Search PubMed⌕ Search

Biomedical subjects

S Mora

Publications and source records attributed to S Mora.

At least 73 records · Page 4Linked to original sources

Human growth hormone treatment in prepubertal children with achondroplasia.

We studied the effects of recombinant human growth hormone (GH) treatment in 6 prepubertal children with achondroplasia. The patients' age ranged from 2 11/12 to 8 5/12 years and the GH dose was of 0.1 IU/kg/day subcutaneously. Auxological assessments and bone age determinations were performed 6 months before, at the beginning, and after 6 and 12 months of therapy. The growth velocity increase during the whole year of treatment ranged from 1.1 to 2.6 cm/year in 3 patients while in the others no variation was detected. No side effects were observed during the trial apart from a slight advancement of bone age in two patients. MRI at the cervicomedullary junction and CT scan of the base of the skull did not show any variation of the dimensions of the foramen magnum at the end of the trial compared to baseline. Our study shows that r-hGH can safely increase short-term growth velocity in some but not all prepubertal children with achondroplasia. Our data confirm the individual variability in the response to the GH treatment.

Achondroplasia↗

Bone density in young patients with congenital adrenal hyperplasia.

One of the major complications of glucocorticoid treatment is bone loss. 21-Hydroxylase deficiency is the most frequent inborn error of steroidogenesis, leading to congenital adrenal hyperplasia (CAH): synthesis of cortisol is impaired and replacement therapy is therefore mandatory. We studied the bone mineral density in a group of patients with congenital adrenal hyperplasia (CAH) on long-term glucocorticoid replacement therapy. We selected 30 Caucasian patients with CAH due to 21-hydroxylase deficiency (mean +/- SD age = 17.45 +/- 2.49 years). 22 patients had the classical CAH form and the remaining 8 had the nonclassical (late-onset) form. The mean duration of therapy was 15.20 +/- 4.04 years. Bone mineral density (BMD) was evaluated with a dual-energy X-ray absorptiometer. BMD was also measured in 73 healthy white volunteers of comparable age (17.35 +/- 2.99 years). BMD values of the spine (sBMD), total body (TBBMD), legs, and arms of CAH patients, adjusted for confounding variables (age, gender, body mass index), did not differ from those of control subjects (p = 0.86; p = 0.17; p = 0.06 and p = 0.26, respectively). sBMD and TBBMD values did not show relationships with the duration of treatment and the dose of corticosteroids. Patients with the classical form of CAH had bone density values comparable with those of patients with the nonclassical form (sBMD: p = 0.33; TBBMD: p = 0.97). Our data show that, despite long-term treatment with glucocorticoids, CAH patients have bone density values comparable with controls.

Absorptiometry, Photon↗

Effects of the estrous cycle and ovarian hormones on behavioral indices of anxiety in female rats.

The influence of the estrous cycle and the effects of exogenous administration of estradiol and progesterone on level of anxiety were studied in intact and ovariectomized rats. Intact Sprague-Dawley female rats were classified according to the stages of estrous cycle. Another group of rats was ovariectomized bilaterally and, 14 days after surgery, they received estradiol benzoate (10 micrograms/kg, s.c.) and/or progesterone (25 mg/kg, s.c.) or corn oil (1 ml/kg). The behavioral tests began 3 h after estradiol or 6 h after progesterone and consisted of: (1) exploration of an elevated plus-maze; and (2) retention of a passive avoidance response. Open-arm exploration of the plus-maze varied according to light intensity and the stages of the estrous cycle. There was a slight increase in open-arm exploration by rats in metestrus, under high light intensity. Low light intensity increased the exploration of the open arms by rats in proestrus and estrus, compared to the other phases of the cycle. Retention of the passive avoidance response was inhibited during proestrus and estrus. Progesterone increased open-arm exploration of the plus-maze under high light conditions, whereas estradiol antagonized this effect. Retention of passive avoidance was inhibited after estradiol or progesterone injection. These results suggest that the behavioral indices of anxiety can vary across the estrous cycle, that low light intensities have anxiolytic-like effects, and that the sensitivity to this effect is higher during proestrus and estrus. This could be explained through modulatory effects of ovarian hormones upon behavioral indices of anxiety.

