An attenuated form of congenital virilizing adrenal hyperplasia

Zev Rosenwaks; Peter A. Lee; Georgeanna S. Jones; Claude J. Migeon; Anne C. Wentz

doi:10.1210/jcem-49-3-335

An attenuated form of congenital virilizing adrenal hyperplasia

Zev Rosenwaks, Peter A. Lee, Georgeanna S. Jones, Claude J. Migeon, Anne C. Wentz

Department of Pediatrics

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Abstract

The families of two patients with hirsutism and oligomenorrhea were studied with iv ACTH (Cortrosyn) stimulation. The parents responded with a combined incremental rise of progesterone and 17-hydroxyprogesterone greater than 6.5 ng/dl-min, a response seen in the heterozygote parents of patients with congenital virilizing adrenal hyperplasia (CVAH). One sibling in one family responded as do heterozygous subjects, while two other siblings responded normally. The abnormal response of the two pairs of parents and the sibling along with the pattern of steroid secretion observed in the propositi suggest that the so-called adult-onset CVAH is a mild form of the homozygous state of CVAH.

Original language	English (US)
Pages (from-to)	335-339
Number of pages	5
Journal	Journal of Clinical Endocrinology and Metabolism
Volume	49
Issue number	3
DOIs	https://doi.org/10.1210/jcem-49-3-335
State	Published - 1979

All Science Journal Classification (ASJC) codes

Endocrinology, Diabetes and Metabolism
Biochemistry
Endocrinology
Clinical Biochemistry
Biochemistry, medical

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10.1210/jcem-49-3-335

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abstract = "The families of two patients with hirsutism and oligomenorrhea were studied with iv ACTH (Cortrosyn) stimulation. The parents responded with a combined incremental rise of progesterone and 17-hydroxyprogesterone greater than 6.5 ng/dl-min, a response seen in the heterozygote parents of patients with congenital virilizing adrenal hyperplasia (CVAH). One sibling in one family responded as do heterozygous subjects, while two other siblings responded normally. The abnormal response of the two pairs of parents and the sibling along with the pattern of steroid secretion observed in the propositi suggest that the so-called adult-onset CVAH is a mild form of the homozygous state of CVAH.",

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T1 - An attenuated form of congenital virilizing adrenal hyperplasia

AU - Rosenwaks, Zev

AU - Lee, Peter A.

AU - Jones, Georgeanna S.

AU - Migeon, Claude J.

AU - Wentz, Anne C.

PY - 1979

Y1 - 1979

N2 - The families of two patients with hirsutism and oligomenorrhea were studied with iv ACTH (Cortrosyn) stimulation. The parents responded with a combined incremental rise of progesterone and 17-hydroxyprogesterone greater than 6.5 ng/dl-min, a response seen in the heterozygote parents of patients with congenital virilizing adrenal hyperplasia (CVAH). One sibling in one family responded as do heterozygous subjects, while two other siblings responded normally. The abnormal response of the two pairs of parents and the sibling along with the pattern of steroid secretion observed in the propositi suggest that the so-called adult-onset CVAH is a mild form of the homozygous state of CVAH.

AB - The families of two patients with hirsutism and oligomenorrhea were studied with iv ACTH (Cortrosyn) stimulation. The parents responded with a combined incremental rise of progesterone and 17-hydroxyprogesterone greater than 6.5 ng/dl-min, a response seen in the heterozygote parents of patients with congenital virilizing adrenal hyperplasia (CVAH). One sibling in one family responded as do heterozygous subjects, while two other siblings responded normally. The abnormal response of the two pairs of parents and the sibling along with the pattern of steroid secretion observed in the propositi suggest that the so-called adult-onset CVAH is a mild form of the homozygous state of CVAH.

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An attenuated form of congenital virilizing adrenal hyperplasia

Abstract

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