Pharmacologic Suppression of the Fetal Adrenal Gland In Utero: Attempted Prevention of Abnormal External Genital Masculinization in Suspected Congenital Adrenal Hyperplasia

Mark I. Evans, George P. Chrousos, Dean W. Mann, John W. Larsen, Ira Green, James McCluskey, Donald (Lynn) Loriaux, John C. Fletcher, Gregory Koons, James Overpeck, Joseph D. Schulman

Research output: Contribution to journalArticle

63 Citations (Scopus)

Abstract

21-Hydroxylase deficiency results in congenital adrenal hyperplasia and leads to masculinization of the external genitalia of affected females. This complication could be avoided if fetal adrenal gland function were suppressed. A woman with mild 21-hydroxylase deficiency whose previous female child had classic congenital adrenal hyperplasia with masculinization was given dexamethasone beginning at the tenth week of gestation. Maternal estriol and cortisol values indicated rapid and sustained fetal and maternal adrenal gland suppression. At 39 weeks' gestation, the patient was spontaneously delivered of a female neonate with normal external genitalia. Postnatal tests indicated the infant was a single heterozygote for 21-hydroxylase deficiency. This study demonstrates prolonged suppression of the fetal adrenal gland with dexamethasone and suggests it might prevent abnormal masculinization in fetuses with severe congenital adrenal hyperplasia.

Original languageEnglish (US)
Pages (from-to)1015-1020
Number of pages6
JournalJAMA - Journal of the American Medical Association
Volume253
Issue number7
DOIs
StatePublished - Feb 15 1985
Externally publishedYes

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Congenital Adrenal Hyperplasia
Adrenal Glands
Dexamethasone
Mothers
Female Genitalia
Pregnancy
Estriol
Genitalia
Heterozygote
Hydrocortisone
Fetus
Newborn Infant
Congenital adrenal hyperplasia due to 21 hydroxylase deficiency

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Pharmacologic Suppression of the Fetal Adrenal Gland In Utero : Attempted Prevention of Abnormal External Genital Masculinization in Suspected Congenital Adrenal Hyperplasia. / Evans, Mark I.; Chrousos, George P.; Mann, Dean W.; Larsen, John W.; Green, Ira; McCluskey, James; Loriaux, Donald (Lynn); Fletcher, John C.; Koons, Gregory; Overpeck, James; Schulman, Joseph D.

In: JAMA - Journal of the American Medical Association, Vol. 253, No. 7, 15.02.1985, p. 1015-1020.

Research output: Contribution to journalArticle

Evans, Mark I. ; Chrousos, George P. ; Mann, Dean W. ; Larsen, John W. ; Green, Ira ; McCluskey, James ; Loriaux, Donald (Lynn) ; Fletcher, John C. ; Koons, Gregory ; Overpeck, James ; Schulman, Joseph D. / Pharmacologic Suppression of the Fetal Adrenal Gland In Utero : Attempted Prevention of Abnormal External Genital Masculinization in Suspected Congenital Adrenal Hyperplasia. In: JAMA - Journal of the American Medical Association. 1985 ; Vol. 253, No. 7. pp. 1015-1020.
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abstract = "21-Hydroxylase deficiency results in congenital adrenal hyperplasia and leads to masculinization of the external genitalia of affected females. This complication could be avoided if fetal adrenal gland function were suppressed. A woman with mild 21-hydroxylase deficiency whose previous female child had classic congenital adrenal hyperplasia with masculinization was given dexamethasone beginning at the tenth week of gestation. Maternal estriol and cortisol values indicated rapid and sustained fetal and maternal adrenal gland suppression. At 39 weeks' gestation, the patient was spontaneously delivered of a female neonate with normal external genitalia. Postnatal tests indicated the infant was a single heterozygote for 21-hydroxylase deficiency. This study demonstrates prolonged suppression of the fetal adrenal gland with dexamethasone and suggests it might prevent abnormal masculinization in fetuses with severe congenital adrenal hyperplasia.",
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