Plasma 17-Hydroxyprogesterone, 21-Deoxycortisol and Cortisol in Congenital Adrenal Hyperplasia*

Franks, Robert C.

doi:10.1210/jcem-39-6-1099

Cited by 51 publications

(15 citation statements)

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“…17-hydroxyprogesterone (17-OHP) can be measured in peripheral blood by competitive protein-binding (3, 6,9,11,14,16) or by radioimmunoassay (2, 5,7,18). The binding agent used in the competitive protein-binding assay was corticoid-binding globulin; for radioimmunoassay the agent used may be a ho mologous antiserum raised against 17-OHP-protein conjugate (7,18), or as de scribed here, a heterologous antiserum raised against ll-desoxycortisol-2 1 -hemisuccinate-bovine serum albumin (BSA) (2, 5), which shows complete reac tivity towards 17-OHP.…”

Section: Introductionmentioning

confidence: 99%

Radioimmunoassay of 17-Hydroxyprogesterone in Serum with or without Thin-Layer Chromatographic Purification

Wickings¹

1975

Horm Res

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A radioimmunoassay for the measurement of 17-hydroxyprogesterone (17-OHP) is described. The antigen ll-desoxycortisol-21-hemisuccinate-bovine serum albumin has been used to produce two antisera of different antibody populations in the same animal. The thin-layer chromatographic system described can be used to separate all the cross-reacting steroids investigated from 17-OHP. A simplified method is also presented using preliminary solvent extraction only. The mean 17-OHP levels measured, using this method, were, for normal men, 0.86 ± 0.19 ng/ml (SD), for normal females 0.30 ± 0.19 ng/ml in the follicular phase and 1.72 ± 0.18 ng/ml in the luteal phase of the menstrual cycle, and 0.45 ± 0.17 ng/ml in normal postmenopausal women.

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Section: Introductionmentioning

confidence: 99%

Radioimmunoassay of 17-Hydroxyprogesterone in Serum with or without Thin-Layer Chromatographic Purification

Wickings¹

1975

Horm Res

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“…The serum cocncentrations of 21-DOF, 21-DOE, A4-A and 17-OHP tended to in crease as time passed, the increases in 21-DOE and 17-OHP being especially large. Concerning 21-DOF values in untreated infants with 21-OH deficiency, Franks [6] has reported that they were 112 ± 45 ng/ml (323 ± 130 nmol/1) for the sodium-losing form and 76 ± 34 ng/ml (219 + 98 nmol/1) for the simple virilizing form. Loriaux et al [22] and Gueux et al [23] have reported lower lev els of 21-DOF (27.7 ± 11.7 ng/ml and 22.9 ± 10.4 ng/ml, respectively) in treated patients with 21-OH defi ciency.…”

Section: Discussionmentioning

confidence: 99%

“…Radioimmunoassays (RIA) have been used to detect 17a-hydroxyprogesterone (17-OHP) and deoxycortisol (S), and to examine the changes in the levels of cortisol (F), 17-OHP and 21-deoxycortisol (21-DOF) [6] before and after treatment of the disease. However, no report on the changes of a number of adrenal steroids in the early neonatal stage of 21-OH deficiency before and after the initiation of therapy has appeared in the liter ature.…”

Section: Introductionmentioning

confidence: 99%

Changes of Several Adrenal Δ<sup>4</sup>-Steroids Measured by HPLC-UV Spectrometry in Neonatal Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Saisho

Shimozawa²,

Yata³

1990

Horm Res

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We have developed an easy and rapid method of reverse-phase high-performance liquid chromatography (HPLC)-UV spectrometry for measuring adrenal Δ⁴-steroids. Three female neonates with adrenal 21-hydroxylase deficiency (2 salt-losers and 1 simple virilizer), two of whom were recalled by neonatal mass-screening for congenital adrenal hyperplasia (CAH), were diagnosed using this method. Changes of several adrenal steroids were examined in these patients before and after treatment with hydrocortisone. Before treatment, the cortisone and cortisol peaks were very low and those of 17α-hyroxyprogesterone (17-OHP) and 21-deoxycortisol (21-DOF) were high in all 3 patients (17-OHP: 79.9-997 nmol/l, 21-DOF: 83.7-324 nmol/l). The androstenedione peak was also high in 2 of them. A peak produced by 21-deoxycortisone, which is a product of oxidation of 21-DOF at the C-11 position, was also detected in all cases (14.5-297 nmol/l). After treatment, all of these abnormally elevated Δ⁴-steroids decreased or disappeared. This new method is thought to be valuable for the rapid diagnosis of CAH, and especially for use in neonatal mass-screening for CAH.

