SummaryThe hGH response to an ornithin or an insulin test was measured in 105 children from 11-18 yr old with delay of growth more than 2 SD. Besides 74 subjects with normal values and 7 with complete lack of response, 24 subjects exhibited a,partial rise of GH. Most of the latter had decreasing growth rate and no sign of puberty. Out of 15 assessed for GH function after onset of puberty, 14 showed a normal response accompanying a markedly increased growth velocity. Four other subjects with partial response who were receiving sexual hormones for 48 hr before the second test showed a normal response also. An exogenous hGH treatment administered in two of these patients resulted in a marked and sustained growth increase far before entering puberty. SpeculationThe findings that plasma GH responses to stimulation tests is reduced in some children with decreasing growth rate before puberty and return to normal after onset of sexual development, would suggest a transient and functional defect in growth hormone secretion and the physiologic role of sexual hormones on GH release mechanism at that period of life.Among the children with short stature referred to our laboratory for somatotropic function investigation are a great number of boys with delayed puberty. Whereas most of these subjects show a normal response to the GH stimulation tests, a subnormal rise of GH has been observed in several cases.The influence of gonadal hormones on G H levels has been investigated by several authors (8,14). However, the precise role of pubertal development on G H secretion has not been clearly elucidated, as evidenced by the conflicting conclusions (1, 7,12,16). The present study was undertaken in order to assess hGH release in response to the ornithin test in prepubertal and pubertal subjects, and to study the relationship between sexual hormones and hGH secretion. MATERIALS AND METHODS MATERIALS105 subjects were studied, 25 girls and 80 boys, from 11-18 yr old, who exhibited isolated short stature. In all cases, height was below 2 SD for age, and in 50 cases, below 4 SD. No endocrine or metabolic disease was present, and psychosocial conditions were deemed to be noncontributory. In most cases, the following parameters were available: growth velocity, bone age, parental height, and stage of pubertal development assessed using Tanner's classification (19). The 105 subjects were submitted to hGH stimulation tests; 14 were given ornithin and insulin and 104 only ornithin. An informed consent was obtained from the parents before the tests.(62.5 g/l) was infused over 30 min. Blood samples were drawn at the end of the infusion and 15 min later. In 22 cases, additional blood samples were drawn I hr before, at the onset, and 1 hr after the infusion.Insulin tolerance test, (0.10 U/Kg) of insulin was administered iv. Blood samples for hGH and glucose determination were drawn at the following times: 0, 30, 60, 90, and 120 min after insulin. hGH levels were determined by radioimmunoassay (3). Normal values in our laboratory are defined as a p...

Plasma 21-deoxycortisol (21-DOF) and 17-hydroxyprogesterone (17-OHP) concentrations were assayed before (basal) and 1 h after ACTH stimulation in 4 groups of normal subjects (35 follicular phase women, 22 luteal phase women, 33 adult men, and 15 prepubertal children) and in a group of 31 patients with the late-onset form of congenital adrenal hyperplasia (LOCAH) due to 21-hydroxylase deficiency as well as in 31 LOCAH) heterozygotes. The mean basal plasma 21-DOF concentrations in each of the 4 groups of normal subjects were between 8 ng/dL (0.23 nmol/L) and 11 ng/dL (0.31 nmol/L), and they increased significantly after ACTH stimulation to between 36 ng/dL (1.04 nmol/L) and 44 ng/dL (1.27 nmol/L). There were no differences in basal or ACTH-stimulated plasma 21-DOF levels in these 4 groups, whereas their basal and post-ACTH plasma 17-OHP levels did vary. Among the LOCAH patients, 83.8% had basal plasma 21-DOF levels and 61.2% had basal plasma 17-OHP levels higher than the highest basal 21-DOF [30 ng/dL (0.86 nmol/L)] and 17-OHP [450 ng/dL (13.61 nmol/L)] concentrations in the normal subjects, and all individual 21-DOF and 17-OHP levels after ACTH stimulation [greater than or equal to 404 ng/dL (11.67 nmol/L) and greater than or equal to 1040 ng/dL (31.47 nmol/L), respectively] were markedly higher than the highest 21-DOF [76 ng/dL (2.19 nmol/L)] and 17-OHP [580 ng/dL (17.55 nmol/L)] levels in the normal subjects. The mean post-ACTH/basal plasma level ratios among the LOCAH patients were 19.75 for 21-DOF and 8.03 for 17-OHP. In LOCAH heterozygotes, basal 21-DOF values were higher than normal in 48.3%, and post-ACTH values were higher than normal in 93.5% of the cases. In contrast, basal plasma 17-OHP levels were similar in LOCAH heterozygotes and normal subjects, and only 16.1% of the LOCAH heterozygotes had post-ACTH plasma 17-OHP levels higher than the highest normal value. If sex and phase of the menstrual cycle are taken into account, along with the incremental responses (post-ACTH minus baseline value) of plasma 21-DOF and 17-OHP, to compare LOCAH heterozygotes and normal subjects, the discriminating power for detection of heterozygocity was somewhat increased for 21-DOF (to 100%) and appreciably increased for 17-OHP (to 30%).(ABSTRACT TRUNCATED AT 400 WORDS)

The di red radiuimmunoassa)• for plasma aldosll'ronc (P ,\) set up in this laboratory has hccn rc1wrtcd elscwcrc. Subjects were 115 normal full term nc,1 horns and infants. During the first

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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.

A radioimmunoassay for 21-deoxycortisol is described. The immunogen, 21-deoxycortisol-3-(O-car-Requests for reprints:

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