic disorders cause 9 17% of cases, infectious processes cause 1 9% of cases, Alagille syndrome causes 2 6% of cases, and idiopathic cases arise 13 30% of the time. The breakdown of hemoglobin and heme releases unconjugated bilirubin into the blood stream. This unconjugated bilirubin is transported to the liver by albumin The common hepatic duct will combine with the cystic duct from the gallbladder to form the common bile duct.

If neonatal cholestasis is suspected or an infant is presenting with jaundice after two weeks of life, total and conjugated bilirubin must be measured. Neonatal cholestasis is present if conjugated bilirubin value is >20% of total serum bilirubin or if serum conjugated bilirubin concentration is greater than 1.0 mg/dL. This modification can be sufficient in treating neonatal complications. Neonatal cholestasis caused by alpha 1 antitrypsin deficiency does not have any specific treatment. Instead, children with alpha 1 antitrypsin deficiency are primarily managed by treating symptoms clinically. Most children will also require a liver transplant in the future because they will progress to having cirrhosis, hepatocellular carcinoma, and/or chronic liver disease. Generally, treatment of neonatal cholestasis involves treating the underlying disease. Prognosis of neonatal cholestasis also varies based on the underlying pathologic process.