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Biomedical subjects

H Domené

Publications and source records attributed to H Domené.

9 recordsLinked to original sources

Persistence of autonomous ovarian activity after discontinuation of therapy for precocious puberty in McCune-Albright syndrome.

OBJECTIVE: The aim of this study was to evaluate the possibility of persistence of autonomous ovarian activity in girls with McCune-Albright syndrome (MAS) after withdrawal of medroxyprogesterone therapy administered for precocious puberty. DESIGN, SETTING, AND PARTICIPANTS: Five girls with MAS were followed-up 1.2 to 8.5 years after the end of treatment. The girls underwent luteinizing hormone-releasing hormone (LH-RH) tests, estradiol (E2) basal measurement, and pelvic ultrasound two times in the follow-up period. RESULTS: Menses resumed in four of five girls, 1.4 +/- 0.9 years after the end of treatment, at chronologic age of 11.3 +/- 1.3 years. Cycles for all girls were irregular. Three patients presented inadequate E2 levels (from 56 to 320 pg/mL) associated with low or absent gonadotropin response to LH-RH tests. The pelvic ultrasound showed ovarian cysts at the time of the study. CONCLUSION: These hormonal and ultrasonographic findings provide evidence of persistence of autonomous ovarian activity in some young women with MAS.

Child↗

Growth hormone (GH) stimulates insulin-like growth factor-I (IGF-I) and IGF-I-binding protein-3, but not GH receptor gene expression in livers of juvenile rats.

In the adult rat, expression of the liver GH receptor, insulin-like growth factor-I (IGF-I), and IGF-I-binding protein-3 (IGFBP-3) genes has been shown to be under GH control. Additionally, hypophysectomy and GH treatment have a differential effect on the relative abundance of liver IGF-I mRNA variants in adult rats. To further elucidate the time of appearance and the extent of GH control of liver GH receptor, IGF-I, and IGFBP-3 gene expression, we studied the effect of hypophysectomy and GH and IGF-I treatment in juvenile rats. Male Wistar rats were hypophysectomized (Hx) on postnatal day 26 and received twice daily sc injections of saline, recombinant human GH (2.5 U/kg.day), or recombinant human IGF-I (500 micrograms/kg.day) for 7 days. Sham-operated rats received the same treatment. Hx animals also received T4 (20 micrograms/kg.day). In Hx animals, there was a significant reduction in body weight (69.8 +/- 6.6 vs. 100.4 +/- 5.4 g; P < 0.001). GH, but not IGF-I, treatment increased body weight (79.6 +/- 9.6 g after GH vs. 69.8 +/- 6.6 g before GH; P < 0.05). GH treatment partially maintained liver, kidney, and lung weights in Hx animals and increased them in intact animals, whereas IGF-I treatment did so only in the lungs of intact and Hx animals. Serum GH and IGF-I levels were markedly reduced in Hx animals compared with those in intact controls, and GH treatment maintained, albeit partially, circulating IGF-I levels compared with those in saline-treated Hx animals. IGF-I mRNA levels were markedly reduced in Hx liver (25.0 +/- 5.4%; P < 0.001 compared with intact controls). GH treatment for 7 days increased IGF-I mRNA levels by 4.8-fold over the levels in 9-day Hx animals and increased IGF-I mRNA levels by 2.2-fold in control rats. Hypophysectomy decreased exon 2-containing transcripts by 7.0-fold and exon 1-containing transcripts by 4.1-fold. GH treatment, however, affected both exon 1- and exon 2-containing transcripts similarly. Hepatic IGFBP-3 mRNA levels were reduced in Hx (53.2 +/- 1.8%; P < 0.01 compared with intact controls) and IGF-treated Hx animals, but were not decreased in Hx GH-treated animals (100.6 +/- 9.5). No changes in GH receptor or GH-binding protein mRNA levels were caused by Hx, GH, or IGF-I treatment.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗

Evaluation of gonadal function following long-term treatment for acute lymphoblastic leukemia in girls.

