Search PubMed⌕ Search

Biomedical subjects

A A Massarano

Publications and source records attributed to A A Massarano.

11 recordsLinked to original sources
PubMed2001

Symptomatic adrenal insufficiency during inhaled corticosteroid treatment.

Symptomatic adrenal insufficiency, presenting as hypoglycaemia or poor weight gain, may occur on withdrawal of corticosteroid treatment but has not previously been reported during inhaled corticosteroid treatment. This case series illustrates the occurrence of clinically significant adrenal insufficiency in asthmatic children while patients were on inhaled corticosteroid treatment and the unexpected modes of presentation. General practitioners and paediatricians need to be aware that this unusual but acute serious complication may occur in patients treated with inhaled corticosteroids.

Administration, Inhalation↗
PubMed1998

Gonadotrophin pulsatility in girls with the Turner syndrome: modulation by exogenous sex steroids.

OBJECTIVES: The endocrine manifestation of puberty, nocturnal pulsatile secretion of gonadotrophins precedes the physical manifestations by 2 years. Whether gonadal steroids and inhibin have a role to play in the regulation of pulsatile gonadotrophin release is unclear. The agonadal model, girls with Turner's syndrome (TS), has been used to determine the role of the hypothalamic pulse generator in the ontogeny of gonadotrophin secretion in man. We evaluated the ontogeny of gonadotrophin secretion in TS girls with respect to amplitude and frequency and compared these results to those obtained in a group of normal girls. The effects of treatment with ethinyloestradiol (EE2) or oxandrolone (OX) on parameters of gonadotrophin secretion were also evaluated. PATIENTS: We studied 32 girls with TS, aged 4.3-12.4 years. All were prepubertal at the start of the study and longterm follow up revealed that none entered spontaneous puberty. The pulse amplitude and frequency was evaluated and compared to the results obtained in 23 normal girls, aged 4.9-12.8 years who acted as controls. MEASUREMENTS: Samples were taken at 20 minute intervals for 24 h for the measurement of serum concentrations of luteinising (LH) and follicle stimulating (FSH) hormones. The girls were than randomized to receive EE2 or OX and were then re-admitted 6 months into the course of the treatment for a repeat 24 h serum profile of LH and FSH levels. RESULTS: The girls with TS showed a clearly defined dominant pulse periodicity of 180 min and that in the normal cohort was 160-220 min. The girls with TS had an increased oscillatory activity between 120 and 260 min compared to the normal. Mean 24 h serum gonadotrophin concentration in TS girls was always higher than in the normal cohort. The inflection points of the fitted polynomial regression equation relating sex hormone concentration with age was similar for the two groups. EE2 lead to a significant change in pulse periodicity in TS girls but OX had no significant effect on the pulse periodicity. CONCLUSION: These results demonstrate that girls with Turner syndrome have gonadotrophin pulse periodicity in the prepubertal years similar to those of normal girls. The oscillatory activity was much greater in girls with Turner syndrome at all ages in the prepubertal years, suggesting a role for the ovary in modulating gonadotrophin secretion in the prepubertal years. Our data confirm that in girls with Turner syndrome the normal pattern of gonadotrophin secretion evolving with time is preserved.

Anabolic Agents↗
PubMed1997

Final height outcome in girls with Turner syndrome treated with a combination of low dose oestrogen and oxandrolone.

Final stature in girls with Turner syndrome treated with combination of low dose oestrogen and oxandrolone. Nineteen prepubertal girls with Turner syndrome (mean age 10.9 years, range, 8.9-14.2 years) were randomly assigned to receive either oxandrolone (0.05 mg/kg/day) or ethinyloestradiol (40 ng/kg/day) for 1 year. Subsequently the alternate therapy was added and the combination given until attainment of final height. Ethinyloestradiol was gradually increased at the age of 12.5 years in order to induce secondary sexual characteristics. The duration of treatment was a mean of 5.2 years (range, 3.7 years) when the 1st year of monotherapy was included. Therapy produced a sustained acceleration in growth rate for a duration of 4 years and eventually has resulted in an increment of mean adult height of 3 cm relative to pre-treatment projected height with mean values of 146.5 cm versus 143.5 cm respectively. The moderate side-effects observed did not cause any of the girls to discontinue treatment. Nevertheless, amelioration of adult height appears to be modest, notably in comparison to published data of growth hormone treatment and 4 girls had a decrease in final height prediction. CONCLUSION Combination of low dose of oxandrolone and oestrogen may have a moderate but positive impact on final height in girls with Turner syndrome. However, some girls do worse than predicted in term of final height using this regimen. Oestrogen therapy started at low dose around the age of 10 years and increased gradually at approximately 12.5 years to induce secondary sexual characteristics does not have a deleterious effect on adult height in Turner syndrome. In summary, low dose oxandrolone-oestrogen treatment was found to accelerate the tempo of growth in girls with Turner syndrome, but did not appear to have a consistent effect on final height.

Adolescent↗
PubMed1996

Noonan syndrome: coagulation and clinical aspects.

