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Biomedical subjects

J C Job

Publications and source records attributed to J C Job.

At least 91 records · Page 5Linked to original sources

Bioassay of growth-stimulating activity of serum: comparison of lymphocyte and cartilage assays in normal and growth hormone-deficient children.

The somatomedin and/or growth-stimulating activity of serum from hypopituitary children and short children with normal growth hormone (GH) response to stimulation tests were studied using different bioassays: thymidine incorporation into human activated lymphocytes; sulfate incorporation into chick embryo cartilage; and simultaneous thymidine uptake into the same cartilages. The results showed that lymphocyte assay is highly sensitive to small amounts of serum and is GH-dependent in children with low GH secretion. On the contrary, the cartilage assays need higher serum concentration and their GH-dependence appears only in subjects with normal or low-normal GH secretion. The lack of correlation between the results of the three bioassays suggests that they measure both somatomedins and different serum factors involved in the regulation of growth.

Adolescent↗

Comparison of growth hormone sleep release and responses to pharmacological tests.

The spontaneous release of growth hormone (GH) during nocturnal sleep was studied at age 5-19 years in 44 male and 15 female patients with severe growth retardation (-2.1 to -6.5 SD) among whom 43 were prepubertal and 16 pubertal. Comparison with the results of classical stimulation tests with ornithine, arginine and/or insulin showed good agreement in cases of classical hypopituitarism (n = 14) as in patients who seemed to be endocrinologically normal (n = 27). In 18 patients (31%) there was a discrepancy between sleep release and responses of GH to stimulation test: treatment with hGH was available in only 4 of these children and enhanced sharply their growth rate. It is suggested that a large span of intermediary situations exists between normal GH secretion and complete GH deficiency, deserving a controlled therapeutic trial with hGH.

Adolescent↗

Bromocriptine treatment in tall adolescents: two years of clinical experience.

34 adolescents referred for excessive height prediction (HP) (11 boys with HP greater than 196 cm, 23 girls with HP greater than 180 cm) were treated for 9-15 months with bromocriptine (5-7.5 mg/day). Minor and transient side effects were observed in 20% of the subjects at the beginning of the treatment. Treatment had to be stopped in 1 boy complaining of asthenia and headache. Puberty developed normally, 19 girls experienced menarche during treatment and 1 continued regular menses. Bromocriptine treatment induced: (1) a significant decrease (p less than 0.001) in growth velocity from (mean +/- SEM) 8.6 +/- 0.4 to 5.3 +/- 1.5 cm/year in boys and from 7.1 +/- 0.2 to 4.6 +/- 0.6 cm/year in girls; (2) a twofold mean increase in skeletal maturation rate. Adult HP was reduced significantly from 202 +/- 1.4 to 195.4 +/- 1.2 cm in boys, and from 184 +/- 0.7 to 179.8 +/- 0.7 in girls. These results confirm our previous report suggesting that bromocriptine is a valuable alternative to sex steroid treatment in order to limit the final height in excessively tall adolescents.

Adolescent↗

Free thyroxine measurement by one-step method in the plasma of treated and untreated hypothyroid infants.

The one-step radioimmunoassay of free thyroxine (f-T4) using a gamma-coated kit was used in this study. In controls the mean plasma levels (pmol/l) were 20.8 (range 13.5-37) during the first days of life, 17.4 (11.5-27) from 1 week to 1 year, and 17.0 (10-24.5) after 1 year. In preterm newborns it was correlated with the length of gestation. Among 23 untreated hypothyroid infants aged 15-22 days, f-T4 was undetectable in those with athyreosis, while in cases with dysgenetic thyroid it was variable, correlated to the width of the gland (r = 0.77, p less than 0.01). In 44 hypothyroid patients treated with l-T4, a highly significant positive correlation was found after the 1st month of treatment between plasma f-T4 and the daily l-T4 dose (r = 0.46, p less than 0.01), and a negative one between f-T4 and plasma TSH (r = -0.59, p less than 0.001). It is concluded that measurement of f-T4 offers a valuable means for control of diagnosis and treatment in congenital hypothyroidism, especially useful for avoiding both under- and overtreatment. Its correlations suggest that it is the most reliable hormonal measurement in the follow-up of thyroid children.

Dose-Response Relationship, Drug↗

Variations of plasma growth hormone (GH)-releasing factor levels during GH stimulation tests in children.

GH-releasing factor (GHRH) was measured by RIA in the plasma of 22 constitutionally short children given an ornithine infusion or an oral dose of L-dopa. After an overnight fast and 1 h of rest, plasma GHRH levels were 49.7 +/- 7.3 pg/ml (+/- SEM). In 5 children, L-dopa induced an increase in mean GH levels from 1.8 to 12 ng/ml at 60 min. Mean plasma GHRH levels increased from 47 pg/ml to a peak of 96 pg/ml at 15 min (P less than 0.02). In 4 other children, no increase in either GH or GHRH occurred after L-dopa treatment. In these 9 children, a significant correlation was found between the peak GH and GHRH concentrations (r = 0.841; P less than 0.001). On the contrary, ornithine-induced GH release was not preceded by a GHRH rise, but was followed by a GHRH decrease, from 51 to 27 pg/ml (P less than 0.02). We conclude that the 2 tests stimulate GH release in different ways, and that GH levels may be involved in the feedback control of GHRH secretion.

