The SIMULTANEOUS ASSAY OF CORTISOL AND 17α-Hydroxyprogesterone IN THE PLASMA OF PATIENTS WITH CONGENITAL ADRENAL HYPERPLASIA

Abstract

The simultaneous determination of cortisol and 17α-hydroxyprogesterone (17-OHP) was carried out on a single plasma sample (0.6 ml) by the competitive protein-binding technique after a steroid partition by water and carbon tetrachloride. This procedure was tested for sensitivity, accuracy, reproducibility and specificity. It was then applied to plasma samples obtained from 20 control subjects and 28 patients with 21-hydroxylase deficiency. The cortisol value in the control group was 9.3 μg/100 ml ± 0.7 (se). This was moderately decreased in the untreated patients (group I): 6.7 μg/100 ml ± 0.9 (se), markedly decreased in those who discontinued treatment for 3 to 15 days (group II): 1.5 μg/100 ml ± 0.3 (se) and was suppressed in the treated patients (group III). In normal children, the 17-OHP level was at the limit of sensitivity of the method. It was increased to 29 μg/100 ml ± 2.8 (se) in group I and was unchanged in group II: 26.5 μg/100 ml ± 3.4 (se). The plasma 17-OHP remained elevated in the patients who received an oral cortisol dose ≦ 20 mg/m2/ day and decreased to ≦ 3 μg/100 ml in those who were treated with a cortisol dose > 20 μg/m2/day. A 17-OHP response to ® and a 17-OHP diurnal rhythm were were observed in the untreated patients. In contrast, the patients who had temporarily stopped their treatment had no reaction. A negative correlation between the cortisol and ACTH levels appeared in groups I and II (N = 19; r = –0.66; P < 0.01) and a positive correlation was noted between the 17-OHP and urinary pregnanetriol values in all the patients (N = 33; r = 0.52; P < 0.01). The reported technique described is simple and contributes to the diagnosis of congenital adrenal hyperplasia; it can also be useful in the follow-up of treated patients.

Plasma Levels of Aldosterone, Corticosterone, 11-Deoxycorticosterone, Progesterone, 17-Hydroxyprogesterone, Cortisol, and Cortisone During Infancy and Childhood

“…Thp majority of these data that in only one report (23) were covering the entire pediatric age period, is in close agreement with those reported here with one exception (40) where, obviously due to a less specific method, considerably higher levels were found. Significantly higher 17-OHP levels in boys than in girls around the second month of life were observed in a large series of normal infants by Winter and coworkers (23,58) and more recently by Forest and Cathiard (17).…”

“…Even basal cortisol plasma levels, to date considered to change very little with age (52) and to be no different in children than in adults (24,40), were significantly lower in our normal infants between 7 days-3 months of age than both below and above this age range. This example already demonstrates the need for properly age-matched control data of plasma steroids in children.…”

“…Levels of 17-OHP have been studied most often simultaneously with P (I, 3.4, 25, 33-35, 54), but also individually (17,20,23,27,36) or in combination with other steroids (5,40,58). Thp majority of these data that in only one report (23) were covering the entire pediatric age period, is in close agreement with those reported here with one exception (40) where, obviously due to a less specific method, considerably higher levels were found.…”

Contribution of the Adrenal Gland to the Production of Androstenedione and Testosterone during the First Two Years of Life*

“…The antiserum used for androstenedione determinations cross-reacted 1.2% with testosterone and less than 0.1% with dihydrotestosterone and dehydroepiandrosterone. Plasma Cortisol was measured by the method of Pham-Huu-Trung et al (6). The interassay variability for a pooled plasma sample was 7.8% for testosterone (mean, 89 ng/dl), 8.2% for androstenedione (mean, 85 ng/dl), and 6.4% for Cortisol (mean, 11.2 /xg/dl).…”

Limitations of 17-hydroxyprogesterone in investigating neonatal hyponatraemia

“…In the majority of cases of 21-hydroxylase de¢ciency (partial or complete) in which plasma 17-OHP and cortisol concentrations have both been measured, the raised plasma 17-OHP concentration has generally been associated with low or`lown ormal' plasma cortisol. 7 An example of this from our own records is a 30-min Synacthen stimulation performed on a female neonate with partial 21-hydroxylase de¢ciency and of comparable age to the current case (12 weeks). Plasma 17-OHP concentration rose from 77 to more than 300 nmol/ L but the plasma cortisol only increased from 182 to 241 nmol/ L. In the acutely ill infant, it appears that simultaneous measurement of both plasma 17-OHP and cortisol could aid interpretation and prevent potential misdiagnosis leading to mismanagement, especially in male infants.…”