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Biomedical subjects

R Stanhope

Publications and source records attributed to R Stanhope.

At least 145 records · Page 8Linked to original sources
PubMed1987

The effect of total body irradiation and bone marrow transplantation during childhood and adolescence on growth and endocrine function.

Seventeen children (11 M, 6 F) with acute leukaemia and myeloproliferative disorders were investigated for growth and endocrine dysfunction. All had undergone bone marrow transplantation prepared with cyclophosphamide and single fraction total body irradiation (900-1000 cGy) between 1.5 and 3.8 (mean 2.2) years previously. The majority of children exhibited growth failure, which was of multiple aetiology. Ten patients, of whom eight had had previous prophylactic cranial irradiation, had evidence of growth hormone deficiency based on the reduced growth hormone response to insulin induced hypoglycaemia. Three patients had evidence of hypothalamic damage as shown by their growth hormone response to 200 micrograms GHRH (1-29) NH2 intravenously. Gonadal failure was common, assessed clinically, and biochemically by basal gonadotrophin and sex steroid concentrations. All four girls of adolescent age (10.6-14.1 years) had ovarian failure requiring sex steroid replacement. Of the eight boys of adolescent age (12.3-18.3 years), two had testicular failure requiring sex steroid supplements. Both of these had had previous testicular irradiation. Five others had compensated gonadal failure, and one had normal Leydig cell function. Abnormalities of the TSH response to TRH occurred in 10 patients but only three had overt hypothyroidism. Unlike growth hormone deficiency, gonadal and thyroid dysfunction showed no correlation with previous cranial radiotherapy.

Adolescent↗
PubMed1987

Physiological growth hormone secretion during slow-wave sleep in short prepubertal children.

The usefulness of a limited nocturnal GH profile has been evaluated for the assessment of physiological GH secretion. We have analysed the complete overnight GH and sleep profiles of 20 short prepubertal children, sampled at 15-min intervals. The mean age was 9.1 years (range 4.3-11.7 years) and mean height velocity standard deviation score (SDS)-1.0 (range -2.4 to +0.4). The sleep stage in which the maximal GH value was reached varied considerably between the patients. Only 55% achieved the maximal GH value during the first slow-wave sleep period, and 65% in the first 2 h of sleep. The maximal values during the first slow-wave period correlated weakly with the sum of the nocturnal peak values and the total area under the curve of the complete GH profile. None of these parameters correlated with height velocity. We conclude that at present there is no substitute for complete overnight or 24 h GH profiles for the assessment of physiological GH secretion, but the clinical significance of the variations seen remains unclear.

Body Height↗
PubMed1987

Cushing's disease and craniopharyngioma.

A 14-year old girl presented with growth failure and Cushing's disease. Histological examination confirmed a craniopharyngioma but failed to show that the tumour secreted adrenocorticotrophic hormone. We suggest that her Cushing's disease was caused by hypothalamic dysfunction associated with increased corticotrophin-releasing hormone secretion, secondary to the craniopharyngioma.

Adolescent↗
PubMed1987

Precocious and premature puberty associated with treatment of acute lymphoblastic leukaemia.

Early puberty in 28 children (23 girls, five boys) treated for acute lymphoblastic leukaemia (ALL) at a mean age of 4.0 years (range 1.4-7.8) is described. All but one had received prophylactic cranial irradiation (1800-2400 cGy) and three children had received additional cranial or craniospinal irradiation as treatment for relapse of their leukaemia. Mean age for the onset of puberty was 8.8 (SD 0.8) years in the girls and 9.3 (0.8) years in the boys; this is greater than two standard deviations from the mean for normal girls and boys. Five children (three girls, two boys) had precocious puberty. The onset of puberty occurred at greater than two standard deviations from the mean for normal girls and boys in 14(13%) girls and 4(3%) boys treated at less than eight years of age between 1970 and 1985. In a group of 55 girls treated for ALL who had survived in first remission for six years or more from diagnosis, there was a relation between young age at onset of treatment and early menarche. We suggest that premature activation of the hypothalamic-pituitary-gonadal axis occurs as a consequence of hypothalamic dysfunction due to cranial irradiation. Precocious and premature puberty in children treated for ALL may be an important factor in contributing to short stature.

Age Factors↗
PubMed1987

Changes of skinfold thickness during puberty induced by pulsatile gonadotrophin-releasing hormone therapy.

We have analysed the changes in triceps and subscapular skinfold thickness ratios of 66 (46 male, 20 female) children with constitutional delay of growth and puberty who progressed through puberty spontaneously and 16 (7 male, 9 female) children with delayed puberty who were treated with pulsatile gonadotrophin-releasing hormone (GnRH). The pattern of change in skinfold thickness in both groups were identical and indistinguishable from standards of normal children when later chronological ages of the patients were allowed for. We observed a decrease in skinfold ratio just prior to the onset of puberty in boys, and such an assessment may be a useful early predictor of the onset of puberty. Our data provided further evidence that all aspects of human puberty are entirely GnRH-dependent.

Adolescent↗
PubMed1987

Oxandrolone induces a sustained rise in physiological growth hormone secretion in boys with constitutional delay of growth and puberty.

