Search PubMed⌕ Search

Biomedical subjects

R Stanhope

Publications and source records attributed to R Stanhope.

At least 55 records · Page 3Linked to original sources
PubMed1998

Metaphyseal growth arrest lines in psychosocial short stature.

Metaphyseal growth arrest lines are seen in children who experience significant physical stress such as infection or malnutrition over a sufficient period of time. These lines have not been reported previously in children with psychosocial short stature (PSS). Two boys and a girl with PSS with metaphyseal growth arrest lines on skeletal radiographs at the time of maximal stress in their homes are described. All three had reversible growth hormone insufficiency during admission, which is pathognomic for PSS. Multiple growth arrest lines in the distal end of the radius or vertebrae should alert clinicians to an alternative diagnosis in a child with growth hormone insufficiency. This may provide a clue to the diagnosis of occult PSS.

Body Height↗
PubMed1998

Growth hormone treatment in growth hormone-sufficient and -insufficient children with intrauterine growth retardation/Russell-Silver syndrome.

Fifty-eight short prepubertal children with IUGR received GH treatment (mean dose: 28 IU/m2/week) for a mean (SEM) period of time of 3.4 (0.13) years (range 1-4 years). They were subdivided according to their GH response to a pharmacological test. Twenty-six were GH insufficient (GHI) (group 1) and 32 were non-GHI (group 2). At the commencement of GH therapy mean chronological age was 6.1 (0.4) years in both groups, mean height SDS (SEM) was -3.5 (0.2) in group 1 and -3.6 (0.2) in group 2, mean growth velocity (GV) SDS (SEM) was -1.9 (0.3) in group 1 and -0.3 (0.2) in group 2. GH therapy induced significant growth acceleration throughout the follow-up period without any significant differences between the two groups. GV SDS (SEM) increased to +3.0 (0.5) in group 1 and to +3.7 (0.4) in group 2 (p < 0.05 compared to baseline) during the first year of therapy. Subsequently, the growth-promoting effects of GH therapy diminished with time but GV remained significantly higher than baseline. This growth enhancement produced a significant rise in height SDS (SEM) reaching - 1.4 (0.2) in group 1 and - 1.7 (0.2) in group 2 after 4 years. In conclusion, our data did not show any significant differences in the growth response to GH therapy between GH-sufficient and -insufficient IUGR children who were only distinguishable by their GH secretion. This indicates that the decision to treat a short IUGR child with GH therapy should not be based upon the GH response to a provocative test.

Body Height↗
PubMed1998

Occult T3 toxicosis in McCune-Albright syndrome.

We report a girl with McCune-Albright syndrome who presented with Cushing syndrome from adrenal hypersecretion and gonadotrophin-independent precocious puberty in the first year of life. At age 5, she failed to gain weight and was found to have hyperthyroidism, which was occult in that she had T3 toxicosis without a goitre or thyroid ultrasound abnormality. The latter has not been previously reported in McCune-Albright syndrome.

Antithyroid Agents↗
PubMed1998

Growth hormone treatment of Russell-Silver syndrome.

Russell-Silver syndrome represents a special group of children with intrauterine growth retardation (IUGR) who do not experience catch-up growth and have characteristic dysmorphic features. They also have characteristics of abnormal growth hormone pulsatility, absence of catch-down growth after growth hormone therapy and inappropriate advancement of bone age during the middle childhood years. Data from children with Russell-Silver syndrome should certainly be analysed as a separate group from short children due to nondysmorphic IUGR. Initial data suggests that final height outcome will be improved by using pharmacological doses of biosynthetic human growth hormone. Indeed, the recent data supports the hypothesis of Blizzard's group in 1974 that if growth hormone became available in sufficient quantities, then final height could be altered in IUGR children. In addition, the early recognition and treatment of spontaneous nocturnal hypoglycaemia may well improve the educational achievement of such children.

Child, Preschool↗
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↗
PubMed1997

Hydrocortisone replacement therapy in children and adolescents with hypopituitarism.