Animals↗

Influence of body composition on bone mineral content in children and adolescents.

Excess fat and fat-free mass have been extensively described in obese children, whereas few data about bone mineral content (BMC) variations are available in children. Dual-energy X-ray absorptiometry (DXA) allows a direct and accurate measurement of three body compartments (fat, lean, and BMC), subdivided into three regions (arms, trunk, and legs). The aim of our study was to evaluate the influence of body compartments on total BMC (TBMC) and regional BMC (RBMC) in obese and normal-weight subjects. Sixty-five obese and 50 normal-weight children and adolescents (age range: 5-18 y relative body weight: 160 +/- 23% and 101 +/- 12%, respectively), matched for sex and pubertal stage underwent a DXA total-body analysis. Obese subjects had significantly greater fat and lean compartments than normal-weight subjects (P < 0.0001). TBMC was larger in obese children (1930 +/- 670 g compared with 1480 +/- 490 g, P < 0.0001) as was RBMC (arms: 182 +/- 81 g compared with 151 +/- 65 g; trunk: 560 +/- 223 g compared with 433 +/- 169 g; legs: 788 +/- 341 g compared with 539 +/- 231 g, P < 0.0001). We found lean mass to be the best correlate with TBMC (r = 0.91 in obese and 0.94 in normal-weight children). Multiple-regression analysis confirmed lean mass as one of the major determinants of TBMC and RBMC in children. However, differences in TBMC and RBMC were no longer present after correction for age, sex, and body-composition variables. There were no differences in TBMC and RBMC between obese and normal-weight children after correction for the confounding variables age and sex.

Absorptiometry, Photon↗

Insulin and insulin-like growth factor I (IGF-I) stimulate GLUT4 glucose transporter translocation in Xenopus oocytes.

1. The heterologous expression of glucose transporters GLUT4 and GLUT1 in Xenopus oocytes has been shown to cause a differential targeting of these glucose-carrier isoforms to cellular membranes and a distinct induction of glucose transport activity. In this study we have evaluated the effect of insulin and insulin-like growth factor I (IGF-I) on glucose uptake and glucose transporter distribution in Xenopus oocytes expressing mammalian GLUT4 and GLUT1 glucose carriers. 2. Insulin and IGF-I stimulated 2-deoxyglucose uptake in GLUT4-expressing oocytes, but not in GLUT1-expressing oocytes or in water-injected oocytes. The stimulatory effect of insulin and IGF-I on 2-deoxyglucose uptake in GLUT4-expressing oocytes occurred via activation of the IGF-I receptor. 3. Subcellular-fractionation studies indicated that insulin and IGF-I stimulated translocation of GLUT4 to the cell surface of the oocyte. 4. Incubation of intact oocytes with insulin stimulated phosphatidylinositol 3-kinase activity, an effect that was blocked by the additional presence of wortmannin. Furthermore, wortmannin totally abolished the insulin-induced stimulation of 2-deoxyglucose uptake in GLUT4-expressing oocytes. 5. In this study, both the insulin-induced GLUT4 carrier translocation and GLUT4-dependent insulin-stimulated glucose transport have been reconstituted in the Xenopus oocyte. These observations, together with the fact that wortmannin, as found in adipocytes, inhibits insulin-stimulated glucose transport in oocytes, suggest that the heterologous expression of GLUT4 in oocytes is a useful experimental model by which to study the cell biology of insulin-induced GLUT4 translocation.

Androstadienes↗

Bone mineral metabolism and thyroid replacement therapy in congenital hypothyroid infants and young children.

