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Biomedical subjects

R Stanhope

Publications and source records attributed to R Stanhope.

At least 73 records · Page 4Linked to original sources
PubMed1996

A new stress-related syndrome of growth failure and hyperphagia in children, associated with reversibility of growth-hormone insufficiency.

BACKGROUND: Growth failure without organic aetiology but associated with behavioural disturbance and psychosocial stress has been termed psychosocial short stature. This condition is not a valid diagnostic entity, but encompasses failure to thrive, stunting secondary to chronic malnutrition, and idiopathic hypopituitarism. Some children show spontaneous catch-up growth when removed from the source of stress, without further treatment, but until now precise definition of this subgroup for the purpose of clinical identification has not been possible. METHODS: Hospital-referred children with growth failure unrelated to organic pathology, who came from stressful homes, were compared with children of short-normal stature identified from an epidemiological survey (n = 31). Growth-hormone dynamics were studied in the hospital group by a combination of diurnal profiles and provocation tests. The tests were repeated after a hospital stay of 3 weeks away from familial stress. Standard behavioural measures were obtained from home and school. FINDINGS: In a distinctive subgroup (n = 29), growth-hormone insufficiency was associated with characteristic behavioural features, especially hyperphagia and polydipsia, and a normal body-mass index. When the children were removed from their stressful home circumstances, growth-hormone insufficiency spontaneously resolved only in formerly hyperphagic subjects. 74% of the non-hyperphagic cases (n = 23) were anorexic, with a low body-mass index and normal growth-hormone responses to provocation tests. INTERPRETATION: We present explicit behavioural and developmental criteria by which the novel syndrome of hyperphagic short stature may be recognised clinically. Such children have a capacity for spontaneous recovery of growth-hormone production on removal from or reduction of stress. Discriminant and predictive validity of the core symptoms are demonstrated. Preliminary familial studies indicate a possible genetic predisposition.

Adolescent↗
PubMed1996

Growth and endocrine sequelae of craniopharyngioma.

The growth and endocrine sequelae of 75 children (33 girls and 42 boys) with craniopharyngioma, treated from 1973 to 1994, were studied by retrospective review and by follow up assessment in 66 survivors, with a mean time from initial surgery of 6.7 years (range 1.5 to 19.8 years). Although infrequently complained of, 71% of patients had symptoms to suggest an endocrinopathy at diagnosis. After surgery, multiple endocrinopathies were almost universal, such that 75% of children had panhypopituitarism at follow up. Hypoadrenal crises in association with intercurrent illness contributed significantly to morbidity and mortality, as did the metabolic consequences of concomitant antidiuretic hormone (ADH) insufficiency and absent thirst. Final height in 25 patients was significantly below genetic target height, particularly in the girls, with loss of height potential occurring during the pubertal years. The endocrine morbidity associated with craniopharyngioma and its treatment remains high but manageable with appropriate hormone replacement. However, the combination of ADH insufficiency and an impaired sense of thirst following aggressive surgery and severe hypothalamic injury remains one of the most complex management problems.

Adolescent↗
PubMed1996

Secondary amenorrhoea after total body irradiation in pre-puberty.

Bone marrow transplant (BMT) has been used as part of the overall treatment of refractory malignant diseases. High dose cyclophosphamide and total body irradiation (TBI) are frequently used as conditioning for BMT. Initial regimens included a single fraction of TBI, with doses varying from 7.5-10 Gy, but this was associated with a high incidence of late sequelae including multiple endocrinopathies. A fractionated irradiation course over 3-4 days of a higher total dose, 12-15 Gy, of TBI is now used. Successfully treated patients with childhood cancer have an increased risk, of developing second tumours. We describe a patient successfully treated for AML who developed multiple endocrine dysfunction and a second benign ovarian tumour.

Acute Disease↗
PubMed1996

Obesity in childhood craniopharyngioma: relation to post-operative hypothalamic damage shown by magnetic resonance imaging.

