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Biomedical subjects

J C Job

Publications and source records attributed to J C Job.

At least 271 records · Page 15Linked to original sources

[Plasma dehydroepiandrosterone concentrations in normal boys and in those with growth retardation].

Radioimmunoassay of plasma DHA in 179 boys 2 to 16 years old has allowed assessment of normal values (mean and 95% confidence limits) increasing with age on a log DHA/age relationship. Plasma DHA was normal in 58 cases of male idiopathic growth retardation and 40 boys with delayed adolescence. It was significantly increased in 42 obese boys. DHA levels were normal in 10 hypopituitary dwarfs with isolated GH deficiency, and significantly decreased in 24 others with multiple pituitary deficiencies. In pituitary dwarfism, a lowered DHA level may be a reliable index of ACTH deficiency, and may be of importance for evaluation of therapeutic programmes.

Adolescent↗

[Congenital adrenal hyperplasia due to blockade of 3-beta-hydroxysteroid dehydrogenase].

Five cases of congenital 3 beta-hydroxysteroid deshydrogenase deficiency in children are reported: four boys with perineal posterior hypospadias and one girl with clitoromegaly. The salt losing syndrome was clinically overt in only three patients. The main biological character was the very high level of plasma dehydroepiandrosterone (DHA) with an elevated DHA/delta 4 androstenedione ratio. The 17 alpha-OH progesterone, though in normal biosynthesis of glucocorticoids being produced beyond the enzymatic block, was raised, but this apparently paradoxical observation may assist making the diagnosis. Deficient production of testosterone was demonstrated in the prepubertal boys by absence of postnatal rise in plasma testosterone or a decreased reponse of plasma testosterone to chorionic gonadotrophin. It is concluded that deficiency of 3 beta-hydroxysteroid deshydrogenase, now easily recognizable with the use of plasma steroids radioimmunoassay, is probably less rare than was apparent with the use of urinary steroid estimations.

3-Hydroxysteroid Dehydrogenases↗

[Hypophyseal gonadotropin secretion in sexual precocity with McCune-Albright's syndrome].

In three young girls with isosexual precocity and fibrous dysplasia of bones, plasma gonadotropins (LH and FSH) response to releasing hormone (LH-RH) has been much under the mean response in true female precocious puberty. These data and those previously reported in the literature suggest that MacCune-Albright's syndrome correlates to an hypothalamo-pituitary dysfunction rather than to a true prematurity.

17-Hydroxycorticosteroids↗

[Evaluation of thyroid function in children (author's transl)].

Two main methods must be used in every case for evaluation of thyroid function in children : measurement of plasma thyroxin T4 and thyroid scanning with technetium. If accurately done, they may be sufficient for practical purposes. However, the wide number and variety of methods available for thyroid evaluation offers to pediatricians a choice sometimes difficult. The aim of this review is to summarize the meaning and accuracy of current methods and their usefulness in diagnosis and study of thyroid diseases of pediatric patients.

Adolescent↗

[Growth retardation in histiocytosis X. Evaluation of anterior pituitary function (author's transl)].

Among six patients with histiocytosis X of long duration and growth retardation of 3 to 4 standard deviations, three had a blunted growth hormone response to stimulation tests, associated to diabetes insipidus. In two of these three children there was a partial catchup of growth without treatment by human growth hormone. The causes and mechanisms of growth retardation in histiocytosis X, the influence of hydroelectrolytic disorders on growth in these patients and their need for treatment with human growth hormone are discussed.

Child↗

[Plasma androgens in boys from birth to adolescence (author's transl)].

Plasma testosterone, androstenedione, dehydroepiandrosterone and dehydroepiandrosterone-sulfate were measured by radioimmunoassay in 222 normal boys aged 1 hour to 18 years and 40 normal men aged 20 to 40 years. Three periods of high testosterone levels were observed in boys during extra-uterine life: first day (mean level 12 nmol/l), from the 12th to the 119th day (9 nmol/l) and from 13 years onwards in pubertal boys; the highest values were reached between 20 and 40 years (18 nmol/l). Androstenedione curve exhibited three coincident high concentrations phases: mean level at birth 8.7 nmol/l, and from 12th to 119th day 2.6 nmol/l. The lowest values were observed between 2 and 4 years; then a progressive increase occured up to adult age levels: 3.5 nmol/l. Dehydroepiandrosterone mean level was high at birth (20 nmol/l) then decreased slowly, either regularly or with a rebound during the 2nd month, a minimum being reached between 2 and 4 years (0.28 nmol/l). It increased thereafter exponentially up to adult levels reached after the age of 16 years (14 nmol/l). Dehydroepiandrosterone-sulfate mean level decreased regularly from birth to the second year, then its curve paralleled the dehydroepiandrosterone curve.