The study aims were to review the clinical features of a group of patients with Noonan syndrome and to further elucidate their bleeding tendency. Eighteen patients (12M, 6F) aged 2.6-13.3 years underwent a clinical assessment, a questionnaire of their bleeding tendency and laboratory coagulation studies. Nine had cyanotic spells or breathing difficulties after birth; 11 had poor feeding or weight gain. Increased bruising or bleeding was reported in 12 (67%), four of whom had bleeding from the oral cavity. Excessive bleeding was not reported from operative procedures in other sites. Partial thromboplastin time was prolonged in 10 (56%) associated with low levels of clotting factors, particularly XI and XII. Bleeding times were normal; one had marginal thrombocytopenia. Coagulation results did not correlate with bruising history and may not predict bleeding risk. Care is required when Noonan syndrome patients undergo surgery, particularly of the oropharynx, with immediate availability of suitable blood products.

Adolescent↗
PubMed1993

Growth in atopic eczema.

Growth was studied in 68 children aged 2-12 years with atopic eczema. Height SD scores were significantly correlated with the surface area of skin affected by eczema. The mean height of 41 patients with less than 50% of their skin surface affected (group I) was normal (mean SD score -0.11). The 27 children with more than 50% of their skin affected (group II) were significantly shorter (SD score -0.83) and were also short allowing for their parental target height. The predicted heights were also normal in group I but were lower than expected in group II. Regression analysis suggested that height was most dependent on parental height. The extent of the disease had a significant additional effect, whereas dietary treatment and treatment with topical steroids had only marginal additional effects. The growth of children with eczema affecting less than 50% of the skin surface area appears to be normal, and impaired growth is confined to those with more extensive disease.

Age Determination by Skeleton↗
PubMed1992

Growth hormone secretion in patients with Turner's syndrome as determined by time series analysis.

Twenty-four-hour growth hormone (GH) profiles in 26 girls with Turner's syndrome were compared with those of 26 normally growing short children and 24 slowly growing short children. All children were prepubertal and below 12 years of age. A subgroup of 13 girls was treated with ethinyl estradiol and a 24-h GH profile was reassessed. In an additional group of 45 girls with Turner's syndrome (aged 6.7-18.9 years) the effect of age, spontaneous breast development and ethinyl estradiol treatment was studied. The profiles were assessed by Fourier analysis. The oscillatory activity and the mean 24-h GH concentration were similar in children with Turner's syndrome and the normally growing short children, in contrast to lower levels in the slowly growing short children. The periodicity of GH secretion was similar in all groups. In the longitudinal study, ethinyl estradiol treatment resulted in a significant increase in pulse amplitude, but not in periodicity. In the cross-sectional study there was no significant difference between the subgroups of girls with either presence or absence of breast development or ethinyl estradiol treatment. GH secretion was not significantly related to age, height in standard deviation score or height velocity. These data imply that there is no abnormality in GH secretion in girls with Turner's syndrome.

Aging↗
PubMed1989

Ovarian ultrasound appearances in Turner syndrome.

We performed pelvic ultrasound assessment in 104 patients with Turner syndrome aged 0.2 to 17.4 years; 69 had the 45,X karyotype and 35 had variant karyotypes. Ovarian appearances were classified as "streak" (n = 70, including 30 patients in whom no ovary could be seen) or "nonstreak" (n = 34). The nonstreak ovaries ranged from small glands, sometimes containing minute cysts, to ovaries indistinguishable from those which are normal for age. Nonstreak ovaries retained a range of function, as evidenced in some cases by spontaneous breast development and uterine enlargement. The proportion of nonstreak ovaries followed a U-shaped pattern, with a nadir from 4 to 10 years; this follows the known biphasic pattern of luteinizing hormone and follicle-stimulating hormone secretion. Only those patients with karyotype variants in which the long arm of the X chromosome was retained fared better than those with the 45,X karyotype.

Adolescent↗
PubMed1989

Growth hormone secretion in Turner's syndrome and influence of oxandrolone and ethinyl oestradiol.

We investigated 24 hour growth hormone secretion by intermittent 20 minute blood sampling in 34 prepubertal patients with Turner's syndrome, aged 4.3-12.4 years. Growth hormone profiles were analysed by the PULSAR programme and results expressed as the sum of growth hormone pulse amplitudes. Six patients had abnormal growth hormone pulse frequencies. In the remaining 28, growth hormone pulse amplitudes declined significantly with increasing age, but there was no correlation between growth hormone pulse amplitudes and growth rates. Concentrations of insulin like growth factor-1 (IGF-1) rose with age but did not correlate with either growth rates or growth hormone secretion. Fifteen patients were given oxandrolone and 11 low dose ethinyl oestradiol. Both agents increased height velocity without increasing growth hormone secretion. We conclude that the relation between growth hormone secretion and growth in Turner's syndrome is less certain than in normal children. End organ resistance is probably due to a skeletal dysplasia. Both oxandrolone and low dose ethinyl oestradiol improve the growth of girls with Turner's syndrome, but their mechanism of action remains uncertain.

Body Height↗