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Biomedical subjects

F Rigon

Publications and source records attributed to F Rigon.

At least 37 records · Page 2Linked to original sources
PubMed2000

Pamidronate treatment of bone fibrous dysplasia in nine children with McCune-Albright syndrome.

McCune-Albright syndrome is a rare genetic disorder consisting of skin and bone dysplasia and peripheral endocrinopathies. Little data have been collected regarding bisphosphonate treatment of bone fibrous dysplasia in paediatric patients with this syndrome. The aim of our study was to investigate the therapeutic efficacy of pamidronate in these patients. Nine patients with moderate to severe forms of bone fibrous dysplasia were treated with pamidronate intravenously (0.5-1 mg/kg/daily for 2-3 d) at 0.5-1-y intervals. Patients were treated over a time period of 0.5-3.5 y. During treatment no spontaneous fracture occurred. Bone pain and gait abnormality due to pain disappeared after 2-3 therapeutic cycles. Cranial asymmetry and limb length discrepancy remained unchanged. Elevated serum alkaline phosphatase and urine hydroxyproline values were reduced by the treatment, demonstrating drug activity at the lesional level. The effectiveness of pamidronate was also seen at the non-lesional level through an increase in bone density. Radiographic and scintigraphic evidence of lesion healing was not attained. Pamidronate treatment can ameliorate the course of bone fibrous dysplasia in children and adolescents with McCune-Albright syndrome.

Adolescent↗
PubMed2000

[Prenatal steroids, cesarean section, and cerebral hemorrhage in newborns with birth weight under 1500 g].

OBJECTIVE: To identify perinatal risk and protective factors for peri-intraventricular hemorrhage (PIVH) in 214 very low birth weight (VLBW) infants. METHOD: Perinatal risk and protective factors for PIVH were examined by univariate analysis and by multivariate logistic regression analysis that adjusted simultaneously for the effects of year of birth and perinatal characteristics. Because mode of delivery and antenatal use of steroid represent the only factors, among those considered, that could in most cases be controlled by the clinician, we calculated for each of the four combinations the relative risk of PIVH. RESULTS: By univariate analysis, antenatal steroid administration, cesarean section delivery, increasing birth weight and increasing gestational age were protective perinatal factors. By using logistic regression, significant protective factors against PIVH included a complete course of antenatal steroid therapy and increasing gestational age. Intrauterine growth retardation was associated with an increased risk of PIVH. The relative risk of intraventricular hemorrhage for each of the four combination of mode of delivery and antenatal steroid exposure demonstrate that antenatal steroid exposure and cesarean section delivery are most protective with a relative risk of 14% compared with vaginal delivery and no antenatal steroid exposure. CONCLUSIONS: Association of antenatal steroid administration with cesarean section delivery has an important role in lowering the risk of PIVH in VLBW infants.

Adrenal Cortex Hormones↗
PubMed2000

[Nasal CPAP in newborns with birth weight under 1500 g].

OBJECTIVE: To evaluate the efficacy and safety of early nasal continuous positive airway pressure (CPAP) in the pulmonary management of very low birth weight (VLBW) infants. METHOD: Since 1993 in our neonatal intensive care unit we decided to reduce the rate of tracheal intubation and mechanical ventilation (IPPV) using more extensively nasal CPAP. By Student's t test and c2 analysis, VLBW infants with gestational age < 32 weeks born in 1988-92 were compared with those born in 1993-97. RESULTS: The 144 enrolled infants were comparable for birth weight and gestational age. There was a difference in the rate of antenatal steroid administration and cesarean section delivery, significantly more used in the second period. In both groups, 93% of the infants required respiratory support. The use of IPPV decreased from 68% in the first period to 30% in the second, conversely the use of CPAP increased from 25 to 63%. With regard to the primary outcome, in the second period the mortality rate and the incidence of pneumothorax were significantly lower. CONCLUSIONS: As the long-term survival of VLBW neonates continues to improve, pulmonary management is increasingly directed at minimizing the invasivity. This study demonstrated that early nasal CPAP may be successfully used in most VLBW infants. Also in the smallest neonates the procedure is safe and effective and may be important in lowering pulmonary morbidity and subsequent mortality.