Impairment of calcium metabolism and low bone density have been found in hypothyroid adults. We investigated the effect of thyroid replacement therapy on calcium metabolism and bone mineralization in congenital hypothyroid (CH) infants and children. One hundred and 16 Caucasian CH consecutive patients were studied and were grouped according to their age: 23 patients at diagnosis, 20 at 3 mo, 24 at 6 mo, 25 at 12 mo and 24 at 36 mo. Thyroid replacement therapy was started at an initial dose of 6-8 micrograms/kg/day of L-thyroxine, and then decreased progressively. Calcium, phosphorus, magnesium, alkaline phosphatase (AP), parathyroid hormone (PTH) and osteocalcin (BGP) were measured as calcium metabolism indices. Bone mineral content (BMC) was measured at the mid-portion of the right radius AP, PTH and BGP concentrations were significantly higher in subjects at 3 mo of age (p < 0.05). This rise coincided with the end of the period of maximum dosage of L-thyroxine. Mild asymptomatic hypercalcemia was observed in 20 patients. All the other indices did not differ between age groups. BMC values and BMC annual increment were not different from those calculated for age-matched controls. We found that L-thyroxine replacement therapy does not alter bone mineralization of CH infants and children. Only a transitory increase of osteoblastic function was observed after the first few months of therapy.

Bone Density↗

Neurophysiologic studies and cognitive function in congenital hypothyroid children.

Minor neurologic and intellectual impairments have been described in some congenital hypothyroid (CH) children in spite of early detection by neonatal screening. The aim of our study was to assess cognitive functions as well as neurophysiologic parameters in hypothyroid children and to compare children detected by neonatal screening (group A) versus hypothyroid patients clinically diagnosed before the beginning of the screening program (group B). Group A consisted of 15 children (13 girls, mean age at the beginning of treatment 33 d). Group B consisted of 11 patients (7 girls, mean age at the start of treatment 10.1 mo). Twenty age-matched healthy children were studied as a control group for neurophysiologic tests. Neurophysiologic tests (Auditory P 300, long latency somatosensory evoked potentials (LL-SEP) were performed along with IQ evaluation. Abnormalities of neurophysiologic tests were detected in 82% of clinically diagnosed hypothyroid children. Surprisingly, 47% of the children detected by neonatal screening, having normal mental development index, showed at least one abnormal neurophysiologic test. LL-SEP latencies were found significantly increased in both groups of CH patients compared with controls. Our data are suggestive for a prenatal or perinatal CNS damage in some children with congenital hypothyroidism, despite early treatment.

Case-Control Studies↗

Influence of the estrous cycle and estradiol on the behavioral effects of amphetamine and apomorphine in rats.

This experiment was designed to investigate the influence of hormonal status of the rat on the effects of two doses of an indirect-acting dopamine agonist (amphetamine 0.25 and 1.0 mg/kg, IP) and a direct-acting dopamine agonist (apomorphine 62.5 and 250 micrograms/kg, SC) on the acquisition of conditioning avoidance responses (CARs) and the performance of some spontaneous behaviors. Active conditioned avoidance was improved by amphetamine in all the groups except in females at diestrus; apomorphine improved this response only in females at estrus and in ovariectomized rats after estradiol replacement, but the avoidance response was deteriorated in males and females at diestrus and after ovariectomy without estradiol replacement. Both dopaminergic drugs had contrasting effects on motor activity, number of rearings, and number of head shakes according to the hormonal status of the rat. Only the time spent in grooming behavior decreased after the treatment with both dopamine agonists in all of the five groups studied. These results provided behavioral evidence for the hypothesis that dopaminergic activity in the CNS is affected distinctively by modifications in the sexual hormone status (gender, estrous cycle, ovariectomy, and estradiol replacement). Relationships between ovarian hormones and dopaminergic system are discussed.

Amphetamine↗

Progesterone effects on the acquisition of conditioned avoidance responses an other motoric behaviors in intact and ovariectomized rats.

