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  • 1
    ISSN: 0022-4731
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Growth hormone ; Precocious puberty ; Growth ; Optic nerve diseases ; Radiotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Hypothalamo-pituitary function in children with optic glioma may be impaired by the tumour itself and by the high cranial radiation doses used in treatment. This study evaluates the effect of optic glioma and its treatment on patient growth and pubertal development. Twenty-one patients (13 boys, 8 girls), treated for optic glioma by cranial irradiation (45–55 Grays) at a mean age of 5.4 years, were evaluated before (n=10) and/or after (n=21) irradiation. Growth hormone (GH) deficiency was present in only 1 patient tested before irradiation and in all patients after irradiation. Precocious puberty occurred in 7/21 cases, before irradiation in 5 patients and after irradiation in 2 patients. The cumulative height loss during the 2 years after irradiation was 0.2±0.2 SD (m±SEM) in 7 patients with precocious puberty and 1.1±0.2 SD in 14 prepubertal patients (P〈0.01). The corresponding bone age advance over chronological age, evaluated 1–3 years after irradiation, was 1.1±0.5 and −0.7±0.3 year in the two groups (P〈0.01). The mean height loss between time of irradiation and the final height was 2.3±0.6 SD (n=6). Primary amenorrhoea, associated with low oestradiol levels, occurred in two of the three girls of pubertal age. These data indicate that the high dose of cranial radiation used to treat optic glioma invariably results in GH deficiency within 2 years and that hGH therapy is required when GH deficiency is documented. Precocious puberty, resulting in apparently normal growth velocity in spite of GH deficiency, should be treated with luteinizing hormone-releasing hormone analogues because of the risk of accelerated bone maturation and reduced final height.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Cranial irradiation ; Brain tumour ; Growth hormone ; Sleep ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The effects of radiotherapy on subsequent growth hormone (GH) secretion and sleep organization was studied by simultaneous evaluation of spontaneous sleep-related GH secretion (SSRGHS), arginine-insulin tolerance test (AITT) and polygraphic sleep recording in a follow up of 19 children who underwent surgical removal of an intracranial tumour and cranial radiotherapy 1–7 years previously. Electrophysiological patterns of sleep phases, the amount and distribution of sleep stages were normal, suggesting that the procedure utilized should not entail modifications of SSRGHS per se. Long-term after radiotherapy SSRGHS and AITT GH responses were lower than those of the medium-term group. The lack of correlation between SSRGHS and AITT GH secretion suggests that SSRGHS and AITT measure different aspects of GH secretion. The highest value of GH plasma level (peak) corresponded to slow wave sleep, mainly during the first non-rapid eye movement-rapid eye movement (NREM-REM) cycle, in practically all the patients. Although the SSRGHS synchronization with SWS of the first NREM-REM cycle was preserved, no sleep measure was correlated with the SSRGHS. The usefulness of polygraphic monitoring of sleep patterns was confirmed.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Key words Cranial irradiation ; Growth factors ; Growth hormone ; Growth hormone deficiency ; Leukaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The dose of prophylactic cranial irradiation given to patients for acute lymphoblastic leukaemia has been decreased from 24 to 18 Gy, but the beneficial effect of this decrease on growth is controversial. This study compares the growth hormone (GH) secretion and growth of 35 patients (20 boys) given 18 Gy at 3.7 ± 0.3 (SE) years, and routinely evaluated 5.4 ± 0.4 years after irradiation to define the indications for GH treatment in these patients. Of these, 63% had a low GH peak (〈10 μg/l) after one (22 cases) or two (17 cases) stimulation tests. The plasma concentrations of insulin-like growth factor I and its GH-dependent binding protein were normal for age in all but two cases. The height changes between irradiation and evaluation were correlated with the GH peaks (P 〈 0.03) and were concordant, except in patients with early puberty. This occurred in 16 patients including all 12 girls irradiated before 4 years of age. A significant (P 〈 0.03) reduction in height (SD) between irradiation and adult height occurred in untreated GH-deficient patients (−1 ± 0.3, n = 6), but not in GH-deficient patients given GH (−0.6 ± 0.3, n = 8) or in those with normal GH peak (−0.4 ± 0.3, n = 7). Conclusion In children irradiated for acute lymphoblastic leukaemia, GH deficiency is frequent after 18 Gy but its impact on adult height is smaller than after higher doses. We suggest that the indications for gonadotropin releasing hormone analogue therapy should be broad in patients with early or rapidly progressing puberty and those for GH therapy in those patients with a below average constitutional height before irradiation.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1076
    Keywords: Puberty ; precocious ; Growth disorders ; LH-FSH releasing hormone therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The factors influencing the final height of central precocious puberty patients treated with gonadotrophin releasing hormone (GnRH) analogues remain a critical issue. This study compares the predicted final height before and after GnRH analogue therapy to identify predictive factors for final height. Fourteen girls with idiopathic central precocious puberty were treated with a GnRH analogue. All had an active non-regressive form before therapy, full and permanent suppression of oestrogenic activity during therapy (duration 〉2 years, 3.1±0.3 years, mean ±SEM), and the pubertal pituitary-ovarian axis had normalized in all of them 1 year after the cessation of therapy. The mean predicted final height increased from 152±1.8 cm before therapy to 162.2±1.2 cm (P〈0.01) at the last evaluation performed 4.5±0.3 years after the onset of therapy. The mean gain in predicted final height between the onset of therapy and the last evaluation was 10.2±1.1 cm. It was correlated with the following data recorded at the onset of therapy: bone age advance over chronological age (r=0.66,P〈0.02), predicted final height at the onset of therapy (r=−0.76,P〈0.001), and the difference between the target height and the predicted height at onset of therapy (r=0.76,P〈0.001). We conclude that GnRH analogue therapy is more likely to improve final height prognosis in girls who initially present with a markedly advanced bone age and a great difference between their target and predicted heights. Both these parameters reflect the severity of the disease at diagnosis.