OBJECTIVE: To quantify the extent of hypothalamic damage after surgery for craniopharyngioma using magnetic resonance imaging (MRI) and to relate the findings to changes in body mass index (BMI). PATIENTS: Sixty-three survivors (36 males, 27 females) of childhood cramopharyngioma were treated surgically between 1973 and early 1994. METHODS: Cranial MRI was performed at a structured follow-up assessment 1.5-19.2 yr after the initial surgery. Hypothalamic damage was scored as 0 (no visible damage), 1 (intermediate), or 2 (severe). RESULTS: After surgery there was an increase in BMI standard deviation (SD) from diagnosis to study assessment in all but 7 patients. However, patients with MRI scores of 2 (n = 17) had a significantly greater increase in median BMI SD score at follow-up (+5.5 SD score), compared with +2.5 SD score and +1.1 SD score for patients with MRI scores of 1 or 0, respectively. Of the 17 cases with MRI scores of 2, 10 had a history of extreme weight loss or weight gain at presentation; preoperative neuroimaging demonstrated extensive hypothalamic infiltration by tumor in these cases. CONCLUSION: MRI gives sufficient anatomical definition to allow assessment of the extent of hypothalamic damage and, thereby, prediction of the patients most at risk for severe post-operative weight gain.

Adolescent↗
PubMed1996

Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted?

Seventy-five children treated for craniopharyngioma between 1973 and 1994 were studied to demonstrate which pre- and intraoperative factors were indicative of a poor outcome as defined by a quantitative assessment of morbidity. This involved a retrospective review of 65 patients and a prospective study of 10 patients focused on clinical details and cranial imaging and a follow-up "study assessment" of 66 survivors performed over the last 2 years. As part of the assessment, 63 patients underwent magnetic resonance imaging with a three-dimensional volume acquisition sequence 1.5 to 19.2 years after initial surgery. Predictors of high morbidity included severe hydrocephalus, intraoperative adverse events, and young age ( < or = 5 years) at presentation. Predictors of increased hypothalamic morbidity included symptoms of hypothalamic disturbance already established at diagnosis, greater height ( > or = 3.5 cm) of the tumor in the midline, and attempts to remove adherent tumor from the region of the hypothalamus at operation. Large tumor size, young age, and severe hydrocephalus were predictors of tumor recurrence, whereas complete tumor resection (as determined by postoperative neuroimaging) and radiotherapy given electively after subtotal excision were less likely to be associated with recurrent disease. Based on these findings, the authors propose an individualized, more flexible treatment approach whereby surgical strategies may be modified to provide long-term tumor control with the lowest morbidity.

Adolescent↗
PubMed1995

Predictive factors in the determination of final height in boys with constitutional delay of growth and puberty.

Seventy-eight patients who had constitutional delay of growth and puberty were included in a retrospective study to determine whether, at the time of first evaluation, any predictive features could suggest final height outcome. Mean chronologic age was 14.3 years (range, 12 to 18 years), and all were either prepubertal or in an early stage of pubertal maturation (4 ml testicular volume). Initial mean (+/- SD) height standard deviation score was -2.74 (+/- 0.71); 85% had a relatively short spine compared with subischial leg length. Mean (+/- SD) growth rate was 4.8 (+/- 1.6) cm/year, and epiphyseal maturation was delayed by 2.4 (+/- 1) years. Sixteen boys were treated with a sustained-action preparation of testosterone (50 mg monthly for 3 to 4 months), six with oxandrolone (1.25 mg daily for a mean of 4 months), and one with both drugs in sequence. At final height attainment, 58% of the boys failed to achieve their full genetic potential; among the remaining 42%, only 0.7% attained a final height above corrected mid-parental height. The relative disproportion between the segments had no significant change at final height attainment. Regression analysis showed that final height impairment (the difference between mid-parental height and final height) was negatively influenced by standing height and growth velocity when initially evaluated and positively by the degree of segmental body proportion; that is, patients who were taller, were growing at a faster rate, and who had a major degree of segmental body disproportion with a short spine and long leg length attained a final height closer to their mid-parental height, irrespective of the degree of delayed epiphyseal maturation. Neither testosterone nor oxandrolone administered during early puberty modified final height attainment or segmental proportion. We conclude that a late onset in the timing of puberty seems to be deleterious to spinal growth and consequently to final height attainment. An alternative diagnosis should be sought among patients with features of constitutional delay of growth and puberty who do not have a significant degree of body disproportion. In these patients, as well as in those who are extremely short, who have a poor growth rate, or who have an unfavorable genetic potential, an alternative therapeutic approach may be required.

