NEONATAL CHOLESTASIS (NC)

NEONATAL CHOLESTASIS (NC)

Jaundice in neonates is common, usually secondary to unconjugated or indirect hyperbilirubinemia, and generally is not dangerous to the infant. However, jaundice should be investigated if it is of very early onset (less than 24 hours of life), prolonged beyond 14 days of life, or at high levels. Conjugated hyperbilirubinemia at any age is pathological and warrants early and rapid evaluation as timing of intervention in some cases directly affect clinical outcomes. Prompt detection of a case and timely referral to a Pediatric Hepatologist is very crucial. Despite so much awareness, many of the NC cases are still diagnosed and referred late and are inadequately treated. Biliary atresia is most common condition which presents as neonatal cholestasis (NC).

Bile, primarily composed of bilirubin, bile acids, and lipids, is formed in the liver cells (hepatocytes), and is secreted into the canaliculi. From the canaliculi, bile flows into biliary ducts from where it is eventually secreted into the intestine. Disruption of this process at any level results in cholestasis, resulting in abnormal accumulation of these substances in liver and in the blood. This, if left uncorrected, gradually damages the liver and leads to chronic liver disease (cirrhosis) and the symptoms. In India, NC constitutes around 15 – 30% of all chronic liver diseases in children.

Cholestasis is often classified by origin and is designated as either (1) Biliary, referring to structural abnormalities and obstruction of intrahepatic or extrahepatic bile ducts (often presents with acholic stools); or (2) Hepatocellular, resulting from infective, metabolic or genetic abnormalities or from impairment of bile transport. Clinically, cholestasis in infants may present as jaundice (dark coloured urine with or without acholic stools), abdomen distension, irritability, failure to gain weight/malnutrition, pruritus, fat-soluble vitamin deficiency, spontaneous bleeding or acute liver failure.