This study demonstrates a significant impairment in the acquisition of conditioned avoidance responses in female rats during their estrus phase. Progesterone (PROG 5 mg) injected 6 h prior to the test, significantly enhanced the performance exhibited by rats at estrus, but not at diestrus. In ovariectomized rats, the acquisition of conditioned avoidance responses was similar to the exhibited during diestrus and this behavior was depressed by a single dose of estradiol benzoate (EB 2 micrograms) injected 48 h prior to the test. PROG antagonized the avoidance depression induced by EB, but it was not able to induce changes in the acquisition of conditioned avoidance response in ovariectomized rats without EB pretreatment. Estradiol appears to be the principal ovarian steroid modulating the acquisition of an avoidance task, whereas PROG seems to have a secondary role in this behavior, regulating the actions of estradiol on the brain. PROG failed to induce consistent changes in some spontaneous motor behaviors in intact and ovariectomized rats.

Animals↗

Age-related changes in cortical and cancellous vertebral bone density in girls: assessment with quantitative CT.

OBJECTIVE: Bone mass increases during growth, but little information is available about the changes in cortical and cancellous bone densities during skeletal development or their relative contributions to age-related increases in bone mass. Accordingly, separate measurements of cancellous and cortical bone density were done at each stage of sexual development in white girls during childhood and adolescence. SUBJECTS AND METHODS: Quantitative CT was used to measure the densities of cortical and cancellous bone of the lumbar spine in 96 healthy white girls 4-20 years old. The relationships among various anthropometric indexes, pubertal status, and corresponding densities of cortical and cancellous bone were then examined. RESULTS: Cortical bone density increased with age, and values were significantly correlated with the anthropometric indexes of height (r = .61), weight (r = .62), body mass index (r = .61), and muscle volume (r = .58). In contrast, cancellous vertebral bone density increased only during the later stages of puberty. Moreover, cancellous bone density in prepubertal girls was inversely related to age (r = -.27) as well as to both the volume (r = -.20) and the height (r = -.15) of the vertebral body. CONCLUSION: The results suggest that weight bearing and/or mechanical stresses are important determinants of cortical bone density in the lumbar spine throughout growth, whereas cancellous vertebral bone density is more strongly influenced by hormonal and/or metabolic factors associated with sexual development during late adolescence.

Adolescent↗

Behavioral effects of a LHRH antagonist in intact and ovariectomized rats.

The effects of the LHRH antagonism on the acquisition of conditioned responses (CARs) and spontaneous motility were studied in intact and ovariectomized rats. A synthetic antagonist of LHRH, [N-acetyl-D-p-chloro-Phe1,2,D-Trp3,D-Arg6,D-Ala10]-LHRH, was injected in a single dose (10 micrograms/rat, SC) at noon on the day of proestrus in the normally cycling rat, and behavioral experiments were carried out on the morning of estrus or metestrus. Two procedures were followed in the ovariectomized rats: in the first, the antagonist was injected 1 h before estradiol, and in the second, at noon on the day after estradiol replacement. The experiments were carried out 24 and 48 h after estradiol, respectively. The LHRH antagonist facilitated the acquisition of CARs in both experimental groups, thus reversing the impairments observed during estrus and metestrus and those induced by estradiol replacement. The antagonist decreased the number of head shakes during estrus, whereas it induced an increase in total motility and rears in ovariectomized control animals. On the other hand, the antagonist increased the number of rears and reversed the decrease in grooming behavior induced by estradiol. The results led to the idea of a role of LHRH in behaviors not apparently related to sex, which could explain the behavioral changes observed across the estrous cycle and those induced by estradiol replacement in ovariectomized rats.

Amino Acid Sequence↗

Effect of gluten-free diet on bone mineral content in growing patients with celiac disease.