Adolescent↗
PubMed1995

Investigation of delayed puberty.

Delayed puberty is defined arbitrarily on the basis of statistical consideration, when no signs of puberty have occurred at 2.0 SD (13.4 years in girls and 13.8 in boys) above the mean chronological age for the onset of puberty. The vast majority of these patients have no endocrine abnormality and their pubertal development and growth spurt are simply consequences of primary delay (constitutional delay of growth and puberty (CDGP)) or secondary delay due to a chronic disease of childhood, such as asthma. However, a small proportion may have pathological causes of delayed puberty which must be careful identified as specific management may be required. Associated with delayed puberty, the growth spurt is always delayed which is why the condition is described as delayed growth and puberty. Short stature and lack of sexual development may lead to emotional and social difficulties and in some patients their consequences can persist when 'normal' height and full sexual maturation are attained. Recent data also suggest that a delay in the 'tempo' of pubertal maturation may interfere with the normal bone accretion occurring during puberty, later causing osteoporosis. Such findings suggest that a new approach in delayed puberty may be necessary not only for psychological reasons but also for optimizing bone mass accretion.

Adolescent↗
PubMed1995

The effect of a continuous infusion of a somatostatin analogue (octreotide) for two years on growth hormone secretion and height prediction in tall children.

OBJECTIVE: Strategies to limit the final height of tall children have centred on the use of high doses of sex steroids to advance skeletal maturation. This limits therapy to the peripubertal years whereas the greatest gain in height is in the prepubertal years. Prepubertal growth is largely GH dependent and previous work has documented modulation of GH secretion by once or twice daily subcutaneous injection of the somatostatin analogue octreotide. In this study we have determined the effects of a nocturnal infusion of octreotide on height prediction, GH and TSH secretion in tall children. DESIGN: A patient controlled study in which height prediction and 24-hour GH and TSH secretion were compared prior to and during the course of a 2-year treatment programme with a nocturnal infusion of octreotide in a dose of 1-1.5 micrograms/kg body weight given subcutaneously. PATIENTS: Nine tall children (4M; 5F) aged 7-14 years with final height predictions of 179 cm or greater in the girls and between 183 and 202 cm in the boys were studied. MEASUREMENTS: Height prediction using the Tanner-Whitehouse system prior to and at the end of 2 years of treatment. Twenty-four-hour serum GH and TSH concentration profiles, thyroxine, IGF-I and GH responses to GHRH(1-29)NH2 were studied prior to and at the end of the first year of therapy. RESULTS: Treatment with octreotide led to a significant reduction in height prediction in 7 of the 8 children who completed treatment (median reduction 3.5 cm, range +2.8 to -11.5; Wilcoxon, P = 0.05). Twenty-four-hour mean serum GH concentration decreased by 50% (MANOVA, P = 0.03) during therapy and this was accompanied by an increase in the percentage of samples giving values less than 0.5 mU/l (MANOVA, P = 0.02). There was no overall change in the serum GH response to GHRH(1-29)NH2 or serum IGF-I concentrations. Nocturnal serum TSH concentrations were reduced to levels observed during the daytime but these changes had no effect on serum thyroxine values. One patient developed gallstones during the course of therapy. CONCLUSIONS: A nocturnal infusion of octreotide reduces GH secretion and height prediction in tall children. Therapy with somatostatin analogues allows a reduction in growth rate to be instigated in the prepubertal years reducing the actual height from which will commence the pubertal growth spurt.

Adolescent↗
PubMed1995

Imperforate anus, bilateral hydronephrosis, bilateral undescended testes and pituitary hypoplasia: a variant of Hall-Pallister syndrome or a new syndrome.