Female↗
PubMed1998

Comparison of clinical-radiological and molecular findings in hypochondroplasia.

Hypochondroplasia is an autosomal dominant skeletal dysplasia characterized by disproportionate short stature. A mutation (N540K) in the fibroblast growth factor receptor 3 (FGFR3) gene was described in some patients with this condition. The aims of the study were to identify the frequency of the FGFR3 gene mutation, to define the salient clinical and radiological abnormalities of the affected subjects, and to verify the contribution of molecular findings to the clinical and radiological definition of hypochondroplasia. Based on the most common radiological criteria, we selected 18 patients with a phenotype compatible with hypochondroplasia. Height, sitting height, and cranial circumference were measured in all patients. Radiographs of the lumbar spine, left leg, pelvis, and left hand were also obtained. The presence of the N540K mutation was verified by restriction enzyme digestions. Half of our patients carried the N540K mutation. Although similar in phenotype to the patients without the mutation, they showed in addition relative macrocephaly. The association of the unchanged/narrow interpedicular distance with the fibula longer than the tibia was more common in patients with gene mutation. Although we did not find a firm correlation between genotype and phenotype, in our study the N540K mutation was most often associated with disproportionate short stature, macrocephaly, and with radiological findings of unchanged/narrow interpedicular distance and fibula longer than tibia.

Adolescent↗
PubMed1997

Hypertensive cardiomegaly caused by an aldosterone-secreting adenoma in a newborn.

A case of primary hyperaldosteronism and cardiomegaly due to a unilateral adrenal adenoma in a newborn is presented. Some peculiarities, most likely in relation to the onset of the disease during fetal life were evident: plasma cortisol was slightly increased before surgery, plasma renin activity was elevated 9 months after surgery and mineralcorticoid receptors remained suppressed 4 months after surgery. Unilateral adrenalectomy reversed both hypertension and cardiomegaly. We speculate that cardiomegaly was related to both hyperaldosteronism and hypertension and that individual factors are involved in the pathogenesis of cardiomegaly in hyperaldosteronism.

Adenoma↗
PubMed1997

Biochemical selection of prepubertal patients with androgen insensitivity syndrome by sex hormone-binding globulin response to the human chorionic gonadotropin test.

Before puberty, the diagnosis of androgen insensitivity syndrome (AIS) can be difficult. We studied whether the decrease of sex hormone-binding globulin (SHBG) during the human chorionic gonadotropin (hCG) test may represent a biochemical test to select prepubertal patients with AIS. We examined prepubertal patients with AIS (n = 9, age 0.9-8.2 y), male pseudohermaphroditism not due to AIS (other-MPH) (n = 8, age 0.6-10.7 y), and control boys (n = 12, age 0.8-12.5 y). Testosterone and SHBG levels (mean +/- SD) were measured before (d 0) and after (d 5) a hCG test (1500 IU X 3 d). Testosterone levels (nmol/L) increased in all groups [AIS: from 1.5 +/- 1.2 to 22.1 +/- 11.8 (p < 0.001); other-MPH: from 0.6 +/- 0.6 to 9.2 +/- 7.4 (p < 0.02); controls: from 1.8 +/- 1.4 to 22.8 +/- 14.4 (p < 0.001)]. SHBG concentrations (nmol/L) did not change in AIS [from 66.2 +/- 15.1 to 67.5 +/- 18.6 (p = NS), delta-variation 1.7 +/- 12.7%], whereas they were significantly decreased in other-MPH [from 59.9 +/- 14.2 to 46.5 +/- 18.6 (p < 0.005), delta-variation -23.7 +/- 19.6%] and controls [from 63.0 +/- 16.9 to 33.7 +/- 14.6 (p < 0.003), delta-variation -46.9 +/- 15.2%]. Our data suggest that the SHBG changes during the hCG test can be used to assess in vivo the biologic response to androgens in prepubertal patients with ambiguous genitalia, selecting those patients in whom it is worth performing second level investigations to confirm the AIS diagnosis.