Adolescent↗

Effects of hypopituitarism and growth hormone replacement therapy on the production and utilization of glucose in childhood.

Glucose metabolism during fasting was investigated in 10 children aged 1.5 month-11.5 yr with deficiency of GH with or without other pituitary hormone deficiencies. After 10-16 h of fasting, mean plasma glucose was 56 +/- 4 (SEM) mg/dl, the result of decreased hepatic production of glucose (3.3 +/- 0.3 mg kg-1 min-1) insufficient to match glucose utilization (3.6 +/- 0.4 mg kg-1 min-1). The diminution of plasma glucose and of glucose production was similar whether ACTH deficiency was present (3.2 +/- mg kg-1 min-1) or not (3.5 +/- 0.6 mg kg-1 min-1). These results indicate that the lack of GH was the primary cause of hypoglycemia. Fasting plasma alanine (212 +/- 41 mumol/liter) and lactate (1222 +/- 136 mumol/liter), the main gluconeogenic substrates, were normal and did not correlate with the decrease of hepatic glucose release. Both plasma FFA (552 +/- 35 microM) and beta-hydroxybutyrate (654 +/- 158 microM) were in the low normal range, and neither correlated with the rate of glucose utilization. hGH replacement therapy resulted in a normalization of fasting plasma glucose concentration (78.5 +/- 6 mg/dl, P less than 0.005) and hepatic glucose production (6.1 +/- 1.2 mg kg-1 min-1). No significant changes occurred in the plasma concentrations of gluconeogenic or lipid substrates. These results, together with the known stimulatory effects of GH on carbohydrate-induced insulin secretion and storage of hepatic glycogen, suggest that the changes in glucose production in untreated and GH treated patients reflect the degree of hepatic glycogen replenishment.

Blood Glucose↗

[Normal male phenotype or hypospadias and female karyotype. XX male syndrome in children and adolescents].

A morphologically normal 46 XX karyotype has been found in 8 patients with male phenotype, either normal (3 cases) or hypospadiac (5 cases) studied at age 1 month to 15 years. Five had cryptorchidism. Pubertal follow-up was obtained in 6 patients and showed that they had hypogonadism with small testes, and a mean adult height of 163 cm. The hormonal investigations gave normal results before puberty, then after the onset of puberty a hypergonadotropic hypogonadism. Testicular biopsy showed alterations from age 8 years. Studies using Y-specific probes in 3 patients have shown the presence of Y genetic material in 2 and absence in 1, thus demonstrating genetic heterogeneity of the XX males.

Adolescent↗

[Tooth and face abnormalities associated with pituitary growth hormone insufficiency].

Dental and facial examination has been performed in sixty-two children with idiopathic or congenital growth hormone deficiency. Fourteen (22%) had a malformation of the upper incisors and/or of the naso-frontal bud or of the eyes, associated in five with a malformation of the brain in the prosencephalon-derived areas. Moreover, fourteen patients had some facial abnormality in an area situated near that derived from the naso-frontal bud. These associations are to be considered as a clinical marker able to call for pituitary investigation in short children. They suggest that some cases of so-called idiopathic hypopituitarism relate in fact to congenital and malformative causes.

Abnormalities, Multiple↗

Isolated menses in prepubertal girls.

Seventeen prepubertal girls 1 to 8 years of age were studied for the complaint of vaginal bleeding of apparent uterine origin. The bleeding was considered as isolated menses because it lasted two to five days and no other signs of sexual development or any detectable vaginal or uterine abnormalities were found. Eleven girls had two or more apparent menstrual periods, six experienced only one period. Height and bone age were not significantly different from normal. Laparoscopy or ultrasonography showed normal prepubertal uterine size, with either prepubertal ovaries or ovaries containing follicular cysts. Plasma gonadotrophins and their response to luteinizing hormone-releasing hormone were at prepubertal levels. Plasma estradiol level was significantly above the normal prepubertal range, suggesting transient ovarian activity and instability of the pituitary-gonadal axis in these girls. Isolated menses occurred mainly during the months of September to January, thus leading us to speculate about possible seasonal variations of hormonal regulation.

Age Determination by Skeleton↗

[Gynecomastia in boys before puberty].

Gynecomastia is seldom observed in prepubertal boys. Sixteen cases are reported in this work, 3 of them being related to a defect of 11 hydroxylase with virilizing adrenal hyperplasia and 13 being considered as idiopathic. These gynecomastias consist of a slight breast enlargement, often unilateral (10 of 16) and may spontaneously disappear after 3 to 12 months (5 of 16 cases). In the so-called idiopathic cases, the hormonal investigations performed gave normal results, so that no physiopathological explanation could be suggested.

