Hyperbilirubinemia

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Introduction:
Newborns appear jaundiced when bilirubin
level > 7 mg/dL while adults appear jaundiced
when bilirubin level > 2 mg/dL.
25-50% of all term newborns develop clinical
jaundice.
6% of term newborns have maximum bilirubin
level >12.9 mg/dL.
3% > 15mg/dL.
Normal Newborn produces 6-10 mg/ kg/day
bilirubin.
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Types of bilirubin:
1. Unconjugated bilirubin tightly bound to
albumin.
2. Free bilirubin.
3. Conjugated bilirubin.
4. Delta fraction.

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Source of bilirubin:
Heme containing proteins:
1. 75% RBCs Hb: 1g Hb - 34mg bilirubin.
Accelerated Hb release from from RBCs:
a) Isoimmunization.
b) RBCs biochemical abnormalities.
c) Abnormal RBCs morphology.
d) Sequestered RBCs.
c) Polycythemia.
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2. 25% early labeled bilirubin.

a) Inefective erythropoiesis.
b) Other heme containing protiens.
c) Free heme.

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Unconjugated hyperbilirubunemia:
1. Increased production from heme:
1)Hemolytic disease: incompatibility, cell wall
defects, enzyme defects, Hb pathy, sepsis,
microangiopathy.
2)Ineffective erythropoiesis.
3)Drugs.
4)infections.
5)Enclosed hematoma.
6)Polycythema.
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2. Decreased delivery to hepatocytes:

1)Right sided congestive heart failure.
2)porta caval shunt.
3. Decreased uptake across hepatocyte
membrane:
1)enzyme transporter defeciency.
2)competitive inhibition: breast milk
jaundice, Lucey-Drscoll, drug inhibition.
3)Miscellaneous: hypothyroidism, hypoxia,
acidosis.
4. Decreased Y & Z proteins:
fever.
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5. Decreased biotransformation:
1)Physiologic jaundice.
2)Inhibition.
3)Crigler-Najjar syndrome:type 1(complete
defeciency), type 2(partial).
4)Gilbert disease.
5)Hepatocellular dysfunction.
6. Enterohepatic circulation:
1)Intestinal obstruction.
2)Antibiotic administration.
7. Breast milk jaundice.
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Cholestasis:
1. Infectious:
1)bacterial sepsis.
2)viral hepatitis.
3)others.
2. Toxic:
1)parenteral nutrition.
2)endotoxemia.
3)drugs.
3. Metabolic:
1)defects in a.a. metabolism.
2)defects in lipid metabolism.
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3)defects in carbohydrates metabolism.

4)defects in bile biosynthesis.
5)other metabolic defects.
4. Genetic/chromosomal:
1)trisomoy E.
2)Down syndrome.
3)donahue syndrome.
5. Intrahepatic disease:
1)intrahepatic cholestasis-persistent:
idiopathic neonatal hepatitis, Alagile
syndrome, nonsyndromic hypoplasia or
paucity of intrahepatic bile ducts, PFIC.
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2)intrahepatic cholestasis-recurrent:
Aagenaes, congenital hepatic fibrosis, Caroli
disease.
6. Extrahepatic disease.
7. Miscellaneous:
1)histiocytosis.
2)shock.
3)enteritis.
4)intestinal obstruction.
5)neonatal lupus.
6)myeloproliferative disease(trisomy 21).
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Fetal bilirubin metabolism:

Is limited due to:
1. Decreased hepatic blood flow.
2. Decreased hepatic ligandin.
3. Decreased UDPG-T activity.
Bilirubin appear in amniotic fluid by 12 weeks of
gestation.
Bilirubin in amniotic fluid increased hemolytic
diseases & by obsruction below the bile
ducts.
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Diagnosis of the etiology of

hyperbilirubiunemia in neonates:
If there is clinical jaundice, measure TSB:
1. TSB < 12 or age > 1 day: follow up.
2. TSB > 12 or age < 1 day:
Then do coombs test
If + ve: incombatibility.
If - ve then do D. bilirubin.

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If D.bilirubin > 2 then consider:

1. Hepatitis.
2. Intrauterine viral or toxoplasma infection.
3. Biliary obstruction.
4. Sepsis.
5. Galactosemia.
6. Alpha-1-antitrypsin defeciency.
7. Cystic fibrosis.
8. Tyrosinosis.
9. Cholestasis.
10. Hyperalimenation.
11. Syphillis.
12. Hemochromatosis.
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If D.bilirubin < 2 then do Hct.

if Hct is high then consider polycythemia.
if Hct low or normal lookmfor RBCs
morphology & reticulocyte count.
if abnormal consider: incompatibility, cell
wall defects, enzyme defects, Hb pathy,
drugs, DIC.

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If normal consider:
enclosed hemorrhage, increased
enterohepatic circulation, breast milk,
hypothyroidism, Crigler-Najjar, diabetic
mother, R.D.S., asphyxia, infection, Gilberts,
drugs, galactosemia.

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