MedlinePlus Medical Encyclopedia: Lucey-Driscoll Syndrome

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Lucey-Driscoll Syndrome

Contents of this page:
Alternative names Definition Causes, incidence, and risk factors Symptoms Signs and tests	Treatment Expectations (prognosis) Complications Calling your health care provider Prevention

Alternative names

Transient familial neonatal hyperbilirubinemia

Definition Return to top

Lucey-Driscol Syndrome is an inherited metabolic disorder characterized by severe hyperbilirubinemia (jaundice) that is present in the infant at birth.

Causes, incidence, and risk factors Return to top

Lucey-Driscol syndrome is thought to be an autosomal recessive inherited disorder. There is an undetermined enzyme defect in the metabolism of bilirubin. Bilirubin accumulates and levels escalate rapidly. These high levels are toxic to the brain and can cause death.

Symptoms Return to top

jaundice (yellow skin) and icterus (yellow eyes) of the newborn
lethargy of the newborn
family history of this disorder
if untreated, seizures and neurologic problems (kernicterus) may develop

Signs and tests Return to top

Blood tests for bilirubin levels are used to confirm the severity of the jaundice.

Treatment Return to top

Phototherapy (with blue light) is used to treat the high level of bilirubin. An exchange transfusion is sometimes necessary to treat this disorder.

Expectations (prognosis) Return to top

If treated, the outcome can be good. Untreated, severe complications develop because of the accumulation of bilirubin. As indicated by the name transient, this disorder tends to improve with time after the critical newborn period.

Complications Return to top

Death or severe neurological deficits can occur if the condition is not treated.

Calling your health care provider Return to top

Although this problem would most likely be noted immediately after delivery, call your doctor if you notice your baby's skin turning yellow. There are other causes for jaundice in the newborn that are easily treated.

Prevention Return to top

Phototherapy can significantly prevent the potentially serious complications of this disorder.

Update Date: 10/9/2003

Updated by: Celeste Krauss, M.D., Board Certified Clinical Genetics, Clinical Cytogenetics and Pediatrics. Founding Fellow, American College of Medical Genetics. Review provided by VeriMed Healthcare Network.

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