etic counseling and prenatal diagnosis in subsequent pregnancies in affected families." The prenatal diagnosis may show that the baby is completely free of the mutation, or that they have only one copy of the defective gene from either the mother or the father. These babies will not develop the disorder. It is only when the baby has defective genes from both the mother and the father that the disease develops in the mother during pregnancy and in the baby a few months after birth.
Once diagnosed, these babies are placed on special diets that reduce the amount of fat and change the type of fat, which counteracts the effect of the gene mutation. For instance, these babies can metabolize coconut oil, Ibdah said. These babies also need to avoid fasting.
Ibdah had reported in the New England Journal of Medicine in June 1999 that he and his colleagues had identified the genetic defect in 24 infants or toddlers who had come in with liver, heart or muscular abnormalities that suggested their bodies were not using fatty acids properly. Seven infants died almost immediately and another one about 18 months later. The remaining babies were given the special diet in formulas; older children follow similar diets.
They documented long-term survival of the children on the special diet. Now, said Ibdah, they are reporting that moving in quickly by screening infants born to mothers with acute fatty liver of pregnancy before symptoms develop can be lifesaving -- though children with this defect will need to remain on special diets. The 108 women in the study were referred from 27 states.
Page: 1 2 Related biology news :1
Contact: Robert Conn
Wake Forest University Baptist Medical Center
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