Androgens↗
PubMed1997

[Graves' disease].

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Antithyroid Agents↗
PubMed1996

[Precocious puberty].

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Antineoplastic Agents, Hormonal↗
PubMed1995

Natural course of subclinical hypothyroidism in Down's syndrome: prospective study results and therapeutic considerations.

Pathogenesis, natural course and therapeutic management of subclinical hypothyroidism (SH) in Down's syndrome (DS) remain object of debate in literature. In the present study thyroid function, antithyroid antibody (ATA) prevalence and serum lipid concentrations were investigated in a group of 344 Down patients (DP) and data were compared with those obtained from a control group of 257 age and sex matched healthy subjects. Thyroid function and ATA prevalence were also studied in 120 parents of DP. SH prevalence was clearly higher in DP (32.5% of cases) than in controls (1.1%) and parents (0%). Similarly, ATA prevalence was higher in DP (18% of cases) than in controls (5.8%) and parents (6.6%). In spite of this, no correlation was found in DP between SH and ATA prevalences, since ATA were detected in 18.7% of SH-DP and in 15.8% of euthyroid DP. Thus, circulating ATA were not detected in the majority of SH-DP. No significant differences regarding T4, FT4, T3 and serum lipid levels among SH and euthyroid DP and controls were found. Moreover, TSH levels were only slightly increased, generally less than 10 microU/ml, in most cases of SH-DP. Follow-up was longer than 24 months (range 2-7 years, mean 3.1) in a group of 201 DP: two different patterns of SH course were observed, mainly depending on the presence or the absence of circulating ATA. In particular, 35.7% of ATA-positive SH-DP developed a clinically evident thyroid disease (overt hypothyroidism or hyperthyroidism), while no similar case was recorded among ATA-negative SH-DP.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1995

Frequency, clinical and laboratory features of thyroiditis in girls with Turner's syndrome. The Italian Study Group for Turner's Syndrome.

A total of 478 patients, mean age 15.5 (3.6-25.3) years, suffering from Turner's syndrome, were studied in order to determine the frequency of autoimmune thyroiditis, which is defined as the presence of antithyroid antibodies (AT-Ab) and typical ultrasound findings. We found 106 (22.2%) patients positive for AT-Ab and of those 49 (10%) also had positive ultrasound findings, and were therefore considered to be affected by thyroiditis. This frequency is significantly higher (p < 0.001) than that seen in the normal population. Goitre was detected on clinical examination in only 16 (33%) and by ultrasound in 19 (39%) patients. Hormonal evaluation showed that 17 patients were euthyroid, 27 had compensated hypothyroidism, 2 were hypothyroid and 3 were in a hyperthyroid phase. Clinical signs or symptoms of hypothyroidism were absent in all hypothyroid patients. In patients with thyroiditis, neither a higher frequency of malformations and autoimmune diseases nor a correlation with karyotype, oestrogens or growth hormone therapy was found.

Adolescent↗
PubMed1995

[Obesity, overweight and physical activity in elementary school children].

This research presents an epidemiological study on the prevalence of obesity and overweightness in an Elementary School population. 243 children of third and fourth grade (males 52.26% and females 47.74%, 9-10 years old) of the Selvazzano (Padova) District, were evaluated during the annual medical visit. The Body Max Index (BMI) and the Cole index were calculated. The parents filled out a questionnaire which investigated the level and quality of involvement in sports. The statistical analysis was executed by the ANOVA test and post Hoc analysis. 59.02% of the children resulted within the ponderal normality with the Cole index < or = 110, 13.5% resulted overweight (Cole index 110 divided by 120), 20% resulted obese (Cole index 120 divided by 140), 7.3% resulted highly obese (Cole index > 140). High level obesity was found in fourth grade boys (p < 0.0001). As far as sports are concerned, it is noted that 173 children (73.3%) practise some sport. The more practised sports, swimming (44.5%), soccer (27.1%) and gymnastic (15.02%), are practised twice a week by 58.3% of the children. Obesity and overweight in the examined population result high even in relation to data found in literature. When choosing a sport activity to prevent overweightness or obesity, one should advise parents, school operators and students to choose a sport with a great caloric consumption, at least 250-300 kcal per session, twice a week, along with a change in active lifestyle. Regarding swimming, the most practised sport, children should be sent at an early age (4-5 years old) in order to anticipate learning of the athletic gesture, and to do this at an age in which obesity and overweightness have less an incidence.