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  • 6
    ISSN: 1433-0350
    Keywords: Key words Cranial tumor ; Growth ; Growth hormone ; Neurosurgery ; Puberty ; Radiotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Intracranial lesions may affect hypothalamic-pituitary (HP) function and growth in several ways, depending on the location of the lesion within this area, the presence or absence of secondary hydrocephalus, and/or treatment of the lesion by surgery and/or radiotherapy. The lesion may cause a deficiency of HP hormones or, conversely, activation of the HP-gonadal axis leading to precocious puberty. Growth hormone (GH) deficiency is the most frequent endocrine abnormality that results from the lesions of the HP area. There has been progress in diagnosis, patterns of replacement therapy and the administration of biosynthetic GH in association with gonadotropin-releasing hormone analogues in precocious puberty. The major problem in these patients is the dramatic increase in their weight, which frequently occurs after surgery and increases their psychosocial and physical disabilities. It may be due to the hyperinsulinism caused by the lesion. This hyperinsulinism may be the factor that replaces GH in stimulating growth factor production and leads to normal growth in some of the patients.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Eight cases of hypothalamic hamartomas (HH) diagnosed in children by clinical, biological and CT studies have been explored by MRI and compared with a control group of twenty children without hypothalamohypophyseal signs. MRI revealed the hamartoma in all cases. Its location and position relative to the floor of the third ventricle was precisely determined. The signal intensity of the lesion was identical to that of normal grey matter on T1-weighted images (300/600.28), but an hyperintense signal was observed over T2-weighted images (1800.60/120) in seven of the eight cases. These data suggest that HH are somewhat different in structure from normal brain tissue. MRI variations in signal intensity should be taken into account in the diagnostic work-up of these lesions.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract MR anatomy of hypothalamo-hypophyseal axis is well established. However data about pituitary gland height (PGH) in children are sparse. A retrospective study was therefore performed in 60 children (30 boys and 30 girls) aged from 8 days to 21 years. All these children had MR for various neurological diseases. Patients with hypothalamo-hypophyseal disease and intracranial hypertension were excluded. The PGH was measured on a strict midline sagittal T1 weighted scan 3 to 7 mm thick. A positive linear correlation was found in children aged from 1 year to puberty followed by a plateau. In the first year of life a negative linear correlation was found. A positive linear correlation was found between PGH and statural height as well.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 0942-0940
    Keywords: Infant ; brain tumours ; irradiation ; sequelae
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Between 1975 and 1989, 98 children with brain tumours under the age of three at time of diagnosis were entered into a retrospective study. Twenty of them are alive and free of tumour more than five years after treatment and were evaluated in this study. Thirteen tumour localizations were infratentorial and 7 were supratentorial. A histological examination was performed in 15 patients: 5 ependymomas, 6 medulloblastomas and 4 astrocytomas were identified. Fifteen patients underwent surgical removal of tumour, all but one received radiotherapy and 8 were given chemotherapy. Only two children have not late effects. Analysis of long-term sequelae in survivors showed central endocrinopathies in 14 (70%), a neurological handicap in 13 (65%) and impaired cognitive functions in 17 (85%). Irradiation was clearly responsible for mental sequelae in 7 patients and endocrinopathies in 6 patients. The other possible causes are tumour injury, hydrocephalus or surgery. The risks incurred with radiotherapy and advances in infant brain tumour therapy are discussed.
    Type of Medium: Electronic Resource
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