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Olav Trygstad

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.

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Philippe E. Gamier, Jean-Louis Chaussain, Elisabeth Binet, Ariane Schlumberger, and Jean-Claude Job

ABSTRACT

Plasma gonadotrophins (LH and FSH) were radio-immunoassayed before and after injection of 0.1 mg/m2 of synthetic luteinizing hormone-releasing hormone (LH-RH) in infants 1 to 12 months old, prepubertal children aged more than 12 months, and pubertal subjects of both sexes. The pubertal changes of gonadotrophins include a highly significant increase of LH pituitary mobilizable reserve in both sexes, while the FSH reserve shows a significant decrease in females and no significant variation in males. From the first year of life up to childhood, the basal blood levels of FSH and LH decrease significantly in girls but do not vary in boys, while the FSH reserve decreases significantly in girls and increases significantly in boys, the LH reserve showing a non-significant decrease in both sexes. In the first year of life, girls show a very significantly higher FSH secretion and reserve than boys, while boys have a significantly higher LH reserve than girls. After the end of the first year up to the onset of puberty, the FSH reserve remains significantly higher in girls than in boys. The interpretation of these facts is discussed.

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Pierre Ferrier and Thérèse Lemarchand-Beraud

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

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L. A. Nilsson

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:

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Naphtali Brand, Ruwen A. Kathein, and Gideon Hasis

ABSTRACT

A parallelism in the incidence of human and bovine goitre in the northern part of Israel is described.

Among 1475 children and adolescents from 8–18 years of age of both sexes and examined from Upper Galilee an average incidence of goitre of more than 10% was found.

A three years follow up study showed an increase in percentage of goitre to 30% among girls. No cretins or deaf or dumb subjects were found.

Thyroid weights of 385 heads of cattle slaughtered at the slaughter-houses or on the farms were determined. While the mean weight of thyroids of cattle from a non-endemic control area was 17.7 g the mean weight for the goitrous area attained 23.4 g. The mean relative thyroid weights for the control and goitrous areas were 3.75 and 6.98 g/100 kg respectively. The histopathological examination of several excised non selected thyroids from Upper Galilee showed pathological findings: hyperplasia, fibrosis, nodular goitre.

These data are the first evidence of the incidence of endemic goitre of cattle in Israel and suggest most strongly that here the unfavourable environment plays a decisive role among the factors which influence the weight of bovine thyroids.

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G. Birke, E. Diczfalusy, L.-O. Plantin, H. Robbe, and A. Westman

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

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R. G. Edwards

The presentation made by Dr. Verbickij is important in several respects. First, it is an excellent piece of work on the immunology of pregnancy. Next, it shows how a model can be established in a non-human primate to facilitate research on clinical problems.

There has been a great amount of interest in the immunology of pregnancy in recent years. The stimulus to much of the early research was the development of methods to prevent rhesus haemolytic disease in human neonates. We are all familiar with the great progress being made in these studies following the introduction of the use of anti-Rh serum (Clarke 1968). There has been a significant reduction in the incidence of afflicted children, and this progress seems bound to continue as more experience is gained on the best ways of using the antiserum.

Yet there are still many cases of sensitised mothers carrying foetuses at risk of

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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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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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J. L. C. Ch'ng, A. Kaiser, J. Lynn, and G. F. Joplin

Abstract. Total parathyroidectomy is required to cure neonatal primary hyperparathyroidism (NPH) as any parathyroid remnant quickly becomes hyperplastic, causing recurrent hypercalcaemia. We present a patient with NPH who had total removal of his eutopic parathyroid glands but continued to have parathyroid hormone secretion from presumed ectopic parathyroid tissue. Hypercalcaemia initially recurred but normal calcium homeostasis was established as the child grew older. We postulate that the underlying defect in NPH is decreased sensitivity to the serum ionic calcium feedback inhibition at the parathyroid receptor level and that this sensitivity can improve with age.