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 to the newborn. It is different from breast milk jaundice (breast-fed infants have higher bilirubin levels than formula-fed ones).
Genetics
Lucey-Driscoll syndrome has an autosomal recessive pattern of inheritance.
A defect in the UGT1A1-gene, also linked to Crigler-Najjar syndrome and Gilbert's syndrome, is responsible for the congenital form of Lucey-Driscoll syndrome
Lucey Driscoll syndrome: A rare condition characterized by severe jaundice at birth and caused by the presence of a gestational hormone that passes from the mother to the infant across the placenta but eventually disappears after birth. Excessive jaundice result in kernicterus can cause complications such as brain damage. More detailed information about the symptoms, causes, and treatments of Lucey Driscoll syndrome is available below.
The common cause is congenital, but it can also be caused by maternal steroids passed on through breast milk to the newborn. It is different from breast milk jaundice (breast-fed infants have higher bilirubine levels than formula-fed ones).
Transient familial hyperbilirubinemia is a metabolic disorder that is passed down through families. Babies with this disorder are born with severe jaundice.
Transient familial hyperbilirubinemia is an inherited disorder. It occurs when the body does not properly break down (metabolize) a certain form of bilirubin. Bilirubin levels rapidly build up in the body. The high levels are poisonous to the brain and can cause death.
Outlook (Prognosis)
Babies who are treated can have a good outcome. If the condition is not treated, severe complications develop. This disorder tends to improve with time.
Possible Complications
Death or severe brain and nervous system (neurological) problems can occur if the condition is not treated.
What Is Eruptive Xanthomatosis?
A harmless skin reaction that looks like small bumps, eruptive xanthomatosis might indicate that you have high cholesterol. You must treat high cholesterol, since it puts you at risk for atherosclerosis.
What Causes Eruptive Xanthomatosis?
Eruptive xanthomatosis results from poorly controlled blood sugar levels. It can also occur when your level of triglycerides (a form of fat) are elevated. Triglycerides exist normally in your body, but also come from food high in sugar, such as candy, honey and alcohol. Often, people with high triglycerides have high LDL, the "bad" cholesterol.
If you are insulin resistant, your body struggles to clear fat from your blood stream, and this can raise your triglycerides.
What Are the Symptoms of Eruptive Xanthomatosis?
Eruptive xanthomatosis usually appears on the shoulders, the buttocks or along the surfaces above the muscles that help you move your joints. On rare occasions, it can occur in your mouth.
Often the itchy, tender, pea-size bumps appear reddish-yellow.
How Can You Treat Eruptive Xanthomatosis?
Eruptive xanthomatosis often disappears by itself within a few weeks. However, you should still seek treatment because of the condition's strong association with high levels of triglycerides.
Treatment involves getting your triglyceride, cholesterol and blood glucose levels under control. Doing so might require the use of lipid-lowering drugs (such as statins like Lipitor® or Zocor®) or fibrates (such as TriCor® or Lopid®).
How Can You Prevent Eruptive Xanthomatosis?
Keep your blood sugar levels in the range recommended by your doctor. Limit your intake of
| Design By : Pichak |