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  • 1
    ISSN: 1432-0827
    Keywords: Idiopathic juvenile osteoporosis ; Osteoblast function ; Osteocalcin ; 1,25(OH)2D3 administration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine , Physics
    Notes: Summary Idiopathic juvenile osteoporosis (IJO) is a rare form of bone demineralization that occurs during childhood. The mechanism of bone loss is unclear. Some bone hystomorphometric studies have found osteoblast failure and decreased bone formation in the affected patients whereas others have reported increased bone resorption. To elucidate this issue, we studied osteoblast function in six patients with IJO (five males, one female; aged 2.3–14.6 years) and five healthy sex- and age-matched subjects (four males, one female; aged 2.0–15.1 years) measuring serum values of osteocalcin under basal condition and during an osteoblast stimulation test performed by oral 1,25-dihydroxyvitamin D3 [1,25(OH)2D3] administration (1.8 μg/1.73 m2/daily). After a baseline day (day 0), all the subjects (patients and controls) received 1,25(OH)2D3 in four divided doses for 6 days (days 1–6). Fasting blood samples were obtained every morning (0800 h) for the determination of serum osteocalcin. Baseline osteocalcin levels were not significantly different between IJO and controls (13.58±6.05 ng/ml versus 16.04±5.09 ng/ml, respectively) even if two patients had low osteocalcin values. During 1,25(OH)2D3 administration, serum osteocalcin values significantly increased (P〈0.001) from baseline in both children with IJO and controls, reaching peak values not significantly different in the two groups. Our results do not support the hypothesis that defective osteoblast function is the primary factor of bone demineralization in IJO.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Calcified tissue international 54 (1994), S. 195-197 
    ISSN: 1432-0827
    Keywords: Corrected calcium ; Parathyroid hormone-related protein ; Pregnancy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine , Physics
    Notes: Abstract The object of this study was to determine whether increased circulating levels of parathyroid hormone-related protein (PTH-rp) may explain the increased parathyroid hormone (PTH) bioactivity in pregnancy. In 41 healthy pregnant women (age 19–41 years), PTH-rp and corrected calcium levels were measured and compared with those of nonpregnant control women (n=18, age 20–39 years). PTH-rp and corrected calcium levels were significantly higher in pregnant women (PTH-rp 21.9±7.9 pg/ml, P〈0.001; corrected calcium 2.38±0.07 mmol/liter, P=0.001) than in nonpregnant women (PTH-rp 10.3±7.8 pg/ml; corrected calcium 2.30±0.10 mmol/liter). Our data indicate that circulating PTH-rp levels may significantly increase in pregnancy, suggesting a possible role of this peptide in the modification of calcium homeostasis in pregnant women.
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  • 3
    ISSN: 1432-0827
    Keywords: Key words: Adolescent females — Androgen receptor blockade — Bone mineral density — Flutamide — Idiopathic hirsutism — Peripheral androgen hypersensitivity.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine , Physics
    Notes: Abstract. The effect of peripheral androgen hypersensitivity on bone mineral density (BMD) was investigated in a group of adolescent women with idiopathic hirsutism (n= 17; mean age 17.0 ± 1.7 years). The effect of long-term androgen-receptor blockade with flutamide (500 mg daily in two divided doses for 12 months) on BMD was assessed too. BMD was measured at lumbar spine (L2–L4) by a dual energy X-ray densitometer. Before flutamide treatment, patient BMD (1.14 ± 0.07 g/cm2) was not significantly different from that of the control group (1.16 ± 0.12 g/cm2, n= 22), and was normal for age and sex (BMD 0.14 ± 0.69 SDS, P= NS vs. 0). After 12 months of treatment, absolute BMD in patients increased (1.18 ± 0.08 g/cm2, P 〈 0.002), but SDS BMD did not change (0.21 ± 0.72, P= NS vs. baseline). Flutamide treatment determined a clinical, marked improvement of androgen hypersensitivity (Ferriman–Gallwey score: before 22.0 ± 6.2; 6 months: 13.2 ± 6.4, P 〈 0.003; 12 months; 7.6 ± 4.1, P 〈 0.001; acne score: before 3.8 ± 0.8; 3 months 0.8 ± 0.5, P 〈 0.001; later disappeared). The serum levels of 3α-androstenediol-glucoronide decreased (before: 8.6 ± 1.1 μg/liter; 12 months: 7.2 ± 1.0 μg/liter, P 〈 0.02), whereas the other endocrinological parameters did not change. No relationship was found between BMD and clinical or biochemical parameters of hyperandrogenism. We concluded that in adolescent women, peripheral hyperandrogenism is not associated with abnormal BMD; long-term treatment with flutamide, which blocks the androgen receptor, does not alter their BMD.
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  • 4
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Growth failure with disproportionate short stature is the major clinical feature of patients with X-linked hypophosphatemic rickets (HYP). We studied the pattern of linear growth and body proportion in an untreated normally growing HYP child also affected by Klinefelter's syndrome. Auxologic data were compared with those of a HYP half-brother who showed growth failure despite long-term treatment either with vitamin D or with vitamin-D-analog plus phosphate salt supplementation. The degree of body disproportion changed from negative values to positive values in the proband, whereas it was reduced in the half-brother. We conclude that, in the proband, the normal pattern of growth and the lack of the typical body disproportion as seen in HYP patients are attributable to the concomitant presence of Klinefelter's syndrome.
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