Adolescent↗

[Evaluation of risks related to human growth hormone (hGH) treatment. Results of an epidemiologic survey conducted in France of patients treated from 1959 to 1985].

Following the notification in the USA and England of four cases of Creutzfeldt-Jacob disease (MCJ) in patients previously treated with hGH, an epidemiological inquiry has been done in France to set up a clinical evaluation of all patients treated from 1959 to 1985. 1698 patients were registered for treatment. Current information (less than three months old) was obtained for 1622 patients (95.5%). Death was reported in 32 patients (2.0%), one is possibly related to a viral infection (malignant lymphoma), but none could be related to MCJ. Accidents were observed in 213 living patients (13.1%). Among them, 4 cases were classified as possibly related to a viral infection: acute lymphoid leukaemia, polyradiculoneuritis associated with hepatitis, acute encephalitis (2 cases). Even though the clinical symptomatology is not consistent with MCJ, a relationship with hGH therapy could not be completely excluded. Finally, six patients undertreatment developed malignancies. During the three last years, the question of side effects of hGH therapy has been raised in the literature two times running: risk of MCJ and risk of leukaemia. Then, the question of the long term vigilance of all treated patients with hGH deficiency should be done.

Creutzfeldt-Jakob Syndrome↗

[Obesity in infants. A series of 14 cases].

The infants aged less than two years, though often overweight, are seldom obese with a weight exceeding 2.5 SD above average for their height. This study includes 14 cases of obesity with early onset. The children were referred at age 17 +/- 5.7 months, with a mean height of +1.07 +/- 1.15 SD for age and a mean weight of +4.54 +/- 2.44 SD for height. They were reported as overfed, without any associated abnormality in their prenatal and postnatal history, and without emotional disturbances. In 8 cases, one of the parents was obese. For obtaining a weight decrease in these very young patients, a severely restricted diet has been necessary, with 50-60 cal/kg/day, corresponding in fact to 65-85 cal/kg of the average weight for height when the weight excess is 4 to 9 SD, and sometimes less, and hospital care. This diet, continued for 1 to 8 months, has allowed to reduce the weight excess at the level of +2.66 +/- 1.38 SD for height. It has been harmless, but not well accepted, so that in spite of the good results 10 of the 14 babies and mothers were lost for follow-up. The data show that true obesity is different from common weight excess in infants, is rare and very difficult to manage, and is poorly understood.

Body Height↗

[Plasma assay of somatocrinin during growth hormone stimulation tests. Results in pediatrics].

Somatocrinin (GHRH) levels were measured by RIA in the plasma of 41 children with constitutionally short stature. Basal plasma GHRH level was 51 +/- 10 pg/ml. L-Dopa induced a two fold increase in circulating GHRH levels 30 to 45 minutes before the elevation of GH. A positive correlation (p less than 0.005) was found between the peak of GH and GHRH levels during the dopaminergic stimulus. Conversely, the secretion of GH induced by amino-acids or clonidine was not preceded by an elevation of GHRH. These results suggest that the various stimulations of GH secretion used for investigations of short stature do not act in the same way at the hypothalamo-pituitary level.

Amino Acids↗

[Responses of somatotropin to stimuli after brief administration of estradiol in short children].

Ethinyl estradiol has been administered orally, 100 micrograms per day during three days, to enhance the growth hormone (GH) response to usual pharmacological stimuli. 102 prepubertal short patients aged 2 to 17 years with height between 2 to 6 SD below the mean, were studied. Human growth hormone (hGH) treatment was given only to those patients whose GH response was still below 10 ng/ml after estradiol. Under hGH treatment, their growth rate increased twofold, as much in patients with partial GH deficiency as in those with complete GH deficiency. It is concluded that the lack of GH response after estradiol priming contributes to the assessment of the indication for treatment with hGH. However, since it has not been possible to give this treatment to very short children whose GH response became normal after priming, this study does not allow to preclude the effect of hGH in such conditions. Thus estradiol priming must not be included among the practical criteria leading to therapeutic decision in doubtful cases.

Adolescent↗