Osteoporosis is a complication of celiac disease in adulthood, but little is known about the influence of the disease on bone mineralization in children. In the present study we evaluated radial bone mineral content (BMC) in celiac children and adolescents at diagnosis and after they consumed a gluten-free diet (GFD). The BMC values of 33 celiac patients at diagnosis were significantly lower than those of 255 control subjects (P < 0.001). There was no difference between diabetic and non-diabetic celiac patients. In 14 patients the BMC increased significantly (P < 0.05, ANCOVA) after 1.28 y of GFD. In these patients the mean annual BMC increment was 0.07 g/cm, significantly greater (P < 0.05) than the increment of normal growing children (0.05 g.cm-1.y-1). Our data indicate that although osteoporosis complicates celiac disease during childhood and adolescence, GFD alone is able to remarkably improve bone mineralization.

Bone Density↗

Effect of estrogen replacement therapy on bone mineral content in girls with Turner syndrome.

Because a close relationship between estrogen deficiency and osteoporosis has been proven, it is possible that lifelong estrogen deficiency might be the cause of osteopenia in Turner syndrome. This study was done to characterize the effect of estrogen therapy on bone mineralization in girls with Turner syndrome. Radial bone mineral content values were found to be below the 95% normal confidence interval in 44 of 49 untreated patients, aged 10.82 +/- 3.45 years. An inverse correlation was found between the patients' ages and their delta bone mineral content values. The effect of beginning estrogen treatment early or late was studied in 16 girls who started the treatment before and 11 who started after age 12. Although they were still deficient compared with controls, the first group had better mineralization than the second (P = .0005). Finally, nine patients were followed prospectively during replacement therapy; their bone mineral content delta values changed significantly (P = .02) during the follow-up period (3.17 +/- 0.33 years), but the bone mineral content did not normalize. Our data show that estrogen deficiency per se does not cause osteoporosis in young girls with Turner syndrome. In fact, estrogen therapy prevented bone loss but failed to normalize the low bone mineral content values. Early treatment is preferable because it reduces the bone density deficit present in untreated patients.

Adolescent↗

Changes in vertebral bone density in black girls and white girls during childhood and puberty.

BACKGROUND: The prevalence of osteoporosis and the incidence of vertebral fractures are lower in black women than in white women, findings generally attributed to racial differences in adult bone mass. Little is known, however, about the factors that contribute to racial variations in bone mass or the time of life when such differences become manifest. This study was done to characterize the changes in vertebral bone density at various stages of sexual development in black and white females. METHODS: We measured cancellous vertebral bone density by quantitative computed tomography in 75 black female subjects between 2 and 20 years old and 75 whites matched for age and stage of sexual development. RESULTS: The vertebral bone density did not differ between black girls and white girls before puberty. Bone density increased during puberty in each racial group, but the magnitude of the increase from prepubertal values was substantially greater in black than in white subjects (34 percent vs. 11 percent). CONCLUSIONS: The marked difference between black and white females in cancellous vertebral bone density occurs during a relatively brief period late in puberty. Metabolic and hormonal events related to the achievement of sexual maturity during adolescence may be important determinants of racial differences in bone mass in women.

Adolescent↗

Vertebral bone density in insulin-dependent diabetic children.

To determine the effect of insulin-dependent diabetes mellitus (IDDM) on bone mass, we compared the trabecular and cortical bone density in lumbar vertebrae, measured by quantitative computed tomography (CT), in 48 white diabetic patients (23 females, 25 males; 5.2 to 19.6 years of age) with those of a control group of 48 healthy subjects, matched for race, sex, and age. Patients with neuropathy, retinopathy, nephropathy, and those with recent ketoacidosis were excluded from the study. The patient and control groups did not differ in sexual or skeletal maturation, weight, height, surface area, body mass index, abdominal fat, or paraspinal musculature. In diabetic children, cortical bone density was slightly but significantly lower than in controls (3.5% lower, P less than .02); there was no difference between patients and controls regarding trabecular bone density. The decrease in cortical bone density in the diabetic group did not correlate with age, sex, duration of diabetes, or glycosylated hemoglobin levels. These results suggest that in children with uncomplicated IDDM, decreased vertebral bone density is a minor abnormality that only affects cortical bone.

Adolescent↗