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Biomedical subjects

J C Job

Publications and source records attributed to J C Job.

At least 145 records · Page 8Linked to original sources

[Epidemiology of hypothyroidism due to congenital malformation of the gland: athyreosis or ectopic thyroid (author's transl)].

The neonatal characteristics of 322 cases of hypothyroidism due to congenital malformation of the gland --athyreosis or ectopic thyroid--were compared with a national sample of births (N = 11,373), and a sample of births in public hospitals in Paris (N = 12, 895), in order to see if genetic factors were involved in the etiology of the disease. The hypothesis of multifactorial inheritance has been especially studied. Factors which appeared compatible with a multifactorial etiology were: marked excess of females (74%); higher frequency of twins (3.4%), of maternal familial thyropathy (9.2% among the mothers), of familial cases of thyroid malformations (11 cases); higher frequency of associated malformations of multifactorial origin (5.4%). But this type of malformation was not more frequent among siblings and relatives of the studied cases; it is thus not possible to accept the hypothesis of multifactorial inheritance without reservation.

Adult↗

[Serum somatomedin activity in children after surgery for craniopharyngioma (authors transl)].

Serum somatomedin activity was measured in 43 sera from 22 children aged 5 to 15 years operated on for a craniopharyngioma. All had a complete growth hormone deficiency and none received growth hormone therapy. At the time of the study, hydrocortisone therapy was given 29 times and thyroid powder 33 times. In these conditions, a highly significant correlation was found (r = 0.652, p less than 0.001) between somatomedin activity and growth velocity during the past three months. A negative correlation was also found with serum cortisol in patients who did not receive hydrocortisone (r = 0.654, P less than 0,05) and a positive correlation with serum concentrations of T4 (r = 0.640, p less than 0.05) and %3 (r = 0.956, p less than 0.001) in patients who did not receive thyroid powder. In contrast, no correlation was found with serum prolactin and insulin concentrations. These data establish than in children operated for craniopharyngioma, growth is related to a generation of somatomedin independent of growth hormone secretion, and closely controlled by endogenous secretions of cortisol and thyroid hormones.

Adolescent↗

Serum branched-chain amino acids in the diagnosis of hyperinsulinism in infancy.

Fasting values of branched-chain amino acids (valine, leucine, and isoleucine) were measured by column chromatography in the sera of 27 normal infants and children, 15 days to 9 years of age, 14 children with documented ketotic hypoglycemia one to 7 years of age, and in 14 sera from six infants, 15 days to 2 years of age, with documented hyperinsulinism. In normal children and those with ketotic hypoglycemia, each individual branched-chain amino acid and their sum were significantly negatively correlated with blood sugar values ranging between 11 and 92 mg/dl (P < 0.001). In infants with hyperinsulinism, branched-chain amino acid concentrations were significantly lower (P < 0.001) without correlation with blood sugar values ranging between 13 and 51 mg/dl, and plasma insulin concentrations (9 to 85 microU/ML). In all the children the sum of branched-chain amino acids was positively correlated with blood beta OH butyrate concentrations measured at the same time (r = 0.75, P < 0.001). The association of low blood sugar and low branched-chain amino acid concentrations during fasting seems characteristic of hyperinsulinism, and the measurement of branched-chain amino acids in these infants offers a physiologic indicator of the diagnosis of hyperinsulinism.

Amino Acids, Branched-Chain↗

Plasma gonadotropin and testosterone values in infants with cryptorchidism.

Plasma gonadotropins and testosterone levels have been studied from day 30 +/- 6 to day 120 +/- 10 in 57 term male infants born with undescended testes-bilaterally in 22 and unilaterally in 35. Clinical follow-up of these infants showed that spontaneous testicular migration occurred at 2 to 4 months in 27 of them; the 30 others remained cryptorchid at 6 months. Plasma LH and the postnatal rise in testosterone concentration were significantly lower in patients remaining cryptorchid, either unilaterally or bilaterally, than in infants with delayed spontaneous descent of one or both testes. A significant positive correlation was found betwen plasma LH and testosterone values within these two groups of subjects. Plasma FSH levels were not different in the two groups. These data suggest a primary LH deficiency in cryptorchidism, resulting in a blunted postnatal secretion of testosterone. It may be speculated that the early postnatal deficiency of the LH-Leydig cell axis in cryptorchid patients contributes to impair both testicular migration and maturation.

