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ABSTRACT

In a series of 146 school girls and nurses in training, the serum levels of growth hormone (GH), follicle stimulating hormone (FSH), and luteinizing hormone (LH) were determined in the afternoon after 4–5 hours' fast. Blood specimens of the menstruating girls were taken on the 7th to 10th day from the beginning of the preceding menstrual bleeding. From the 24-hour urines collected on the previous day the excretion of total oestrogens, 17-ketosteroids and 17-hydrocorticosteroids was determined. The series was grouped according to skeletal age which varied from 8.0 years to the adult stage.

In the girls aged 11-13 years the GH curve showed a significant rise parallel with the mean height curve. The FSH values at the early age of 8–9 years corresponded to the follicular phase of adult women. The LH level increased steeply up to the 15th year of bone age and the mean values of adults were found to be about three times those of childhood. The excretion of total oestrogens and 17-ketosteroids increased steadily up to the full maturity of skeletal age. In the oldest group of girls the excretion was about four times that of the group aged 8–9 years. The hormonal maturation seems to continue until late puberty. The 17-OHCS, on the other hand, reached the adult level in the group with a bone age of 13 years. The excretion of 17-OHCS shows a steep rise from the age of 11 to 12 years and this acceleration of excretion perhaps exceeds the increase in surface area.

Very little is known yet about thyroid hormone transport capacity of the serum and thyroid hormone protein binding in children. Except for the studies by Haddad (1) and by Dreyer and Man (2), all observations so far published are concerned with hormone transport mechanisms in the adult. In order to establish reference values, thyroid function tests were performed in 35 eumetabolic children (20 boys and 15 girls) aged from 6 weeks to 11 years. In vitro erythrocyte uptake of T³ was measured according to the procedure of Hamolsky et al. (3). Protein binding of T⁴ was studied at progressive degrees of saturation by paper electrophoresis in tris-maleate buffer at pH 8.6 according to Ingbar et al. (4). Values were compared with those from a group of 21 euthyroid adults, tested in the same laboratory. PBI was found to be higher in children than in adults. This tendency has been noted

Two infants (pat. 2 and 3) with a salt-losing syndrome were studied. They were 5 and 15 weeks old on admission in the hospital. The 4 parents of these infants were all related. Another child in the same family, born in 1950 (pat. 1), was seen 3 weeks old in the hospital with the same clinical syndrome. The presenting clinical symptoms in these children were: dehydration, poor feeding, occasional vomiting and intermittent febrile temperature. Serum sodium was low, serum potassium elevated. All cases demonstrated a good response to desoxycorticosterone acetate (DOCA). External genitalia were normal; urinary 17-ketosteroid excretion was normal. Pat. 1 is now a well developed 13 year old girl; DOCA therapy was discontinued at 1 year of age, she is still using a salt rich diet. Pat. 3 is doing well sofar on DOCA therapy. Pat. 2 died unfortunately by an intercurrent viral infection.

Six generations in this

Cytologic aspiration biopsy of the thyroid was used in 48 of 63 children being the material of juvenile atoxic goitre (aged 10–15 years) from the last 31/2 years in Gothenburg. Cytologic signs of lymphoid thyroiditis were found in 23 girls and 3 boys.

Clinical signs and symptoms in 35 children with auto-immune thyroiditis are reported. One third had no complaints apart from the goitre, 12 were more or less hypothyroid and the rest had chiefly mild and diffuse symptoms. Increased firmness of the goitre was found in 27 of 32 cases, the surface was nodular or bosselated in 15.

Thyroid function: PBI was low only in 3 cases with myxedema, was above the upper normal limit in several cases. The difference between the PBI- and the BEI-levels was disproportionately great in most cases examined. The thyroid uptake of I¹³¹ showed abnormal discharge of iodide in every fourth patient.

Thyroid antibodies:

This girl had precocious menstruation for two years before investigation in the hospital at the age of 5 years. There was no breast development although the nipples were enlarged, and only a few pigmented pubic hairs were present. The cell nuclei showed 47 chromosomes with trisomy for 21. The sella turcica was enlarged. A serum protein bound iodine was obtained, although there were no signs of hypothyroidism. Before the result of the protein bound iodine had been obtained two injections of medroxyprogesterone acetate were given (200 mgr. intramuscularly). The serum protein bound iodine was 2.3 μg./100 ml. and when she was re-admitted to the hospital for assessment of the results she was frankly myxoedematous with a protein bound iodine level of 0.9 μg./100 ml. The endocrine assays are reported and it is suggested that this child was suffering from unrecognised hypothyroidism which, with pituitary overlap, produced an increased secretion

Congenital hyperplasia of the adrenal cortex is manifested in boys as macrogenitosomia praecox and in girls as female pseudohermaphroditism, with or without concomitant adrenocortical insufficiency.

Some tendency to familial occurrence of congenital adrenocortical hyperplasia has previously been observed. According to a survey by Bentinck et al. (1952) a total of 97 cases of familial somatosexual aberrations affecting 42 families were reported between 1852 and 1952. Eighty of these children were either definitely or probably female pseudohermaphrodites and 17 had either demonstrable or probable macrogenitosomia praecox. In no family, however. was it possible to demonstrate virilizing adrenocortical hyperplasia in more than one generation. Wilkins et al. (1950, 1955) have accordingly assumed that congenital hyperplasia of the adrenal cortex is due to a recessive hereditary genital defect, clinically manifest only in homozygotes.

In this paper a description will be given of the clinical findings in a family with six children, including three

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.

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.

ABSTRACT

A standard LH-RH test (50 μg/m²) given iv was carried out in 65 normal girls, 42 of them pre-pubertal aged from 47/12 to 11 years and 23 in the early stage of puberty, aged from 9 to 129/12 years.

The results indicate that in pre-pubertal girls the basal levels of the plasma gonadotrophins remain steady (LH 0.6 ± 0.1 mIU/ml; FSH 0.8 ± 0.1 mIU/ml, m ± sd) and that there is a small but significant response of LH to LH-RH (1.6 ± 0.2 mIU/ml). During this period the FSH response to LH-RH is very marked (8.0 ± 1.0 mIU/ml) with a gradual, significant decrease seen towards the onset of puberty (6.5 ± 0.9 mIU/ml, P < 0.001). These results support earlier reports that the LH-RH test is a useful tool to evaluate the secretion of pituitary LH and FSH in early childhood.

Abstract. Current medical and surgical therapies of precocious puberty in McCune-Albright syndrome are often unsatisfactory. We used an aromatase inhibitor, testolactone, to treat precocious puberty in a girl with McCune-Albright syndrome. This child was unresponsive to 28 weeks of treatment with the long-acting agonist of LRH, D-trp⁶-pro⁹-NEt-LRH. During testolactone therapy, menses ceased, bone age advancement and height velocity diminished, and plasma oestradiol levels were suppressed. Serum gonadotrophin levels remained in the prepubertal range. Testolactone may be an effective therapy of precocious puberty in girls with McCune-Albright syndrome.