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“…Since, in infants below two months old, the normal concentrations of these steroids decreased consecutively according to the equivalent week of the gestational age, the sampling ages of the neonates and infants with 21-OH deficiency were also represented by this expression. (Franks, 1972;Franks, 1974;Hughes and Winter, 1976;Lippe et al,, 1974). However, there have been no reports dealing with changes in serum 17-OH-pregnenolone and its sulfate levels in both untreated and treated patients with 21-OH deficiency, except for several papers only describing the concentrations of 17-OH-pregnenolone in plasma (McKenna et al, 1976) and amniotic fluid (Pang et al, 1980;Tulchinsky, 1977).…”

Section: Methodsmentioning

confidence: 99%

Serum 17-Hydroxypregnenolone and 17-Hydroxypregnenolone Sulfate Concentrations in Patients with Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.

et al. 1988

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Serum concentrations of 17-hydroxypregnenolone, 17-hydroxypregnenolone sulfate and 17-hydroxyprogesterone were measured simultaneously in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, using a combined radioimmunoassay method. All these precursor steroids were found to be markedly elevated in the sera of untreated patients with a salt-losing form of the disease, whereas, in untreated patients with a simple virilizing form, only the concentration of unconjugated steroids was increased and the 17-hydroxypregnenolone sulfate concentration remained within the normal range.Among the patients with a salt-losing form under maintenance therapy, these steroids were all still significantly increased in those on insufficient control, whereas only 17-hydroxyprogesterone was significantly but slightly increased in those on adequate control.Although the mechanism whereby the serum 17-hydroxypregnenolone sulfate concentration is not increased in the untreated simple virilizers is unknown, both a milder degree of 21-hydroxylase deficiency and a role of 17-hydroxypregnenolone sulfate in adrenal steroid production as a kind of supplier are suggested as possible explanations, especially in the neonatal period and early infancy. Thus, this study showed the serum concentrations of 17-hydroxypregnenolone and its sulfate together with 17-hydroxyprogesterone in patients with 21-hydroxylase deficiency in various conditions. It is widely recognized that 17-hydroxyprogesterone (17-OH-progesterone) which is a substrate for adrenal 21-hydroxylase is markedly increased in the serum of untreated and insufficiently controlled patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OH deficiency). Therefore, the measurement of 17-OH-progesterone has been applied to diagnosis and the evaluation of manage-

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Plasma 17-Hydroxyprogesterone, 21-Deoxycortisol and Cortisol in Congenital Adrenal Hyperplasia*

Cited by 51 publications

References 0 publications

Radioimmunoassay of 17-Hydroxyprogesterone in Serum with or without Thin-Layer Chromatographic Purification

Radioimmunoassay of 17-Hydroxyprogesterone in Serum with or without Thin-Layer Chromatographic Purification

Changes of Several Adrenal Δ<sup>4</sup>-Steroids Measured by HPLC-UV Spectrometry in Neonatal Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Serum 17-Hydroxypregnenolone and 17-Hydroxypregnenolone Sulfate Concentrations in Patients with Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.

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Plasma 17-Hydroxyprogesterone, 21-Deoxycortisol and Cortisol in Congenital Adrenal Hyperplasia*

Cited by 51 publications

References 0 publications

Radioimmunoassay of 17-Hydroxyprogesterone in Serum with or without Thin-Layer Chromatographic Purification

Radioimmunoassay of 17-Hydroxyprogesterone in Serum with or without Thin-Layer Chromatographic Purification

Changes of Several Adrenal &Delta;<sup>4</sup>-Steroids Measured by HPLC-UV Spectrometry in Neonatal Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Serum 17-Hydroxypregnenolone and 17-Hydroxypregnenolone Sulfate Concentrations in Patients with Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.

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Changes of Several Adrenal Δ<sup>4</sup>-Steroids Measured by HPLC-UV Spectrometry in Neonatal Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency