The development of steroidogenesis

Villee, Dorothy B.

doi:10.1016/0002-9343(72)90151-9

Cited by 57 publications

(13 citation statements)

References 51 publications

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“…By the 6th or 7th week of gestation, the provisional zones, the functional adrenal cortex of the fetus [6], secrete steroids. In affected females, elevated levels of circulating adrenal androgens interfere with vaginal and urethral canal separation, leading to a common urogenital sinus.…”

Section: Prenatal Developmentmentioning

confidence: 99%

Prenatal Diagnosis and Treatment of Congenital Adrenal Hyperplasia

Nimkarn

New

2006

Horm Res Paediatr

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Congenital adrenal hyperplasia is a group of inherited disorders caused by an enzyme deficiency in steroid biosynthesis. The most common form of congenital adrenal hyperplasia is 21-hydroxylase deficiency, which in its severe form can cause genital ambiguity in females. Steroid 21-hydroxylase deficiency can be diagnosed in utero through molecular genetic analysis of fetal DNA. Prenatal treatment successfully reduces genital ambiguity, and the subsequent problems of sex misassignment and gender confusion. Data from current studies show that prenatal diagnosis and treatment are safe for the mother and the fetus. The evidence also suggests that it is safe over the long term, but all subjects exposed to dexamethasone treatment during embryonic and fetal life should have their physical, cognitive and emotional developments recorded.

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Section: Prenatal Developmentmentioning

confidence: 99%

Prenatal Diagnosis and Treatment of Congenital Adrenal Hyperplasia

Nimkarn

New

2006

Horm Res Paediatr

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“…As data accrued, the presumed physiological model was altered to include the metabolic sequence depicted in Fig. 2 [4][5][6][7][8][9][10][11][12][13][14]A by oxidation with t-butyl chromate according to the method of Menini and Norymberski (18). Tracer purification methods have been described previously (16 …”

Section: Experimental Design and Tracer Administrationmentioning

confidence: 99%

“…We have studied a prepubertal boy with advanced feminization, but (5)(6)(7)(8)(9)(10); however, the level of expression of these enzymes diminishes rapidly after birth. Thus, the continued expression of the fetal levels of both the aromatase and sulfokinase enzyme activites after birth could give rise to increased estrogen formation from plasma prehormones and give rise to feminization such as that observed in this child.…”

Section: Introductionmentioning

confidence: 99%

Massive Extraglandular Aromatization of Plasma Androstenedione Resulting in Feminization of a Prepubertal Boy

Hemsell¹,

Edman²,

Marks³

et al. 1977

J. Clin. Invest.

100

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“…The adrenal cortex is formed in the 4 th week of gestation, functionally secreting steroids by the 6-7 th week of gestation [2]. Biosynthesis of the steroids: cortisol, aldosterone, and androgens from cholesterol occurs under the Adrenocorticotrophic Hormone (ACTH) stimulus with the involvement of five key enzymes : P450scc, 3β-OH dehydrogenase (3β-HSD), 17α -hydroxylase (17α-OH), 21-hydroxylase (21-OH), 11β-hydroxylase (11β-OH) [3,4] (Figure 1).…”

Section: Introductionmentioning

confidence: 99%

Congenital Adrenal Hyperplasia, the Origin of Combined Infertility: A Case Report and a Review of Literature

Ellenbogen¹

2014

Reprod Syst Sex Disord

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Congenital Adrenal Hyperplasia (CAH) -a complex and heterogeneous group of conditions is inherited as Autosomal Recessive (AR) disorders. The resultant deficiencies in one of the five enzymes involved in adrenal steroidogenesis lead to defects in the steroidogenic pathways and biosynthesis of cortisol, aldosterone and androgens. Precursor steroids proximal to the blocked step accumulate and can be shunted into other metabolic pathways, particularly that of androgen biosynthesis.CAH due to 21-Hydroxylase deficiency is traditionally separated into two clinical groups: the Classical form (C-CAH), which is further separated into salt-wasting (75%) and simple-virilizing (25%) phenotypes, and the Nonclassical form (NC-CAH). They are differentiated by their hormonal profile, predominant clinical features and age of appearance.CAH can affect fertility in females due to inadequate introitus, oligomenorrhea and elevated progesterone levels. Many authors reported an effect on male fertility as well.This editorial describes a case report of combined infertility due to mutations in the CYP21A2 gene and a review of literature on this subject.

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The development of steroidogenesis

Cited by 57 publications

References 51 publications

Prenatal Diagnosis and Treatment of Congenital Adrenal Hyperplasia

Prenatal Diagnosis and Treatment of Congenital Adrenal Hyperplasia

Massive Extraglandular Aromatization of Plasma Androstenedione Resulting in Feminization of a Prepubertal Boy

Congenital Adrenal Hyperplasia, the Origin of Combined Infertility: A Case Report and a Review of Literature

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