Neonatal Jaundice Part 1 : Pediatrics
Автор: Dr.G.Bhanu Prakash
Загружено: 2022-02-19
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Neonatal Jaundice Part 1 : Pediatrics
Bilirubin is a waste product of the heme from degraded blood cells. It is conjugated in the liver by UDP-glucuronosyltransferase (UDPGT) to produce conjugated bilirubin, which is water soluble and can be excreted renally or in the bile.
Neonatal jaundice occurs because of physiologic reduction of UDPGT at birth, maternal-fetal blood type incompatibility with hemolysis, or genetic disorders of liver bilirubin conjugation or excretion.
Neonatal jaundice presents with yellow skin and eyes shortly after birth and can lead to neurologic deficits (kernicterus) if severe.
Physiologic neonatal jaundice is due to a combination of reduced RBC lifespan, increased RBCs at birth, and temporary reduction in UDPGT enzyme at birth. It is self-limited.
In physiologic neonatal jaundice, severe increases in bilirubin are treated with phototherapy to prevent kernicterus.
Hemolytic disease of the newborn occurs when an Rh-negative mother produces anti-Rh antibodies against the red blood cells of an Rh-positive baby, or more rarely, when there is ABO incompatibility.
Hemolytic disease of the newborn is treated with fetal and newborn transfusion. It is prevented by Rh immunoglobulin therapy for Rh-negative mothers carrying Rh-positive babies. Complications include fetal hydrops, which is often fatal.
Gilbert syndrome is a common autosomal recessive disease caused by a mild decrease in UDPGT, leading to mild jaundice in response to stressors.
Crigler-Najjar syndrome is a rare autosomal recessive disease caused by the complete absence of UDPGT. It can be mild or it can present with jaundice, kernicterus, and high levels of unconjugated bilirubin.
Dubin-Johnson and Rotor syndromes are rare, mild autosomal recessive diseases caused by defective liver secretion of conjugated bilirubin.
#neonataljaundice #pediatrics #hyperbilirubinemia #Conjugatedhyperbilirubinemia #unconjugatedhyperbilirubinemia #Hemolyticdiseaseofthenewborn #kernicterus #GilbertSyndrome #CriglerNajjar Syndrome
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