Adrenal Glands↗

[Mixed gonadal dysgenesis. Apropos of a series of 21 cases].

Twenty-one cases of mixed gonadal dysgenesis referred at age 1 to 16 years are studied. External genitalia were in most cases of types III-IV, with a small penis and posterior hypospadias, asymmetrical genital folds containing an externalized testis on one side. The internal genitalia varied according to the degree of dysgenesis of the gonads, and included an uterus and/or a vagina in 18 among the 21 cases. A chromosomal mosaicism XO/XY or XX/XY was found in 11 patients, the other 10 having a normal 46 XY caryotype. Pubertal follow-up was obtained in 10 cases, and showed always a male sexual development, without possibility to exactly evaluate the function of the testis. Choosing the sex assignment is relatively easy in newborns or infants with mixed gonadal dysgenesis. It relies more on anatomy (size of corpora cavernosa, feasibility of urethroplasty or vaginoplasty) than on the results of hormonal measurements. The presence of an Y chromosome is not by itself an argument to choose the male sex. In most cases, the choice of the female sex is the easiest and relies on strong clinical arguments, but it leads unavoidably to suppress both the testis and the dysgenetic gonad.

Adolescent↗

[Effects of weakly androgenic anabolic steroids on growth in Turner's syndrome].

The effects of weakly androgenic steroids at minimal doses (norethandrolone 2.5 to 10 mg/day or methandienone 1 mg/day, administered 2 months every trimester) have been studied in 67 patients with Turner syndrome: 37 with 45 XO karyotype and 30 with mosaicism or partial X deletion. Mean bone age at the onset of treatment was 10 4/12 +/- 1 8/12 years, and the mean height retardation, adjusted to parents' height, was 3.7 +/- 1.2 standard deviations. Growth velocity was very significantly increased during the first 3 semesters of treatment. The ratio of bone age to height age, evaluated after 2 years of treatment, did not increase. The adult height reached by 37 treated patients was at the average 1.96 cm higher than that of a control group of 25 adult untreated Turner cases, this difference being not significant. The results varied greatly between individuals, without relation to the type of chromosomal abnormality or the kind and dose of steroid received. The psychological evolution of the treated patients was studied accordingly to their school and/or professional accomplishments. It was satisfactory in most of them. The anabolic treatment and/or the gain in growth velocity during treatment seemed to have positive psychological effect. No side-effects were noticed.

Body Height↗

[Solitary solid cold thyroid nodule in children and adolescents].

The present series includes 19 children and adolescents aged 8 to 15 years, 14 females (74%) and 5 males (26%), who underwent surgery because of solitary hypofunctioning solid thyroid mass. Microscopic examination of the mass was consistent with adenoma in 11 cases, carcinoma in 7 cases and thyroiditis in one case. Neither clinical examination nor isotopic and endocrinological investigations were predictive of the histological diagnosis. A cold nodule which is solid by ultrasonography and hypofixing by scintiscan requires, after observation for a few months, biopsy with histologic assessment during the operation.

Adenocarcinoma↗

[Effects of human growth hormone on very short children with intermediate responses to somatotropic stimulation tests].

The effects of treatment with human growth hormone (hGH) were studied in 29 patients aged 2 to 16 years with growth retardation between 2.2 and 6 standard deviations and insufficient growth velocity in whom repeated pharmacologic somatotropic stimulation tests had shown discordant responses either above 7 ng/ml, or lower than 6 ng/ml. An important acceleration of growth was obtained in 10 of 16 patients before puberty and in all the 13 cases treated at the beginning of puberty. No correlation was observed between the GH levels observed during the stimulation tests and the clinical results of treatment. These data lead to discuss partial GH deficiency and to propose a trial of treatment in very short children with low and/or dissociated responses to repeated tests of pituitary somatotropic secretion.

Adolescent↗

[Treatment of virilizing adrenal hyperplasia in adolescents. Use and side-effects of dexamethasone].

Dexamethasone (DXM) has been used to reduce clinical and/or biological hyperandrogenism in 18 adolescent patients treated by hydrocortisone and fludrocortisone for congenital virilizing adrenal hyperplasia (CAH). The doses and duration of treatment did vary among these patients. Androgen excess has been suppressed in all cases, even treated with low doses. Side-effects have been noticed in 9 patients, 5 having large purple striae, and 4 having mild and transient hypercortisolism. The analysis of data shows that DXM may be used for the treatment of CAH in pubertal patients provided that its use remains limited to cases in which the usual treatment does not allow to avoid the androgen excess. In such patients, the dose of DXM at the onset of treatment should be 0.25 mg given once a day, at night, and should not exceed 0.5 mg daily; it has to be adjusted according to clinical results, avoiding to obtain subnormal androgen levels. Hydrocortisone and/or fludrocortisone have to be prescribed for short periods in situations of stress, but not as permanent association. Within these limits, DXM may be considered as a valuable means to obtain hormonal adjustment in CAH adolescent patients.

17-alpha-Hydroxyprogesterone↗