Twenty-four girls were studied following long-term treatment (mean: 50 months) for acute lymphoblastic leukemia; 14 were prepubertal and 10 pubertal. Follow-up during endocrine studies ranged from 2 months to 6.7 years (mean: 2.3 years). Five of 14 prepubertal patients started clinical pubertal development at a normal age and were reevaluated during puberty, increasing the pubertal group to 15 patients. Thirteen of 15 pubertal patients had received cranial radiotherapy. Ten of 15 pubertal patients started menses during the endocrine study. Although age of menarche was normal, in nine patients it was below the normal mean. Except for the remaining patient, all had received cranial cobalt therapy. In 6 of 19 patients bone age was significantly accelerated. Serum gonadotrophin response to LH-RH was normal in 13 prepubertal patients and in 10 pubertal patients. In 3 of 10 pubertal patients follicle-stimulating hormone (FSH) values were temporarily elevated. Only one pubertal patient had oligoamenorrhea. Five patients were studied by measuring serum progesterone on days 19-22 of the cycle to determine corpus luteum function. Three of them showed progesterone levels compatible with adequate corpus luteum function (6, 19, and 12 ng/ml, respectively) and two presented low progesterone levels (2 ng/ml), probably because of their short gynecological age (0.24 and 0.3 years, respectively). This study suggests that neither the disease nor the long-term antileukemia therapy seems to injure gonadal function in girls. A tendency to early sexual development was observed, which may be related to cranial cobalt therapy.

Acute Disease↗

Growth in Turner's syndrome: long term treatment with low dose ethinyl estradiol.

The growth response to 100 ng/kg BW X day ethinyl estradiol (2-4 micrograms/day) given for 18 months was studied in a group of nine patients with Turner's syndrome, aged 8.6-13.3 yr. All patients were prepubertal and had elevated gonadotropin levels. Growth velocity increased from 3.09 +/- 1.05 (+/-SD) to 7.09 +/- 1.47 cm/yr in the first 6 months, 6.08 +/- 1.78 cm/yr in the second 6 months, and 4.03 +/- 1.65 cm/yr in the third 6 months. Bone age increased a mean of 0.82 +/- 0.34, 0.89 +/- 0.79, and 0.74 +/- 0.59 yr, respectively, in the three 6-month periods. Predicted height prognosis did not change after any period. All patients had pubertal changes, limited in most to slight breast development. Mean diurnal spontaneous GH secretion did not change during treatment. Plasma somatomedin-C levels were low before treatment, and ethinyl estradiol did not significantly increase somatomedin-C values. Our data confirm the ability of a low dose of ethinyl estradiol to increase growth velocity in girls with Turner's syndrome, although the effect diminished with time, and the excess bone age advancement precluded improvement of predicted height.

Adolescent↗

Comparison of single and combined tests for the evaluation of plasma growth hormone secretion in normal short children.

Growth hormone (GH) response to provocative tests was compared in normal short children. Seven of 23 children failed to respond to insulin hypoglycemia. Using insulin hypoglycemia followed by L-dopa only 2 of 23 children did not respond and giving bromocriptine combined with insulin hypoglycemia only 1 of 8 children failed to respond. All children submitted to propranolol followed by exercise (n = 14) and to bromocriptine followed by exercise (n = 6) responded with a satisfactory increase in plasma GH levels. The increase elicited by propranolol and exercise was higher than that induced by insulin hypoglycemia alone (p less than 0.005), exercise alone (p less than 0.05) or L-dopa after insulin hypoglycemia (p less than 0.01). The rise of GH induced by bromocriptine and exercise was higher than that obtained with insulin hypoglycemia alone (p less than 0.05). This study suggests that both adrenergic and dopaminergic mechanisms are involved in exercise induced GH release and confirms that combined tests are more useful than a single test to evaluate GH secretion.

Adolescent↗

Evaluation of testicular function following long-term treatment for acute lymphoblastic leukemia.

