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Abstract.

Anti-thyroid antibodies are frequently found in otherwise normal populations (4.5–25.8%); however, there is scanty information about thyroid function status in affected individuals. In this report, the serum concentrations of TSH, T₃, T₄, rT₃ and TBG and the titre of anti-thyroglobulin and anti-microsomal antibodies (haemagglutination technique) were studied in 520 healthy school children (260 boys and 260 girls) aged 6.0–17.9 years. Titres equal or greater than 1:16 of one or both antibodies were detected in 58 boys and in 77 girls (in 33 boys and in 24 girls with, and in 25 boys and 43 girls without, associated abnormalities in the serum concentrations of one or several hormones). The age distribution of thyroid antibodies followed a trimodal pattern with peaks at 7, 11 and 16–17 years in both sexes. The most striking finding was an abnormally elevated T₃ concentration in 22 boys and 5 girls with positive antibodies, with no symptoms of thyroid dysfunction and with no clear relationship with simultaneous abnormalities in TSH, T₄ or rT₃; however, in 5 boys the TBG serum levels were increased. Serum from these patients was incubated with [¹²⁵I]T₃ before free radioactivity was precipitated with dextran-coated charcoal and the aliquots were analyzed by paper electrophoresis. Serum samples with high T₃ levels bound significantly more radioactivity than normal or T₃-free serum (P < 0.001) and an abnormal peak of radioactivity was present in the gamma globulin fraction, in the former but not in the latter two types of sera. The presence of high serum T₃ levels in the absence of clinical symptoms of hyperthyroidism was probably due to sequestration of T₃ by the anti-thyroglobulin antibody, which may have cross-reactivity with T₃ and T₄, as has previously been demonstrated both in animals and humans.

Abstract.

A study of steroid metabolism by a tumour of the specific gonadal stroma was carried out in a 10 year old boy. Tumours developed in the two testes from multiple foci, and clinically, no signs of sexual development were evident. Four testicular enzymes necessary for testosterone biosynthesis were estimated in the child, in two adult controls, and in three pre-pubertal boys with male pseudohermaphroditism but normal tests of Leydig cell function. 17α-Hydroxylase and 17β-hydroxysteroid dehydrogenase were similar in the five controls and in the gonad with the tumour, while 17,20-desmolase and 3β-hydroxysteroid dehydrogenase were grossly deficient in the child with the tumour. These enzyme deficiencies might explain the absence of peripheral virilization in a boy with a tumour of Leydig and Sertoli cells.

An early diagnosis of Turner's syndrome is usually difficult, especially in cases with female-type chromatin. Hence the frequent occurrence of changes of the medial tibial condyle, resembling Blount's disease, deserves special attention.

Blount's disease belongs to the group of aseptic necrosis of the bone and cartilage (osteochondritis). The causes of the aseptic necrosis remain, however, still unknown. Some authors believe that trauma may be responsible for an arterial occlusion and impairment of blood supply with subsequent necrosis. The fact that the changes are bilateral and early in onset in infancy or childhood may also suggest that osteochondritis is some form of local disturbance of growth. According to its location, osteochondritis is called Legg-Perthes' disease, Scheuermann's disease, Köhler's disease, Osgood-Schlatter's disease – according to the names of the authors who first described the condition. The above mentioned diseases occur rather frequently in children and adolescents. The aseptic necrosis of the medial

ABSTRACT

A primary intracranial HCG-producing tumour was studied in an 8 year old boy with congenital adrenal hyperplasia. The case provided a unique opportunity to study the sequential changes in serum and urinary androgens and HCG as measured by radioreceptor assay for HCG and by radioimmunoassay for HCG using antisera raised against the hormone specific for the β subunit of HCG. Plasma concentrations of HCG, measured by the radioreceptor assay, closely correlated with the biologic activity of his tumour, as measured by serum testosterone concentration. This case demonstrates that precocious puberty in any child, including one with a known androgen disorder such as congenital adrenal hyperplasia, warrants thorough investigation.

ABSTRACT

Two women with normally developed secondary sex characteristics are reported. Both had spontaneous menstrual cycles, the first one during a period of 4 years, the second one started menstrual bleedings at the age of 17 and had menstrual cycles of regular intervals up to the present age of 39. She had given birth to a healthy boy at the age of 31. Both patients are chromatinnegative, of short stature and one has a unilateral webbed neck. Therefore they had to be classified as cases of Turner's syndrome. In cultures of bone marrow and skin fibroblasts the patient who has born a child was shown to have 45 chromosomes (2n + OX). The findings presented are in contrast to latest hypothesis of chromatinnegative Turner's syndrome according to which sex chromosomal anomaly XO leads to development of rudimentary ovaries.

ABSTRACT

Fasting serum C-peptide and total immunoreactive insulin (IRI) were determined in 38 non-diabetic children and adolescents 6–22 years old. C-peptide varied between 0.22–0.73 pmol/ml (mean ± sd, 0.45 ± 0.11). There was a tendency to higher values during puberty. No difference was found between subjects with or without a family history for diabetes. IRI varied between 0–31 μU/ml (mean ± sd, 11.3 ± 6.5).

The C-peptide response to glucagon was studied in 10 insulin dependent juvenile diabetics 11–19 years old, who had had measurable amounts of fasting C-peptide on some occasions during the previous years. Duration of diabetes varied between 4–12 years. A slight but significant rise in C-peptide level occurred in 3 patients. Their metabolic control estimated on the basis of daily urinalysis was "excellent" or "good". The results support the hypothesis that even trace remnants of the beta cell function may be of importance for the metabolic control in juvenile diabetes.

