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Biomedical subjects

P Canlorbe

Publications and source records attributed to P Canlorbe.

16 recordsLinked to original sources

Endocrine studies in male pseudohermaphroditism in childhood and adolescence.

The clinical and endocrine features of fifty cases of male pseudohermaphroditism and XY karyotype aged from 6 months to 20 years have been studied. Thirty-two subjects were pre-pubertal and eighteen, of whom ten developed gynaecomastia, were pubertal. A definite aetiology was established in 12%. 4% had deficient testosterone biosynthesis and 8% mixed gonadal dysgenesis. In the remaining 88% an aetiology of androgen unresponsiveness at the target areas is suggested. Hormonal investigations in these subjects showed that before puberty basal plasma testosterone and oestradiol were slightly but significantly elevated, whereas testosterone response to stimulation with human chorionic gonadotrophin was significantly diminished. In the pubertal subjects basal plasma testosterone, oestradiol, serum binding capacity of testosterone-oestradiol binding globulin and basal plasma LH were significantly elevated. These hormonal features in pubertal or post-pubertal male pseudohermaphrodites appear to be characteristic of androgen unresponsiveness. The presence in addition of elevated basal plasma testosterone and oestradiol in the pre-pubertal subjects suggest that some of these findings may be presented from early childhood.

Adolescent

Hypothalamo-pituitary-gonadal function in male central precocious puberty.

Eleven boys aged 1-10 years with central precocious puberty were studied. According to the pubertal development six were classified as P2, one as P3 and four as P5. In all cases plasma testosterone levels were definitely elevated (1.7-5.8 ng/ml) when compared with pre-pubertal controls. Peak values after HCG (3 X 1500 units) in four of the boys were in the high adult range. The binding capacity of serum testosterone oestradiol binding globuline (TeBG) ranged between 0.5 and 7.30 microgram/dl. Basal plasma levels of LH and FSH were respectively 2.06 +/- 0.64 and 1.2 +/- 0.25 miu/ml, and peak levels after LHRH (0.1 mg/m2) 13.9 +/- 3.7 and 2.6 +/- 0.43 miu/ml respectively. The data demonstrated a significant increase of plasma testosterone and post LHRH LH peak levels in boys with central precocious puberty when compared with pre-pubertal controls. The patients at stage P2 exhibited high levels of plasma testosterone contrasting with the degree of pubertal maturation, high values of TeBG and low response to LHRH which were in the pre-pubertal range. These findings suggest that the testicular sensitivity to LH increases early in boys with central precocious puberty, while the testosterone responsiveness, both at peripheral and hypothalamic levels, is delayed.

Child

[Endocrine data in cryptorchism].

Endocrine evaluation with LH-RH (0.1 mg/m2) and chorionic gonadotrophin (HCG 3 X 1,500 I.U.) in 154 cryptorchid boys aged 1 month to 15 years showed a decrease of LH pituitary secretion and Leydig-cells response to HCG in prepubertal and early pubertal patients. These deficiencies were positively correlated. Partial and at least transient descent of cryptorchid testis or testes has been obtained in 87 of 265 patients treated with HCG (3 to 9 X 1,500 I.U.). Plasma testosterone after HCG 3 X 1,500 I.U. was less increased in patients whose cryptorchid testis or testes descended after 9 X 1,500 I.U. than in those whose testes remained undescended. These data suggest that a partial, early and transient deficiency of pituitary LH secretion may be responsible for testicular maldescent in part of cryptorchid boys.

Adolescent

Correlation of pituitary and testicular responses to stimulation tests in cryptorchid children.

LH-RH test and HCG stimulation test were performed in 154 cryptorchid boys aged 1 month to 15 years (64 unilateral and 90 bilateral). Basal plasma LH levels and LH response to LH-RH were significantly lower from infancy to early puberty in cryptorchids compared with controls. Basal FSH levels and FSH response to LH-RH were normal. The post-HCG rise of plasma testosterone was reduced until mid-puberty. A significant positive correlation was found between post-HCG testosterone levels and pre- and post-LH-RH levels of LH. This correlation suggests that testicular maldescent and the decreased ability of Leydig cells to respond to a short course of HCG may result from an early defect or a delay of pituitary LH secretion.

Adolescent

[Weights and heights of newborn infants with hypothyroidism].

Among 166 congenitally hypothyroid children, 30% had a birth weight over the 90 degrees percentile of normal newborns, and 17% of those born after a full-term gestation weight 4000 g or more. The birth length was known in 84 cases and was not different from normal standards. An excess of birth weight relatively to birth length must suggest, among other factors, the possibility of congenital hypothyroidism.

Body Height

[Evaluation of gonadotropic and gonadal secretions in primary abnormalities of the gonads and male pseudohermaphrodism before and after the age of puberty].

Blood levels of gonadotropins (FSH and LH) and gonadal steroids (testosterone or estradiol) have been evaluated in 72 children and adolescents with primary gonadal defects (45 agonadal patients, 7 with asymetrical gonadal dysgenesis, 9 with Klinefelter syndrome, 4 with partial testicular dysgenesis and 7 with partial ovarian dysgenesis) and in 17 cases of male pseudohermaphrodism. A LH-RH test has been performed in most cases, and a testicular stimulation test with chorionic gonadotropin in patients with testicular tissue. Agonadal subjects had increased blood and pituitary releasable gonadotropins: very high in infants and young children, much less from 7 to 11 years, a high spurt being observed at 12 years. This diphasic pattern relates to the varying sensitivity of hypothalamic receptors and suggests that adrenal steroids may restrain gonadotropic secretion at 7-8 years. As a diagnostic tool, the increase of gonadotropins may be missing in the 7-11 years group. In the 1-12 years patients with partially defective gonads, blood and pituitary releasable gonadotropins and blood steroids are usually normal, but testosterone response to chorionic gonadotropin may be already blunted. From 13 years the gonadotropic secretion is usually increased, even when testosterone secretion and reserve are within normal range. Most male pseudohermaphrodites showed normal gonadotropic and testicular secretions. But four patients had highly increased responses to LH-RH, suggesting a defect of testicular secretion or of receptors, and demonstrating some heterogeneity in the male pseudohermaphrodism group.

Adolescent

[Plasma estorgens and testosterone in the normal young girl before and during puberty].

Base levels of estrone (E1), estradiol-17-beta (E2) and testosterone (T) were measured by radio-immunoassay. Results were classified according to the stages of puberty (Tanner scale) in 89 normal girls ranging from 1 to 22 years of age. Fourty-three pathological cases were studied. In 22 cases of ovarian agenesis E2 and T were most often low. In 7 cases of isolated breast prococity, 4 cases of isolated pilous precocity, one case of isolated genital hemorrhage and 5 cases of hypopituitarism, E1, E2 and T levels were similar to those of impuberal girls. Levels of oestrogens were increased in 4 cases of true precocious puberty.

Adolescent

[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

[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