NEONATAL JAUNDICE

  •  

    NEONATAL JAUNDICE

     

    DEFINITION:

    A disorder caused by an excess of unconjugated or conjugated bilirubin in the newborn period.

    EPIDEMIOLOGY:

    • incidence: ?
    • age of onset:
      • newborn period
    • risk factors:
      • see differential diagnosis

    DIFFERENTIAL DIAGNOSIS FOR UNCONJUGATED HYPERBILIRUBINEMIA:

    1. Increased Production

  • 1. Hemolytic Diseases
    • Intrinsic
      • Membrane
      • Enzyme
      • Hemoglobin Synthesis
    • Extrinsic
      • Immune
      • Non-immune
  • 2. Infection
    • Sepsis
    • TORCH infections
  • 3. Extravesated
    • bruising
    • cephalohematoma
    • delayed cord clamping
    • transfusion (twin-twin, maternal-fetal)
  • 4. Enterohepatic Circulation Increase
    • delayed feeding
    • delayed stooling
    • dehydration
    • GI obstruction
  • 5. Polycythemia
  • 2. Decreased Conjugation

  • 1. Crigler-Najjar Syndrome - I
  • 2. Gilbert Syndrome
  • 3. Lucy-Driscoll Syndrome
  • 4. Breast Milk Jaundice
  • DIFFERENTIAL DIAGNOSIS OF CONJUGATED HYPERBILIRUBINEMIA:

    1. Intrahepatic Cholestasis

  • 1. Persistent
    • Alagille Syndrome (arteriohepatic dysplasia)
    • Benign Recurrent Intrahepatic Cholestasis
    • Byler Disease
    • Cholestasis - Lymphedema
    • Neonatal Hepatitis
    • NSP of Interlobular Bile Ducts
  • 2. Acquired
    • Infections
      • Toxoplasmosis
      • Others - Echovirus, Leptospirosis, Syphilis, TB,
      • Varicella
      • Rubella
      • CMV, Coxsackievirus
      • Hepatitis B (?C), HSV
    • Drug-Induced
  • 3. Metabolic/Genetic Disorders
    • Alpha-1-Antitrypsin Deficiency
    • Cystic Fibrosis
    • Dubin-Johnson Syndrome
    • Familial Hepatosteatosis
    • Rotor Syndrome
    • Trihydroxycoprostanic Acidemia
    • Zellweger's Syndrome
    • Trisomies 18 and 21
  • 2. Extrahepatic Obstruction

  • 1. Infantile Obstructive Cholangiopathy
    • Biliary Atresia
    • Bile Plug Syndrome
    • Choledochal Cyst
    • Choledocholithiasis
    • extrinsic bile duct compression
    • spontaneous bile duct perforation
  • 3. Metabolic Disorders

  • 1. Disorders of Carbohydrate Metabolism
    • Hereditary Fructose Intolerance
    • Galactosemia-I+III
    • Glycogen Storage Diseases - Types I,III,IV,VI,IX
  • 2. Disorders of Amino Acid Metabolism
    • Tyrosinemia
  • 3. Disorders of Lipid Metabolism
    • Gaucher Disease
    • Niemann-Pick Disease
    • Wolman Disease
    • Cholesteryl Ester Storage Disease
  • PATHOPHYSIOLOGY:

    1. Bilirubin Metabolism:

    • end product of heme degradation
    • majority derived from RBC's
    • 8-10 mg/kg/day produced
    • 1g Hb = 35 mg bilirubin
    • two types:
      • unconjugated (indirect) - prehepatic, enterohepatic
      • conjugated (direct) - hepatic

    2. Physiological Jaundice:

  • 1. Elevated Bilirubin Load
    • increased RBC volume
    • decreased RBC survival
    • increased enterohepatic circulation
  • 2. Decreased Hepatic Uptake of Bilirubin
    • low level of ligandin
    • competition for binding to intracellular proteins
  •  

