Benign disorder of metabolism of bilirubin resulting
in transient neonatal hyperbilirubinemia (unconjugated bilirubin).
Transient Familial Neonatal Hyperbilirubinemia.
NB: Lucey-Driscoll syndrome is occasionally misspelled
Familial or sporadic. Usually all siblings are
affected. Cases transmitted as an autosomal recessive trait (gene map locus
is 2q37) have been reported.
A substance, probably a metabolite of
gestational hormones (inhibitor of uridine diphosphate
[UDP]-glucuronosyltransferase activity), that inhibits bilirubin
conjugation is responsible for the disease. This inhibitor is present in
the sera of both mother and infant. Unconjugated hyperbilirubinemia, which is
more severe than the form observed in breast milk jaundice, is present. Serum
bilirubin may reach 40 mg/dl (680
The presence of significant neonatal jaundice frequently
associated with a familial pattern. All siblings are usually affected, and
there is frequently a history of the phenomenon occurring in previous
generations. Other causes of hyperbilirubinemia should be excluded.
Unconjugated hyperbilirubinemia resulting in
jaundice usually presenting on the third to fifth day of life and persisting
for 3 weeks. Left untreated, kernicterus may develop. Phototherapy is used
to treat the hyperbilirubinemia.
Exclude other causes of
hyperbilirubinemia, such as sepsis, hemolytic disease of the newborn, and
biliary atresia. Ensure adequate hydration. Obtain coagulation profile and
This syndrome occurs only
in neonates; therefore, basic principles of safe neonatal anesthesia must be
Drugs that interfere with
metabolism of bilirubin or that may displace bilirubin from albumin could
increase the risk of hyperbilirubinemia or kernicterus. Sulfonamides,
ceftriaxone, pancuronium, and chloral hydrate are associated with
hyperbilirubinemias include (1) those resulting in predominantly
unconjugated hyperbilirubinemia, such as Gilbert or Arias syndrome,
Crigler-Najjar syndrome type I, and Crigler-Najjar syndrome type II; and (2)
those resulting in predominantly conjugated hyperbilirubinemia, such as
Dubin-Johnson syndrome, Rotor syndrome, and other forms of intrahepatic
Breast Milk Jaundice: Jaundice occurring in breast-fed neonate
around the fourth to seventh day of life, persisting beyond physiologic
jaundice, and with no other identifiable cause, probably resulting from a
milk component that inhibits uridine diphosphoglucuronic acid (UDPGA)
glucuronyl transferase, thus resulting in a prolonged unconjugated
Crigler-Najjar Syndrome: Inherited error of bilirubin metabolism
in which bilirubin cannot be converted into water-soluble bilirubin
glucuronide because of a defect of hepatic glucuronyl transferase.
Crigler-Najjar syndrome type II is less severe than type I.
Gilbert Syndrome: Characterized by normal liver function tests,
normal liver histology, delayed clearance of bilirubin from the blood, and
mild jaundice that tends to fluctuate in severity, particularly after
fasting. It is defined as a mild unconjugated hyperbilirubinemia resulting
from a mutation in the promoter of the UDP-glucuronosyltransferase gene
(allelic to the mutation for Crigler-Najjar syndrome type I).