Lucey-Driscoll syndrome
Encyclopedia
Lucey-Driscoll syndrome is an autosomal recessive metabolic disorder affecting enzymes involved in bilirubin
Bilirubin
Bilirubin is the yellow breakdown product of normal heme catabolism. Heme is found in hemoglobin, a principal component of red blood cells. Bilirubin is excreted in bile and urine, and elevated levels may indicate certain diseases...

 metabolism.

It is one of several disorders classified as a transient familial neonatal unconjugated hyperbilirubinemia.

Cause

The common cause is congenital, but it can also be caused by maternal steroids passed on through breast milk
Breast milk
Breast milk, more specifically human milk, is the milk produced by the breasts of a human female for her infant offspring...

 to the newborn. It is different from breast milk jaundice (breast-fed infants have higher bilirubin levels than formula-fed ones).

Genetics

A defect in the UGT1A1
UGT1A1
UDP-glucuronosyltransferase 1-1 also known as UGT-1A is an enzyme that in humans is encoded by the UGT1A1 gene.UGT-1A is a uridine diphosphate glucuronyltransferase , an enzyme of the glucuronidation pathway that transforms small lipophilic molecules, such as steroids, bilirubin, hormones, and...

-gene, also linked to Crigler-Najjar syndrome
Crigler-Najjar syndrome
Crigler-Najjar Syndrome or CNS is a rare disorder affecting the metabolism of bilirubin, a chemical formed from the breakdown of blood. The disorder results in an inherited form of non-hemolytic jaundice, which results in high levels of unconjugated bilirubin and often leads to brain damage in...

 and Gilbert's syndrome
Gilbert's syndrome
Gilbert's syndrome , often shortened to GS, also called Gilbert-Meulengracht syndrome, is the most common hereditary cause of increased bilirubin and is found in up to 5% of the population...

, is responsible for the congenital form of Lucey-Driscoll syndrome.

External links

- transient familial neonatal hyperbilirubinemia, breast feeding jaundice included
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