  • 3. Defective Bilirubin Conjugation
    • decreased UDP glucuronyl transferase activity
  •  

  • 4. Defective Bilirubin Excretion
  •  

    3. Non-Physiological Jaundice:

    • see differential diagnosis

    CLINICAL FEATURES:

    1. Physiologic Jaundice

    1. Features

    • appears on Day 2
    • peaks on Days 2-5
    • disappears after Day 7 (term infants)
    • disappears after Day 14 (premature infants)
    • of term babies:
      • 60% are jaundiced within the first week
      • 95% have a bilirubin < 260 umol/L
    • visible jaundice occurs at levels > 85 umol/L

    2. Non-Physiologic Jaundice

    1. Features

    • within 24 hours
    • total bilirubin > 225 umol/L
    • direct bilirubin > 35 umol/L or > 30-40% of total
    • rate of rise > 85 umol/L in a 24 hour period
    • persists > 7 days (term) or > 14 days (preterm)

    3. Approach

    1. History

  • 1. Maternal History
    • blood type
    • past obstetrical history
      • abortions (spontaneous or induced)
      • jaundiced sibs
    • past medical history
      • IDDM
    • family history
      • inborn errors of metabolism, cystic fibrosis
      • congenital hepatic diseases
      • congenital hemolytic anemia
        • jaundice, anemia, splenectomy, gallbladder disease
  • 2. Pregnancy History
    • complications
      • infections (TORCH), illnesses, PIH, GDM
      • sepsis - premie, fever, increased WBC, PROM, amnionitis, etc
  • 3. Labour and Delivery History
    • gestational age - ? premie
    • method of delivery - ? traumatic
    • APGAR or cord gases - ? birth asphyxia
    • ingestion of maternal blood
    • delayed cord clamping
  • 4. Post Natal History
    • characteristics of jaundice - ? onset, duration, etc.
    • method of feeding - ? breast milk jaundice
    • infants blood group - ? Rh or ABO incompatability
    • evidence of underlying illness:
      • delayed passage of meconium, nausea/vomiting, sepsis,
      • failure to thrive, decreased caloric/fluid intake
  • 2. Physical

  • 1. Vitals
    • temperature (sepsis)
    • weight/length (SGA, LGA)
    • head circumferance (decreased size with TORCH infections)
  • 2. HEENT
    • cephalohematoma
    • bruising
  • 3. Gastrointestinal
    • hepatosplenomegaly (hemolytic anemia, congenital infections, inborn errors of metabolism)
    • mass/distention (obstruction, adrenal hematoma)
  • 4. Skin
    • plethoric (polycythemia)
    • pallor (anemia)
    • petchechiae (sepsis, congenital infections, severe hemolytic anemia)
  • INVESTIGATIONS:

    1. First Line

    • bilirubin (total and direct)
    • blood groups and Rh (mother and infant)
    • CBC (Hct, Hb, Platelets)
    • Coombs test
    • peripheral blood smear/morphology
    • reticulocyte count

    2. Second Line

    • septic work-up
      • cultures (blood, CSF, urine), chest x-ray
    • screen for hypothyroidism
      • TSH, T4
    • screen for inborn errors of metabolism
      • urine for organic and amino acids
      • plasma for amino acids
    • alkaline denaturation of Hb test of emesis
    • - PT, PTT, haemoglobin electrophoresis

    MANAGEMENT:

    1. Alter Feeding

    • interrupt breast-feeding
    • stimulate enterohepatic circulation

    2. Phototherapy

    • based on birth weight and age of patient
    • white or blue lights, single or double bank
    • principle: photoisomerization

    3. Exchange Transfusion

    • direct removal of bilirubin

    4. Medications

    • phenobarbital trial to induce glucuronyl transferase activity

     

     

    Pediatric Database - NEONATAL JAUNDICE

    Pediatric Organization - Pedbase [at] Gmail.com