SAN FRANCISCO -- Prenatal treatment of congenital adrenal hyperplasia with dexamethasone effectively reduced virilization of the genitalia in female infants with few, mostly mild effects on the mothers, according to one of the largest series of such cases ever reported.
"All of the fetuses who were treated from the sixth or seventh week and were treated to term ... ended up with [genitalia rated as] Prader 1, which is normal, or Prader 2 at the most," Dr. Maria I. New said at the annual meeting of the Endocrine Society.
The case series presented by Dr. New, a professor of pediatrics at Cornell University, New York, included 576 pregnant women undergoing amniocentesis or chorionic villi sampling for prenatal diagnosis of congenital adrenal hyperplasia because of a family history of the disorder.
The women were seen at New York-Presbyterian hospital between 1978 and 2002.
There were 64 female infants with classic 21-hydroxylase deficiency. A total of 27 women started treatment before the ninth week of gestation, 24 either started treatment after the ninth week or were not compliant throughout pregnancy, and 13 were untreated.
Treatment was 20 [micro]g of dexamethasone daily in three divided doses.
Infants of treated women had a mean Prader grade of 1. Those who were treated late had infants with a mean grade of 3, and the untreated women's babies had a mean grade of almost 4, according to Dr. New
Prior reports of dexamethasone treatment have not described such good results. They tended to suggest that it is successful in preventing virilization of genitalia 75% of the time, with one-third of the successful cases appearing normal and two-thirds with slight virilization, she commented.
But those reports did not rigorously separate pregnancies treated very early and with good compliance, Dr. New said.
"When you look at those treated too late, you don't really have much effect on the external genitalia," she maintained. "Treatment must begin in the eighth week of gestation or earlier."
There was no statistically significant difference in the percentage of hypertension cases among treated and untreated women. Differences that were significant included striae, edema, and weight gain.
In the coming year, Dr. New said she intends to investigate lowering the dexamethasone dosage.
COPYRIGHT 2002 International Medical News Group
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