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At a glance

A benign disorder causing defects in the metabolism of bilirubin resulting in transient neonatal hyperbilirubinemia (unconjugated bilirubin).

Synonym

Transient Familial Neonatal Hyperbilirubinemia Syndrome.

N.B.: Lucey-Driscoll Syndrome is occasionally misspelled “Lucy-Driscoll Syndrome.”

Incidence

The exact incidence remains unknown. It is a very rare medical condition and only 24 cases have been reported in the literature.

Genetic inheritance

Familial or sporadic. Usually all siblings are affected. Cases transmitted as an autosomal recessive trait (gene map locus is 2q37) have been reported.

Pathophysiology

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 µmol/L).

Diagnosis

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.

Clinical aspects

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.

Precautions before anesthesia

Exclude other causes of hyperbilirubinemia such as sepsis, hemolytic disease of the newborn, and biliary atresia. Ensure adequate hydration. Obtain coagulation profile and bleeding time.

Anesthetic considerations

This syndrome occurs only in neonates; therefore, basic principles of safe neonatal anesthesia must be applied.

Pharmacological implications

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 hyperbilirubinemia.

Other conditions to be considered

The hereditary 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 cholestasis.

  • Breast Milk Jaundice Syndrome: 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 hyperbilirubinemia.

  • Crigler-Najjar Syndrome: Inherited error of bilirubin metabolism in which bilirubin cannot be converted into water-soluble bilirubin glucuronide because of a defect of ...

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