A patient with multiple congenital malformations, including imperforate anus, bilateral cryptorchidism and microphallus, is described. At 4 months of age the infant had generalized convulsions and hypoglycaemia. Bilateral hydronephrosis was diagnosed at 8 months of age. At 10 months he was diagnosed as having panhypopituitarism secondary to anterior pituitary hypoplasia, shown on CT and MRI scans. This clinical picture partially resembles that of Hall-Pallister syndrome. However, the absence of some typical features such as craniofacial and limb abnormalities and, above all, hypothalamic hamartoblastoma, would also suggest the possibility of a new, previously unreported, syndrome.

Amino Acids↗
PubMed1995

The use of a computerized method of bone age assessment in clinical practice.

We assessed the reliability and repeatability of a new computerized bone age system, both versions 3.4 and 3.5 (licensed by Discerning System Inc. and Ares Service SA, Serono), able to automatically assess bone age on a left hand and wrist radiograph. This computer system is based upon Tanner and Whitehouse's method (TW2), but there are important differences. Our sample included an initial group of 40 patients who had growth delay/constitutional delay of growth and puberty (n = 10), growth hormone insufficiency/deficiency (n = 15), low birth weight/Silver-Russell syndrome (n = 9), precocious puberty (n = 6), as well as 20 patients with various skeletal dysplasias (multiple epiphyseal dysplasia n = 7, pseudoachondroplasia n = 7, acrodysostosis n = 5, achondroplasia n = 1), 7 girls with Turner syndrome, and 10 boys with nephrotic syndrome on chronic corticosteroid treatment. Multiple anthropometric readings of the same radiographs demonstrated excellent repeatability of the assessment. In addition, the number of times that a manual insertion of a grade was required was similar in four different assessments. The computerized method did not entirely avoid errors in interpretation as the position of the x-ray on the screen was critical. There was a high manual insertion rate in radiographs of children with skeletal dysplasia. However, the computer assessment system, version 3.5, performed adequately for radiographs of children with normal bone morphology and Turner syndrome and had the advantage of a continuous scale of assessment.

Adolescent↗
PubMed1995

Growth hormone, growth factors and the gonad.

During puberty, the growth spurt and stage of pubertal maturation are two processes intimately related both being dependent on the combined action of growth hormone (GH) and sex steroids. While it is well established that both GH and sex steroids are required for a pubertal growth spurt, the precise role played by GH on the timing of onset and rate of progression through different stages of pubertal maturation is not entirely clear. A better understanding of the involved physiologic mechanisms will help clinicians in the management of adolescents with growth and/or pubertal disorders.

Gonadal Steroid Hormones↗
PubMed1994

Reversibility of physiological growth hormone secretion in children with psychosocial dwarfism.

OBJECTIVE: Reversibility of GH insufficiency with a change of environment is characteristic of psychosocial dwarfism, and excludes an organic endocrinopathy. However, the change in GH pulsatility has not previously been described. We therefore wished to study spontaneous GH secretion before and after change to a more favourable environment in 11 children with psychosocial deprivation and short stature in order to evaluate if separation from the families can modify their patterns of GH secretion. PATIENTS AND DESIGN: We describe 11 prepubertal children (6 M and 5 F; 2.2-13.5 years of age) who had growth failure and psychosocial deprivation. They were diagnosed by a multidisciplinary team as having environmental growth failure after admission to hospital for 3 weeks. Six of them were discovered to have been sexually abused. During the uninterrupted hospital admission parental access was restricted. Three sets of 18-hour GH profiles were performed on each child, except one child who had only two, during the 3-week admission. MEASUREMENTS: Pulse analysis of GH profiles was by Fourier transformation. RESULTS: On the first day of admission spontaneous GH secretion demonstrated a spectrum of abnormalities in the pattern of basal values, pulse frequency and pulse amplitude. Such GH insufficiency showed reversibility during the 3 weeks in hospital. Indeed, there was a significant increase in GH secretion which was amplitude modulated without any significant modification in pulse frequency. CONCLUSION: Our data indicate that there is abnormal physiological GH secretion in children with psychosocial deprivation, which is associated with growth failure. Despite a pathological situation, each child retained his own characteristic pattern of GH pulsatility. The pattern of reversibility of abnormal GH pulsatility provides information for the mechanism of the control of GH secretion.

