Plasma somatomedin activity measured as thymidine factor in normal children with average and constitutionally tall stature.
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Biomedical subjects
Publications and source records attributed to J C Job.
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An ornithine infusion test was performed in fifty-four children with constitutional short stature. The ornithine infusion induced an elevated level of GH at 45 min (mean value = 873 pmol/l) and a similar rise of cortisol levels (mean value = 544 nmol/l). An important peak of ACTH appeared 15 min before the increase of cortisol. In three panhypopituitary dwarfs studied, no elevation of GH or cortisol was observed. The well tolerated ornithine infusion test allows the simultaneous study of the somatotropic and corticotropic pituitary secretions in children with delayed growth.
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The age-related variations in the growth-promoting activity of human plasma have been studied from birth (cord blood) to adulthood using a bioassay which measures the incorporation of tritiated thymidine into lectin-activated human lymphocytes. Cord blood values were low (0.69 +/- 0.004 U/ml). A definite increase was found at 5 days of age, correlating with the level at birth. Higher levels were attained after 1 month of age, with a 2-fold increase during the first months of life. Lower values were found in children 1-10 yr old, and high levels were found during puberty. This pattern, different from those of sulfation activity and plasma somatomedins suggests that factors other than somatomedins may be involved in growth stimulation during the first year of life in humans.
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Two adolescents with chronic adrenal insufficiency, presumably secondary to the cytomegalic type of congenital adrenal hypoplasia, developed a gonadotropin deficiency at puberty, which was both delayed and incomplete. Endocrine studies demonstrated in plasma low LH levels which failed to increase after LH-RH, higher levels of FSH, low basal testosterone concentration but increasing normally after HCG stimulation. Azoospermia was demonstrated in one case. In parallel with those previously reported, these two new cases show that the association between gonadotropin deficiency and congenital adrenal hypoplasia is not accidental, even if the relationship between the two components of the syndrome is still unclear.
Delayed puberty is defined as the total or almost total absence of development of sex characteristics at an age exceeding the mean by two standard deviations: approximately 15 years in boys and 13 years in girls. A double sense has been given to this term, which is usually applied to permanent hypogonadism as well as to normal but delayed maturation. In practice, certain signs can assist formulation of a prognosis, and if necessary the type of therapy required. Data on which can be based the diagnosis, surveillance, and medical conduct in sexually immature adolescents are discussed. Their interpretation is simple when elevated gonadotrophins levels indicate a primary gonadal lesion, or when the delayed puberty results from a general disease disturbing body maturation. In other cases, interpretation of data is often very difficult and is sometimes a very slow process. Because of these difficulties, the diagnosis between simple delay and gonadotrophic insufficiency is discussed in this report on the basis of a retrospective study conducted for a long enough period to have arrived at a definite conclusion.
A study of 98 cases of true sexual precocity in girls shows a frequency three times higher between 5 and 7 years than before age 5. Detectable causes (intracranial lesion and Mac Cune-Albright syndrome) were found in 56% of cases before 5 years and only 18% after age 5. The plasma levels of estradiol and gonadotropins and the gonadotropins response to LHRH did not differ significantly from those found at the corresponding stages of normal puberty. Plasma dehydroepiandrosterone was lower. Pelvic ultrasonography sometimes discovered follicular cysts. 44 patients treated with medroxyprogesterone acetate were followed for one or several years. Results on sexual development and plasma estradiol were good in most of them, with individual variations, Improvement in the ratio of bone age as height age and in the expected adult height was obtained but without statistical significance.
A case of goiter in a 8 year-old child is reported. It resulted from a protracted treatment with lithium at high daily dose. The blood level of lithium was very high. Blood iodine and thyroid hormones were normal. This fact points out the importance of careful anamnestic investigation.
In a TSH screening program for congenital hypothyroidism we detected seven newborn infants with normal plasma T4 and T3 levels but high immunoassayable TSH. Similar findings were obtained in their mothers. Serial plasma dilution curves, with and without the addition of normal rabbit serum to the samples, showed that the result of TSH assay performed with antihuman TSH rabbit antiserum was falsely elevated in mothers and infants by an interfering factor. Follow-up of the infants demonstrated that the falsely elevated plasma TSH levels returned to normal within the first 6 months of life. On the contrary, plasma TSH levels remained high in the mothers. These results suggested a placental transfer of maternal antibodies. Indeed, the analysis of the mothers anamnesis revealed that all had previously received injections of a microbial vaccine cultured on a rabbit lung-containing medium. We conclude that placental transfer of a maternal antirabbit factor may cause an artefactual hyperthyrotropinemia in the newborn and the incorrect diagnosis of neonatal hypothyroidism. This can be avoided by the addition of normal rabbit serum or immunoglobulin to the TSH RIA tubes.
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14 cases of hypopituitarism associated with mid-line defects are reported: 7 with septo-optic dysplasia, 5 with agenesis of corpus callosum and septum pellucidum without optical lesion (2 with cleft palate), 1 with familial pituitary aplasia and 1 with mediofrontal cutaneous aplasia. The most striking features in these patients are: precocious signs of pituitary deficiency, mainly hypoglycemia; micropenis and cryptorchidism in males; decrease of growth velocity 2 months to 6 years after birth. Neuroradiological investigations, evaluation of somatotropic and corticotropic secretions with glucagon test, and evaluation of thyrotropin and prolactin secretion with thyroliberin test, offer in the youngest patients the best way to precocious diagnosis and treatment.