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D. Gupta and W. A. Marshall

ABSTRACT

A longitudinal study was made of the daily urinary excretion, on or near each birthday, of a number of C19 and C21 steroids in 9 healthy girls and 5 healthy boys aged 3 to 7 years. The amount of androsterone excreted by each individual increased slowly during the period of study but the absolute amounts varied greatly between individuals. The excretion of aetiocholanolone was greater than that of androsterone, contrary to reported findings in older children. Small amounts of DHA were found. Testosterone was found in only about 40% of samples; epitestosterone in 70 % and 11β-OH-androsterone in only 62 %. Cortisol metabolites were excreted in amounts which increased with age and all three metabolites of corticosterone were present in most specimens. 11-Deoxycortisol was found in about 50 % of the samples and THS in 63 %. The mean trend in the ratio of glucuronides to sulphates of the 11-deoxy-17-oxosteroids decreased with increasing age, but the 11-deoxy-11-oxy ratio of 17-oxosteroids increased as did the 5α/5β ratio of the C19 and C21 steroids.

No sex differences were observed. The excretion of cortisol metabolites showed a positive correlation with height and weight. 11-Deoxy-17-oxosteroids were positively correlated with the weight. No significant relationships between steroid excretion and skeletal maturity were found.

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M. S. Laurent de Angulo and H. H. van Gelderen

ABSTRACT

An attempt has been made to determine the value of the lysine-8-vasopressin test when used together with the insulin-induced hypoglycaemia and the metyrapone test for the differentiation between hypothalamic and pituitary secondary adrenocortical insufficiency. This study was carried out in 65 children and adolescents with various disorders associated with growth retardation or overweight. The criteria for normal responses to the tests were based on findings in a 'control' group consisting of children with short stature without endocrinological disease. These criteria have been discussed in detail.

In the control group, the concordance between the results of each of the tests was good. As far as the groups with proven or possible hypothalamic and/or pituitary disorders were concerned, discrepancies between the LVP test results and those of the ITT were found in a considerably higher proportion of cases. The combination of an abnormal ITT and a normal LVP test was encountered significantly more often than the reverse situation. This is in agreement with the hypothesis that LVP acts directly on the adenohypophysis while ITT acts at the hypothalamic or higher levels. The LVP test seems to be a valuable additionto the tests of hypothalamic-hypophyseal function.

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Paul Starr and John Nicoloff

ABSTRACT

Measurement of PBI values in healthy pre-adolescent Negro school children (N = 437) revealed a mean value of 6.4 ±.06 mg/100 ml while in a similar population of Caucasian children (N = 614) a normal mean value of 5.6 ±.07 mg/100 ml was observed. The difference had a P value <.001. Comparison of 131I T3 red cell uptake values in these two groups demonstrated a significant depression (P <.01) in the Negro children. TBG saturation capacities of Caucasian children were 24.7 ± 0.6 (S.E.M.) for ages 6 to 11 years and 21.3 ± 1.1 for ages 12 to 19 years which were not different from the adult values of 22.9 ± 0.8. In Negro children these values were 28.9 ± 0.7 for ages 6 to 11 and 25.4 ± 1.9 for ages 12 to 19 which were significantly different from their Caucasian counterparts. Adult Negro PBI values of 5.6 ± 0.25 and TBG of 24.5 ± 1.5 were not different from adult Caucasian values. Thyroxine-binding pre-albumin (TBPA) saturation capacities were significantly depressed in pre-adolescents (Caucasian 71 ± 7; Negro 69 ± 4) and adolescents (Caucasian 105 ± 13; Negro 109 ± 19) below the normal adult values but no racial difference was displayed. It is now apparent that racial origin must be a consideration in the evaluation of pre-adolescent and adolescent PBI and 131I T3 red cell uptake results and that TBPA and TBG capacities may normally alter during puberty in a manner independent of each other rather than reciprocally as has been recently proposed.

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M. Karp, A. Pertzelan, M. Doron, A. Kowadlo-Silbergeld, and Z. Laron

ABSTRACT

Lysine-8-vasopressin (LVP) was administered intramuscularly in a dose of 5–10 units to 20 children and adolescents, divided into the following 3 groups: I – normal controls; II – patients with isolated growth hormone deficiency; III – patients with pituitary insufficiency involving several hormones. In all the patients studied, LVP induced a rise in plasma 11-hydroxycorticosteroids. In the normal controls LVP induced a moderate rise in blood glucose. Patients with isolated growth hormone deficiency showed no rise, and those with pituitary insufficiency involving several hormones showed a great variability in response. In all 3 groups there was a sharp decrease in plasma free fatty acids, most marked in the patients with isolated growth hormone deficiency, and a slower return to base line levels. There was no change in the plasma concentration of growth hormone in any of the patients. Plasma insulin rose moderately in the normal controls; there was little or no change in any of the patients with pituitary insufficiency. The above finding that LVP induced a reduction in plasma free fatty acids in the patients with pituitary insufficiency without any concomitant elevation of plasma insulin and blood glucose, is in agreement with the assumption that LVP acts directly on adipose tissue.

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Peter Christiansen

ABSTRACT

The influence of ovine follicle stimulating hormone (NIH-FSH-S-3) on ovine luteinizing hormone (NIH-LH-S-8) in the Ventral Prostate Weight method (VPW) was studied. Adding of NIH-FSH-S-3 to NIH-LH-S-8 at ratios of 1:1, 4:1 and 10:1 gave no significantly higher responses than did NIH-LH-S-8 alone.