ABSTRACT

In 22 normal boys, 33 unilateral and 14 bilateral cryptorchids, a gonadal function test (2000 IU of HCG im each a day for three days and assays of plasma testosterone and plasma oestradiol-17β before and after the HCG administration) as well as an LH-RH test were carried out.

In 60% of the cases, both normal and cryptorchid boys, plasma oestradiol-17β (both in basal conditions and after stimulus) were found to be less than the sensitivity (5 pg/ml) of the method. While the plasma testosterone was similar under basal conditions in the three groups of children, after HCG it was significantly lower than the mean value of the control group only in the bilateral cryptorchids. The testosterone levels, both under basal conditions and after stimulus, are correlated to bone age only in the normal boys and in the unilateral cryptorchids. There were no significant differences among the various groups for either LH and FSH both under basal conditions and after LH-RH. The LH curve area during the LH-RH test is in correlation with bone age only in the normal children.

ABSTRACT

The excretion of 7 individual 17-oxosteroids and 7 individual corticosteroids in 24 h urine samples from 62 normal infants, children and adolescents, based on an accurate and specific paper chromatographic method for their separation and quantitation, is reported. The excretion of the 11-deoxy-17-oxosteroids gradually increases from 7 years of age and the increase becomes more rapid 2 or 3 years before the clinical signs of puberty appear. The rise continues throughout puberty and beyond it until the adult level is reached. The increase far exceeds that which would be accounted for by the growth of the individual. The increase in the excretion of the 11-oxy-17-oxosteroids with age is much more gradual. Androgens favour the formation of 5α metabolites and the 5α:5β ratio of the total 5α 17-oxosteroids and the total 5β 17-oxosteroids shows a statistically significant increase with age. In addition, a relatively high 5α:5β ratio is noted in male infants, which is likely to be related to their relatively high plasma testosterone levels. The excretion of the 17-hydroxycorticosteroids and the α-ketolic metabolites of cortisol gradually rises with age and correlates with body weight. The α-ketolic metabolites of corticosterone are relatively high in infancy, but after the age of 4 years their excretion also correlates with body weight. An increase in the 5α:5β ratio of allo-THF to THF is noted at puberty similar to that found with the 5α:5β ratios of the 17-oxosteroids.

ABSTRACT

Insulin and propranolol-glucagon stimulation tests were carried out on 28 children and 5 adolescents and the results of their growth hormone and plasma cortisol estimations were compared.

Twenty-nine subjects with normal growth hormone reserves showed a mean maximum rise of 17.4 μU/ml of serum growth hormone in the insulin test whereas the intramuscular injection of glucagon after oral premedication with propranolol produced a rise of 38.5 μU/ml. Five subjects with normal growth hormone reserves showed a reduced hormone output in the insulin stimulation tests but normal response in the propranolol-glucagon stimulation tests. Only one subject showed a poor response in the propranolol-glucagon but normal response in the insulin stimulation test.

In 30 subjects with normal adrenocortical function the mean maximum increase of plasma cortisol was 15.6 μU/ml in the insulin – and 14.9 μU/ml in the propranolol-glucagon stimulation tests, respectively. Both methods are suitable for studying the pituitary-adrenocortical interrelationships. The mechanism of the release of glucagon-induced growth hormone is not clear but the fall in blood glucose does not seem to play a major role in the process. A stress-like mechanism is equally unlikely because vegetative symptoms occurred only i a small number of subjects after intramuscular glucagon administration. It is possible that glucagon possesses a releasing-like mechanism which operates in the pituitary itself.

ABSTRACT

This study was carried out in order to determine whether children with a transitory type of growth hormone deficiency showed an accelerated growth in height velocity on treatment with human growth hormone (HGH).

Following careful diagnostic routine procedures 13 extremely short children were diagnosed as having isolated growth hormone deficiency, and were successfully treated with HGH. A true isolated growth hormone deficiency was present in 5 of the children, whereas 8 showed a normal increase in serum growth hormone on repeated growth hormone stimulation tests after their development of puberty and termination of HGH treatment. Three boys with bone ages of 5.5, 8.0 and 9.5 years showed an undisputable effect following HGH administration. They showed an initial growth at the start of treatment, and a second growth spurt during development of puberty. Two of the boys reached final statures of 14 cm taller than the predicted heights. The other patients, including the children with true isolated growth hormone deficiency showed an initial spurt of growth at the start of the HGH treatment immediately followed by a pubertal growth spurt. The mean acceleration of height velocity for the children with true isolated growth hormone deficiency was from 3.4 cm during the year before treatment to 7.0 cm during the first year on treatment, as compared to 2.8 and 7.4 cm, respectively, for the children with transitory growth hormone deficiency. A girl with severe anorexia nervosa who had a transitory growth hormone deficiency, showed an accelerated high velocity from 1.1 cm to 7.6 cm during the first year following treatment with HGH.

The question whether HGH treatment should be made available to all short children with no known syndrome, and presenting a height less than −3.5 sds, a bone age/chronological age ratio of less than ⅔, and a height velocity less than −2 sds is discussed. The only way to know if a child will respond to HGH treatment is to give it for a trial period of at least six months. At least a physiological stimulus to growth hormone secretion should be decisive in the selection of growth retarded children for HGH treatment. Different mechanisms seem to be responsible for physiological growth hormone secretion to sleep or exercise, and the secretion obtained with pharmacological stimuli.