We describe the treatment of 3 boys, mean age 14.9 years (range 13.8-16.1 years) with constitutional delay of growth and puberty using oxandrolone in two dose regimens, 2.5 or 1.25 mg/day for 3 months. Treatment induced an increase in mean height velocity from 5.1 to 8.5 cm/year; this was sustained at 8.8 cm/year in the period following treatment. Growth hormone (GH), luteinising hormone and testosterone secretion were assessed by overnight or 24-hour venous sampling at 15-min intervals before, during treatment and after cessation of treatment. A computer programme was used to analyse GH secretory dynamics. The 3 boys had a mean sustained increase in total GH secretion of 190%. The increase in GH secretion was associated with an increase in amplitude of GH pulses rather than an alteration in pulse frequency.

Adolescent↗
PubMed1986

Studies of gonadotrophin pulsatility and pelvic ultrasound examinations distinguish between isolated premature thelarche and central precocious puberty.

We have studied the pulsatile secretion of gonadotrophins at night and made ovarian ultrasound examinations in three girls with central precocious puberty and three with isolated premature thelarche. The three girls with precocious puberty had well-defined pulsatile secretion of LH and FSH with LH predominating, as would be expected in normal puberty. Pulsatile secretion of gonadotrophins was also seen in girls with premature thelarche but the pattern was reversed. In girls with precocious puberty, large "multicystic" ovaries and large uteri were seen on ultrasound examination, whereas girls with isolated premature thelarche had small uteri and ovaries with less than four "cysts" up to 15 mm in diameter. These data provide the key to understanding the aetiology of isolated premature thelarche.

Breast↗
PubMed1986

Energy balance in low birth weight infants fed formula of high or low medium-chain triglyceride content.

We tested the effect on energy balance of the partial substitution of medium-chain for long-chain triglycerides in the diet of growing low birth weight infants. Fifteen infants were studied in a randomized double-blind crossover clinical trial in which each infant was fed each of two formulas, which were of equal gross energy and protein content but differed in fat composition. The high medium-chain triglyceride (MCT) formula contained medium- and long-chain triglycerides in a weight/weight ratio of 46:54; in the low MCT formula the ratio was 4:96. The hypothesis tested was that under conditions of equal gross energy intake the two diets would differ in their digestible and metabolizable energy contents and would produce differences in the infants' rates of energy expenditure and energy storage. Gross energy intakes averaged 562 and 555 kJ/kg/day (134 and 133 kcal/kg/day) with the high and low MCT formulas, respectively. With each diet, coefficients of energy digestibility (0.93) and metabolizability (0.91) were identical; the rates of energy expenditure were 262 (high MCT) and 265 (low MCT) kJ/kg/day (63 kcal/kg/day for both diets), and of energy storage were 246 (high MCT) and 239 (low MCT) kJ/kg/day (59 and 57 kcal/day). These differences were not significant. There were also no significant differences between the two diets in coefficients of nitrogen retention (mean 0.70) or in rate of weight gain (mean 21.5 gm/kg/day). The use of high MCT content in infant formula neither provided a nutritional advantage in energy digestibility or metabolizability nor resulted in an increased rate of energy expenditure or of energy storage.

Body Weight↗
PubMed1986

Tall stature: a clinical, endocrinological and radiological study.

We have studied GH secretory dynamics in 38 (25 F, 13 M) children referred for investigation and treatment of tall stature (Height SD score (SDS) greater than 1.8). Five children had elevated basal GH levels (mean 20.4 mU/l) and a further four had abnormal GH responses to exogenous TRH. Two other children had paradoxical GH responses to oral glucose loading. Although one of these was receiving high dose testosterone to limit final height. All these children were pubertal and did not show concordance between peak GH concentration observed after insulin-induced hypoglycaemia and during stage IV sleep. Twenty-four hour GH profiles revealed increased GH pulse amplitude and area under the pulse during puberty. Morning GH secretion was also regularly observed and it is possible that the paradoxical responses seen by us and reported by others reflect this increased physiological secretion. Pituitary radiology revealed morphological abnormalities suggestive of microadenomata in 17 patients but these were all in late puberty and these changes may be related more to the effects of sex hormones than to somatotroph pathology. 'Abnormal' endocrinology and radiology are seen in tall children in puberty and must be interpreted with caution.

Body Height↗
PubMed1986

High resolution CT scanning of the pituitary gland in growth disorders.

We have performed 217 GEC 8800 CT scans of the hypothalamus and pituitary glands of 202 children with disorders of growth and development. Pituitary morphological abnormalities were common. Intrapituitary low density lesions were found in 17% of the whole series and in 58% of children with tall stature. Seventy-seven children with idiopathic growth hormone deficiency could be divided on the basis of pituitary morphology seen on CT scan into pituitary aplasia (n = 11) and pituitary hypoplasia (n = 53). Patients with pituitary aplasia had an absent adenohypophysis which probably dated from early intrauterine life and therefore could not be related to birth trauma. We have found a high incidence of evolving endocrinopathy in children with pituitary insufficiency: thus, if a short child is investigated the initial endocrine findings need to be repeated as the pattern of pituitary insufficiency changes with time. An evolving endocrinopathy starting in later childhood is suggestive of the presence of a cerebral tumour. Children with subnormal growth velocities and a normal growth hormone response to pharmacological tests have a wide spectrum of pituitary morphological abnormalities which may be associated with growth hormone neurosecretory dysfunction.

Adolescent↗
PubMed1986

Diabetes insipidus and occult intracranial tumours.

We describe four patients with intracranial tumours who presented with diabetes insipidus and subsequently developed an evolving anterior pituitary endocrinopathy. The delay in imaging the tumours varied between 2.1 and 11.2 years (mean 5.4 years).

Brain Neoplasms↗