Body Mass Index↗
PubMed1994

Final height in girls with central precocious puberty: comparison of two different luteinizing hormone-releasing hormone agonist treatments.

In order to evaluate the effects of two long-acting luteinizing hormone-releasing hormone agonists on growth, bone maturation and final height in girls with central precocious puberty, we analyzed growth data from 40 girls (15 treated with buserelin intranasal spray (group A), 15 treated with triptorelin depot im every 28 days (group B) and 10 untreated (group C)). Patients in group A started treatment when chronological age (CA) was 7.7 +/- 0.9 years, bone age (BA) was 10.2 +/- 1.1 years and height was 131.9 +/- 5.0 cm. Patients in group B started therapy when CA was 7.6 +/- 0.5 years, BA 9.8 +/- 1.0 years and height 133.2 +/- 7.6 cm. The diagnosis of untreated patients (group C) was made when CA was 7.2 +/- 0.9 years, BA 9.6 +/- 2.2 years and height 130.2 +/- 8.6 cm. Both luteinizing hormone-releasing hormone agonists appeared to control precocious puberty. Final height in group B (160.6 +/- 5.7 cm) was significantly higher than that of group A (153.2 +/- 5.0 cm: p < 0.05) and group C (149.6 +/- 6.3; p < 0.01), whereas the difference between groups A and C was not statistically significant. In group B a positive difference was observed between final height (160.6 +/- 5.7 cm) and target height (157.6 +/- 5.9 cm) (ns); on the contrary, in groups A and C, final height was lower than target height (155.5 +/- 5.3 and 156.4 +/- 1.3 cm, respectively), but only in group C the difference was statistically significant (p < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Intranasal↗
PubMed1993

Childhood obesity treatment: double blinded trial on dietary fibres (glucomannan) versus placebo.

Dietary fibres are frequently used for the treatment of paediatric obesity. The aim of this clinical trial is to evaluate the efficacy of glucomannan in the child obesity management. This experimental design was double blinded with a block randomisation, alpha = 0.05, beta = 0.2 and delta = 50%. The study involved 60 children under 15 years of age (mean age 11.2 years, mean overweight 46%), 30 of them under glucomannan treatment (1 g twice a day for two months) and 30 under placebo and the same schedule. The drug and the placebo were indistinguishable both for the family and the physician. During the two months study period the children followed a normocaloric diet evaluated every two weeks by a dietetic record book. At the beginning of the study the drug and the placebo groups were comparable in regards to anthropometric data. At the end, the mean overweight of the drug group was decreased from 49.5% to 41% and that of the placebo group from 43.9% to 41.7%. Both decreases were significant (p < 0.01), but no significant difference was observed between the drug and the placebo groups. The only significant difference concerned the lipid metabolism. The children under glucomannan treatment manifested a significant decrease of alpha-lipoprotein and an increase of pre-beta-lipoprotein and triglycerides; the children under placebo manifested only a decrease of triglycerides and apo beta-lipoprotein. We suggest that this metabolic alteration may derive from a primary decrease of alpha-lipoprotein, most likely because of an inadequate water intake.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Hyperthyroid-induced chorea in an adolescent girl.

Hyperthyroidism is invariably accompanied by nervous system dysfunctions. Irritability, emotional lability and hyperkinesia are the signs and symptoms most frequently observed. Chorea or choreoathetosis are only rarely associated with hyperthyroidism. It is the purpose of this work to describe the case of a young girl in whom chorea was the main manifestation of thyrotoxicosis. The chorea receded and disappeared as the patient became euthyroid. Hyperthyroidism, therefore, is to be considered an unusual cause of chorea and every patient with choreiform movements should be examined also for thyroid function.

Adolescent↗