Cryptorchidism↗

A possible relationship between serum transferrin, growth hormone secretion and height velocity in children.

Since transferrin (Tf) in vitro has a growth-promoting activity and is associated with NSILA properties, the aim of this work was to study in vivo the relationships between Tf, somatomedin activity (SM), growth hormone (GH) secretion, and height velocity in children. An iv infusion of ornithine hydrochloride was given to 23 controls; the induced rise of GH was accompanied by a simultaneous fall of SM (r = -0.711, P less than 0.001) and was preceded by a fall of Tf (r = -0.610, P less than 0.01). In 17 obese children SM was within the normal range, when Tf levels were higher and arginine-induced GH peaks lower than in the controls, and a negative correlation was found between Tf basal levels and GH peaks (r = -0.608, P less than 0.01). In 9 children with confirmed hypopituitarism the Tf levels were significantly lower than in the controls. In 14 children with confirmed or suspected hypopituitarism a single im injection of hGH (6 mg) failed to induce Tf variations over 24 h. In 39 of these children the height velocity was significantly correlated with Tf basal levels (r = 0.701, P less than 0.001). These data suggest that transferrin is involved in growth regulation, and that GH secretion is related to transferrin levels by a feed-back mechanism.

Adolescent↗

[Transferrin, somatomedins and growth].

Plasma transferrin is significantly positively correlated to the growth velocity in normal children and children with various growth disorders. In hypopituitary dwarfs, both plasma transferrin and somatomedin are significantly lower than in controls. Acute variations of the plasma transferrin levels could be involved in the regulation of the GH secretion by a feedback mechanism. Yet, transferrin is neither a somatomedin or a somatomedin-binding protein, and the mechanisms relating growth to transferrin remain unknown.

Adolescent↗

[Early effects of intramuscular growth hormone on plasma somatomedin activity (author's transl)].

The early effects of intramuscular injection of human growth hormone (hGH) on plasma sulfation activity (Sm) have been followed for 72 hours: 1) after one injection of 6 mg in control children and in pituitary dwarfs (isolated idiopathic GH deficiency, multiple idiopathic pituitary deficiencies, and post-surgical or post-radiotherapic hypopituitarism); 2) after 6 injections of 1 mg every 12 hours in idiopathic pituitary dwarfs. Following injection of 6 mg, Sm decreases during 2-4 hours in all groups studied, then rises and peaks at 24 hours. The early decrease of Sm could relate to a rise of Sm-binding protein, as suggested by data obtained in the dog after intravenous injection of bovine GH. Following 6 injections of 1 mg, the rise of Sm is slower but higher and more protracted than with one injection of 6 mg. This fact suggests that the clinical effects of fractionation of treatment with HGH require further study. The lack of correlation between the biological data obtained and the clinical effects of hGH treatment upon the growth of pituitary dwarfs shows that the short-term hGH test used does not allow to foresee the effects of treatment.

Adolescent↗

Longitudinal study of plasma testosterone in male pseudohermaphrodites during early infancy.

Plasma testosterone was longitudinally studied during the first months of life in 7 XY infants with male pseudohermaphroditism. In two, the physiological postnatal rise of plasma testosterone was absent or blunted. A combined adrenal and testicular enzymatic defect was demonstrated in these two boys. In 5, a normal postnatal testosterone rise demonstrated a normal Leydig cell function. The longitudinal study of the physiologic postnatal rise of testosterone may be useful to distinguish secretory defects from responsiveness abnormalities thus improving the choice of gender in male pseudohermaphrodites.

Age Factors↗