Write a short note on congenital jaundice.
The best example of congenital jaundice is Criggler Najjar syndrome in which the enzyme involved in glucuronide conjugation is absent or reduced in amount, this can be treated by phenobarbitone that induces hepatic microsmomal enzymes.
Jaundice is the yellow discoloration of the skin and eyes due to elevated serum bilirubin concentration. The serum bilirubin level required to cause yellow discoloration is usually at a level of 2 to 3 mg/dL. With increasing bilirubin levels, it advances in the head-to-foot direction, appearing at the umbilicus at about 15 mg/dL and at the feet at about 20 mg/dL.
Consequences of hyperbilirubinemia: Kernicterus is the major consequence of neonatal hyperbilirubinemia. It is brain damage caused by unconjugated bilirubin deposition in the basal ganglia and brain stem nuclei, caused by either acute or chronic hyperbilirubinemia. Normally, bilirubin binds to serum albumin and stays in the intravascular space, but in certain conditions it crosses the blood brain barrier, like:
- When serum bilirubin concentration is markedly elevated
- When serum albumin level is markedly low
- When bilirubin is displaced from albumin by competitive binders (eg, sulfisoxazole , ceftriaxone, aspirin) and free fatty acids and hydrogen ions (eg, in fasting, septic, or acidotic infants).
Mechanisms of hyperbilirubinemia: Hyperbilirubinemia can be caused by one or more of the following processes:
- Increased production
- Decreased hepatic uptake
- Decreased conjugation
- Impaired excretion
- Impaired bile flow (cholestasis)
- Increased enterohepatic circulation
Etiology: Hyperbilirubinemia can be classified as physiologic or pathologic. It can be classified by whether the hyperbilirubinemia is unconjugated, conjugated, or both. Some of the jaundice are:
- Physiologic hyperbilirubinemia
- Breastfeeding jaundice
- Breast milk jaundice
- Pathologic hyperbilirubinemia due to hemolytic disease
- Liver dysfunction (eg, caused by parenteral alimentation causing
- cholestasis, neonatal sepsis, neonatal hepatitis) may cause a
- conjugated or mixed hyperbilirubinemia
Author: Ridham Khanderia
- Dutta's textbook of obstetrics Chapter 32, 7th edition
- Ghai's textbook of Pediatrics Chapter 8: Newborn baby , 8th edition.