A study of reproductive function was carried out in 31 patients following long-term treatment of acute lymphoblastic leukemia (mean: 4 1/12 years); 17 prepubertal; nine pubertal, and five adults. Spontaneous clinical pubertal development was observed in 4 of 17 prepubertal patients. Serum testosterone response to hCG was normal or increased in the nine prepubertal patients studied. Intratesticular testosterone concentration was normal in 11 out of 12 patients. Only two of 14 prepubertal patients had a reduced number of germ cells. Normal progress of spermatogenesis was seen in six out of seven pubertal patients and only moderate hypospermatogenesis with focal hyalinosis was found in the five adults. Two of them had normal sperm count and one fathered two children. In six out of 14 prepubertal patients unexpected early signs of pubertal stimulation were found. Serum gonadotrophins response to LH-RH was normal in most patients. Testicular infiltration of blastic cells was found in four of 26 biopsies, but only one of 23 patients was without clinical signs of testicular relapse. Computerized axial tomography was normal in 10 out of 13 patients. Bone age was significantly below chronological age in four out of 15 patients. This study suggests that the gonad is moderately injured by long-term antileukemic therapy. In addition, microscopic testicular infiltration is not frequent in subjects without testicular enlargement.

Adolescent↗

Effects of alpha-bromoergocryptine on pituitary hormone secretion in children.

The effect of bromoergocryptine (BE) on human GH (hGH), PRL, LH, FSH, TSH, and blood sugar levels was evaluated in a series of 22 normal children. Blood samples were collected in basal conditions and after 30, 60, 90, 120, 150, and 180 min of BE administration (1.25 or 0.62 mg/os). The drug induced a marked and sustained increase in hGH secretion when basal serum levels of the hormone were lower than 10.0 ng/ml (n = 15). When basal hGH serum levels were higher (n = 7), BE provoked either a later increase of serum hGH in most children (n = 5) or a persistent decrease in hGH (n = 2). On the other hand, BE markedly inhibited PRL secretion without modifying LH, FSH, or TSH. The data obtained suggest that BE can be used as a useful tool for the assessment of hGH secretion in children.

Adolescent↗

Testicular function in varicocele.

Testicular testosterone concentration serum testosterone, LH and FSH, sperm count and testicular histology were evaluated in 17 patients with varicocele. Testicular testosterone was either normal or high (mean 906 +/- 723 ng/g of tissue), and serum testosterone was within the normal range in most patients. Serum LH was elevated in half of the patients. The degree of testicular damage observed was extremely variable and correlated with sperm analysis. Testicular testosterone tended to be higher in patients with severe microscopic lesions of the testis. It is concluded that even though Leydig cell function is partially altered, this deficiency is compensated by LH stimulation and therefore, failure of spermatogenesis is not secondary to low testosterone levels.

Adult↗

The hormonal regulation of premeiotic steps of spermatogenesis in the newborn rat.

.he effect of high doses of testosterone propionate (TP) on the development of the first spermatogenic wave was systematically analyzed to determine the period of maximal susceptibility to testosterone. Two mg of TP were administered daily to groups of immature male rats, starting from birth, or days 5, 10, 15, and 20 until day 35 of life. Animals injected from birth or day 5 showed severe testicular atrophy, with reductions of more than 70% of testicular weight, diminished tubular diameters, and spermatogenic arrest during the meiotic prophase. Groups treated from days 10 or 15 showed increasing testicular weights with qualitatively normal spermatogenesis. When treatment started at 20 days, completely normal testicular development was achieved. To test the responsiveness of the neonatal hypothalamo-pituitary axis to TP administration, groups of 5-day-old male rats received daily injections of TP, and plasma FSH was determined at ten, 20, and 35 days. FSH levels were not detectable at ten and 20 days, and extremely depressed at 35. A group of 5-day-old male rats was injected simultaneously with 2 mg of TP and 14 IU of FSH (human menopausal gonadotropin: 71 IU of FSH and 80 IU of LH/ml) until day 35. Testicular weights and tubular diameters were increased compared to controls, and spermatid differentiation had proceeded to more mature steps than those seen in control animals. Inhibition of testicular development by neonatal TP administration was paralleled by a sharp decrease in circulating FSH levels and reversed by FSH replacement.(ABSTRACT TRUNCATED AT 250 WORDS)

Aging↗