Urinary extracts from 2 women, hypophysectomized for metastasizing mammary carcinoma and from 3 children between 2 and 5 years old (1 boy, 2 girls) gave no positive response with the doses employed (1/4 of a 24 hours urine sample total per rat). It is concluded that the Ventral Prostate Weight method in hypophysectomized rats is specific for the assay of luteinizing hormone.

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Z. Laron, M. Karp, M. Nitzan, and A. Pertzelan

ABSTRACT

The insulin tolerance test (ITT) was performed in 102 children and adolescents belonging to the following 6 groups: controls (56 subjects), pituitary insufficiency (17 patients), delayed puberty (13 patients), obesity (6 patients), idiopathic precocious sexual development (6 patients) and congenital adrenal virilization (4 patients). All subjects showed hypoglycaemia. The mean percentage of maximal reduction in the blood sugar from the fasting concentration varied from 41 to 50 in all the groups. The control subjects and the patients with delayed puberty, obesity, precocious sexual development and congenital adrenal virilization showed an increase in plasma 11-hydroxycorticosteroids (11-OHCS) to a mean peak value ranging between 20 and 25 μg/100 ml, measured 60 min after the injection of insulin. From these results it was concluded that a rise in plasma 11-OHCS to a value of 20 μg/100 ml or more after hypoglycaemic stress denotes a normal corticotrophin releasing factor (CRF) and corticotrophin (ACTH) reserve. The patients with pituitary insufficiency showed 3 types of response independent of the degree of hypoglycaemia: nine patients showed a normal response, four had a medium response and four showed no response. There was a good correlation between lack of response of plasma 11-OHCS induced by hypoglycaemia and the urinary 17-OHCS response during the metopiron test, indicating that both these tests act at the hypothalamic level. Although the rise in plasma 11-OHCS after ITT was less marked than after the lysine vasopressin (LVP) test, it is suggested that the ITT is to be preferred as a clinical screening test, as it permits concomitant evaluation of both ACTH and growth hormone reserve. Another advantage of performing the ITT before the LVP test is that it allows of the diagnosis of a hypothalamic disturbance in ACTH secretion.

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Tohru Uozumi, Hisao Manabe, Hideyuki Tanaka, Yuji Hamanaka, Kiyoshi Kotoh, Keiichiro Suzuki, and Keishi Matsumoto

ABSTRACT

Urinary 17-hydroxycorticosteroids, 17-ketosteroids and 16α-hydroxydehydroepiandrosterone from eighty-six healthy subjects from the ages of 2 to 79 of both sexes were determined quantitatively by elution chromatography on cation exchange resin. Daily urinary excretions of 17-hydroxycorticosteroids and 17-ketosteroids increased from childhood to the ages of 15 to 40 and then decreased gradually with advancing ages over 60 to 79. The ratio of tetrahydrocortisone to tetrahydrocortisol decreased gradually from adolescence to old age. The ratios of 17-ketosteroids to 17-hydroxycorticosteroids and C19O2- 17-ketosteroids to total 17-ketosteroids were much higher between the ages of 20 and 40 than at other ages particularly in males. The ratios of aetiocholanolone to androsterone and tetrahydrocortisol to allo-tetrahydrocortisol seemed to be lower between the ages of 15 and 40 than at other ages. It was possible to determine the daily urinary excretions of 16α-hydroxydehydroepiandrosterone only in subjects over the age of 15, particularly between the ages of 20 and 40.

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L. Laurian, M. Herzberg, and L. Balas

ABSTRACT

The metopirone test was performed in 54 subjects: 49 overweight subjects and 5 controls. While normal in some of these, the test showed a poor reaction in a group of 14 overweight children, 12 of them being boys with a gynoid type of obesity.

It is suggested that the poor reaction to metopirone possibly represents only one aspect of reduced pituitary functional state – including gonadotrophins – and, if confirmed, these findings may support the concept of a functional Babinski-Fröhlich syndrome.

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C. C. Jensen and S.-I. Björklund

With the discovery of cortisone the adrenogenital syndrome with or without clinical signs of adrenocortical dysfunction, but always showing increased urinary 17-ketosteroids (17-KS) and genital symptoms, became capable of treatment (Wilkins et al., 1950). Although the etiology of the disease is still obscure, cumulative evidence suggests the occurrence of a hereditary factor. The underlying pathogenic mechanism is not clearly understood, but the modern trend is to ascribe the syndrome to a disturbance of the steroid metabolism rather than to an increased production of a single hormone.

This paper is concerned with (a) the excretion of 17-KS before, during and after cortisone therapy, by a boy in whom the adrenogenital syndrome was diagnosed at 14 days of age, and (b) the steroid pattern of the parents, an aspect that has apparently never before received attention.

CASE RECORD

The patient was an only child (birthweight 4250 gm.). The mother was 26 and

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Shaul Feldman and Aharon M. Cohen

There is growing evidence that the central nervous system, particularly the hypothalamic centres, have an important influence on the function of the endocrine glands and that pathological changes in the diencephalic region may cause aberration in their function (Harris, 1955, Benoit & Assenmacher, 1955, and Bauer, 1954).

The following is the report of a case of a disorder of the central nervous system manifested by mental deficiency and epilepsy associated with secondary hypogonadism and dysthyroidism.

CLINICAL HISTORY

The patient is an 18 year old male. The parents are not related. The patient and his only sibling, a 11 year old boy, are both mentally deficient. One uncle and 2 children of 2 different cousins have a speech defect and are also mentally retarded.

The patient's birth was normal. Until the age of 4 he had no bladder control. At the age of 5 he started to talk and it became