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Updated: Dec 29 2021

Neonatal Jaundice

  • Snapshot
    • A healthy 2-week-old male presents with mild jaundice. He was born at term. Labs show a total billirubin of 18 mg/dl (normal <7 mg/dl), and a direct billirubin of 0.8 mg/dl (normal 0-0.4).
  • Introduction
    • Physiologic jaundice
      • occurs between days 3-5 and is clinically benign
        • indirect (unconjugated) bilirubin
          • commonly rises to 9 mg/dl days 3-5 & begins to fall
          • occurs in 50% of neonates during the first week of life
          • results from increased bilirubin production and
          • relative deficiency in glucuronyl transferase in the immature liver
    • Pathologic jaundice
      • The presence of the following factors in an infant is indicative of pathologic jaundice and warrants evaluation for an underlying etiology:
        • 1) Jaundice in the first 24 hours of life is always pathologic
        • 2) Total serum bilirubin > 12 mg/dL in a term infant
        • 3) Conjugated bilirubin > 2 mg/dL or > 20% of total bilirubin level
        • 4) Total serum bilirubin rate of rise > 5 mg/dL/day
        • 5) Persistence of jaundice beyond 10-14 days
        • 6) Presence of any additional signs or symptoms indicative of potential underlying diseasedirect (conjugated) bilirubinemia
      • Bilirubin levels > 20 mg/dL can lead to kernicterus and associated neurotoxicity
        • Results from the irreversible deposition of billirubin in the basal ganglia, pons, and cerebellum
        • Potentially fatal
    • Indirect (unconjugated) hyperbilirubinemia occurs in
      • Criggler-Najjar syndrome
      • Gilbert's syndrome
      • Congenital hypothyroidism
      • Breast feeding failure
        • caused by insufficient breast milk consumption
        • reduced oral intake results in inadequate quantities of bowel movements to excrete bilirubin from the body
        • improves with increased breast milk intake
      • Breast milk
        • infant liver is not mature enough to process lipids
        • presents between 4th and 7th day of life
        • indirect bilirubin as high as 27 mg/dl during the 3rd week of life
        • no intervention needed (mother does not need to stop breast feeding)
          • if breast feeding stops, bilirubin levels fall rapidly
          • if breast feeding continues, bilirubin levels will fall slowly
      • Hemolytic anemia leading to hyperbilirubinemia caused by
        • hereditary spherocytosis
        • G6PD-deficiency
        • pyruvate kinase deficiency
        • ABO/Rh isoimmunization
        • acute or chronic infection
        • drugs
        • maternal diabetes
        • chronic fetal hypoxia
        • cephalohematoma
    • Conjugated (direct) hyperbilirubinemia occurs in
      • infections
      • galactosemia
      • alpha-1-antitrypsin deficiency
      • congenital causes include
        • extrahepatic biliary atresia
        • Dubin-Johnson syndrome
        • Rotor syndrome
  • Presentation
    • Neonates may be jaundiced with bilirubin levels > 5 mg/dl
    • Presents on physical exam with yellowish discoloration of the skin (progresses from head to toe with increasing bilirubin levels) and scleral icterus
    • Billirubin levels rising above 9 are considered abnormal and need treatment
    • Additionally, the presence of a factor listed above under "pathologic jaundice" always necessitates further work-up to determine a potential cause for the infant's jaundice
    • Blood smear
      • may show hemolysis in cases where indirect bilirubin alone is elevated
    • Coombs' test
      • used to distinguish between
        • immune mediated hemolytic disorders (ABO incompatability)
        • and non-immune-mediated hemolytic disorders including
          • G6PD deficiency
          • hereditary spherocytosis
    • LFTs, alk phos, bile acids, and sweat test
      • may identify diagnosis if direct bilirubin is elevated
      • help rule out aminoacidopathies and alpha-1-antitrypsin deficiency
    • Septic work-up
      • indicated in any jaundiced neonate with fever, hypotension, and tachypnea
    • Maternal-fetal ABO and Rh incompatibilites
      • may identify immune-related hyperbilirubinemia
  • Differential
    • Within 24 hoursHemolysis (ABO/Rh isoimmunization or hereiditary spherocytosis)
      Within 48 hoursHemolysis
      After 48 hoursInfection
      Breast Milk
      Congenital malformation (biliary atresia)
  • Treatment
    • Prevention
      • preventive measures are usually not available
      • stop breast-feeding to speed resolution of neonatal jaundice, though not required
      • avoid drugs that result in hemolytic anemia in certain patient groups
    • Medical management
      • no treatment indicated in physiologic jaundice
      • treat underlying infectious causes
      • phototherapy
        • indicated when bilirubin levels > 15-20 mg/dl regardless of cause
        • Specific nomograms exist to govern when blue-light therapy is indicated
      • exchange transfusion
        • may be indicated in very severe cases of hyperbilirubinemia
  • Complications
    • Retinal damage, dehydration, dermatitis, and diarrhea all related to UV exposure
  • Prognosis
    • Ranges widely
    • Can spontaneously resolve or be fatal depending on etiology
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