Adolescent↗
PubMed1994

Impaired growth in children with asthma during treatment with conventional doses of inhaled corticosteroids.

We describe 6 (4F, 2M) prepubertal children with moderate asthma diagnosed at a mean age of 2.8 years. All patients were treated with inhaled corticosteroids in a dose of between 300 and 800 mcg of beclomethasone dipropionate (becotide) daily, given either as an aerosol or rotahaler. Mean height velocity SDS decreased from -0.8 (range +0.5 to -2.0) to -3.2 (range -1.3 to -4.8) when the dose was increased. Alternatively, when the dose was reduced or stopped, mean height velocity SDS increased from -3.2 (range -2.0 to -4.8) to +0.8 (range -1.2 to +2.7). Careful assessment of height velocity is indicated in all children receiving treatment with inhaled corticosteroids.

Administration, Inhalation↗
PubMed1994

Diabetes insipidus with impaired osmotic regulation in septo-optic dysplasia and agenesis of the corpus callosum.

The clinical and endocrinological findings in 24 children with septo-optic dysplasia and/or agenesis of the corpus callosum are described with particular reference to posterior pituitary function. Nine had diabetes insipidus. The prevalence of diabetes insipidus was similar in children with complete and incomplete forms of septo-optic dysplasia. Maintenance of normal osmotic balance was very difficult in six of these children, even after the introduction of treatment with vasopressin, either as desmopressin, or lysine vasopressin spray in one of the early cases.

Abnormalities, Multiple↗
PubMed1994

Oral treatment for constitutional delay of growth and puberty in boys: a randomised trial of an anabolic steroid or testosterone undecanoate.

Thirty three boys (mean 14.6 years old, range 12.8-16.2 years) with constitutional delay of growth and puberty were randomised into two groups to determine which form of oral treatment would give the better anthropometric response. The two drugs were administered by mouth (one tablet/day) for a mean of 3.5 months (range 3-7 months). At randomisation, 17 boys received testosterone undecanoate (40 mg/day) and 16 oxandrolone (2.5 mg/day). At the start of treatment they were prepubertal or in early puberty, their height SD score was -1.97 in boys treated with testosterone and -2.21 in those treated with oxandrolone, and their growth rates were 4.3 and 4.2 cm/year respectively. Both sex steroid and anabolic steroid treatments induced a significant growth acceleration in all patients except four (three treated with testosterone and one with oxandrolone). When treated with the alternative sex steroid, all four non-responders had a significant anthropometric response. In all boys the induced growth acceleration was sustained when treatment was interrupted. There was no significant difference in the induced growth spurt and bone maturation between the two groups. Spontaneous progress into puberty was achieved in all boys with an increase in testicular volume from a mean of 4.6 to 8.5 ml. The rate of development in secondary sexual characteristics was also similar in the two groups. These data suggest that oral testosterone and oxandrolone are equally effective in the treatment of growth delay in boys with constitutional delay of growth and puberty.

Administration, Oral↗
PubMed1994

Cognitive abilities associated with the Silver-Russell syndrome.

There is no consensus opinion on whether or not cognitive impairments are found in the Silver-Russell syndrome. An investigation of a substantial sample was undertaken, using standardised assessments, in 20 boys and five girls aged 6.0 years to 11.8 years. Mean (SD) birth weights were -2.65 (0.95) SD scores, corrected for gestation. At evaluation the children had a mean (SD) age of 8.8 (1.8) years and a mean height of -2.26 (1.5) SD scores. Tests of cognitive abilities included assessments of general intelligence, reading and arithmetic attainments, and a cognitive processing task. Most had some degree of developmental delay: mean (SD) full scale IQ was 86 (24); 32% scored within the learning disability range (that is, IQ < 70); 40% were reading at least 24 months below their chronological age. Current head circumference correlated highly with full scale IQ. Assessments of special educational needs had been completed on 36%; 48% were receiving speech therapy. Approximately half of children with the Silver-Russell syndrome have significant impairment of their cognitive abilities.

Birth Weight↗