OBJECTIVE: Appropriate replacement doses of glucocorticoid are important to determine in primary and secondary adrenal deficiency in children, both to avoid the risks of hypoglycaemia and adrenal crisis associated with undertreatment, and to avoid growth suppression and reduced final height potential associated with steroid excess. The aim of this study was to assess how closely conventional twice daily hydrocortisone administration mimics physiological cortisol secretion in a group of ACTH-deficient children and adolescents. PATIENTS: Fifty children and adolescents (aged 3-20 years) were studied who had had surgery +/- radiotherapy to the hypothalamopituitary region for removal of a craniopharyngioma. The patients were subdivided into two groups: group I comprised 44 patients known to be ACTH deficient (as determined by glucagon or insulin provocation tests of anterior pituitary function performed after surgery) and maintained on twice daily oral hydrocortisone replacement; group II comprised six patients known to be ACTH sufficient at their last assessment of pituitary function and not on hydrocortisone replacement. A third group of 10 boys (aged 7-13 years) who had no known endocrinopathy were used as controls (group III). MEASUREMENTS: After intravenous cannula insertion, blood samples were taken every 2h for measurement of plasma cortisol and glucose over a period of 24h. Patients in group I continued on their usual doses of hydrocortisone, prescribed at 0800 and 1800 h. RESULTS: The mean total daily replacement dose of hydrocortisone for patients in group I was 12.3 mg/m2/ day (range, 5.5-18.5). On the conventional twice daily dose regimen, there was a supraphysiological medium plasma cortisol level (629 nmol/l, range 185-1600; z = -3.76, P = 0.0002) 2 h after the morning dose relative to the control group, and a prolonged and unphysiological nadir from 1400-1800 h (median at 1600 h 42 nmol/l, range 13-1170; z = -3.13, P < 0.002) before the second dose of hydrocortisone was administered. Cortisol values were low, and often negligible, during the early hours of the morning (median at 0600 h 15 nmol/l, range 13-277, z = -4.87, P < 0.00001) and spontaneous hypoglycaemia was documented in one patient on a single 0800 h sample. One patient in group II was shown to be unequivocally cortisol deficient and median cortisol values for the remaining five suggested a suboptimal rise in plasma cortisol during the early hours of the morning. CONCLUSION: Our cohort of patients provides an excellent model for the study of glucocorticoid replacement in cortisol-deficient children and adolescents and shows, as in adults, that the aim of mimicking the physiological nyctohemeral secretion of cortisol is difficult to achieve in practice and raises a number of important considerations unique to steroid substitution therapy in this age group.

Adolescent↗
PubMed1997

Precocious pseudopuberty due to a granulosa cell tumour in a seven-month-old female.

We describe a juvenile granulosa cell tumour resulting in pseudopuberty in an infant female. The progression of the clinical signs of puberty were non-consonant and the diagnosis was complicated by marginally elevated serum alpha-fetoprotein levels. The histological appearance of the resected tumour and binding of MIC2 antibody to tumour cells confirmed the diagnosis.

Diagnosis, Differential↗
PubMed1997

Growth hormone treatment without a needle using the Preci-Jet 50 transjector.

A new delivery system (Preci-Jet 50) which administers growth hormone through the skin using high pressure and without a needle was evaluated. This device was inconvenient and painful compared with a pen injection system. The conclusion is that the Preci-Jet is not the panacea for solving the problem of compliance with subcutaneous growth hormone injections.

Administration, Cutaneous↗
PubMed1997

Survival and endocrine outcome after testicular relapse in acute lymphoblastic leukaemia.

Survival and endocrine status in a cohort of boys with acute lymphoblastic leukaemia (ALL) who started treatment between 1972 and 1987 and subsequently developed a testicular relapse were analysed. During this period there was a significant improvement in the overall event free survival for boys, but no significant decrease in the testicular relapse rate. Thirty three boys had an apparently isolated testicular relapse, whereas 21 boys had a combined relapse. The event free survival for boys with an isolated testicular relapse was 59% at six years (95% confidence interval (CI) 42 to 74%). The event free survival for the 16 patients with a combined relapse who received a second course of treatment was 32% (95% CI 17 to 60%). Those patients receiving adequate second line treatment for an isolated testicular relapse whose first remission was longer than or equal to two years had an event free survival of 82% (95% CI 63 to 93%) at six years. No boy relapsing within two years from diagnosis has survived. Endocrine late effects are significant, with 82% of the boys requiring hormonal treatment at some stage for induction of puberty or continuing pubertal maturation, or both. It is concluded that, despite the increasing intensity of initial treatment for ALL, isolated testicular relapse is treatable by conventional means in most patients. Careful endocrine follow up of these patients is essential as most will require hormone replacement treatment.

Adolescent↗
PubMed1997

GH treatment induces sustained catch-up growth in children with intrauterine growth retardation: 7-year results.

The anthropometric response to 7 years of GH treatment was assessed in 11 short children with Russell-Silver syndrome (RSS) and in 5 with non-dysmorphic intrauterine growth retardation (NRSS). GH treatment induced a significant increase (p < 0.0001) in the mean height standard deviation score (SDS) and at the 7-year follow-up a height appropriate for the natural history of final stature in NRSS/RSS was already attained. An appreciable growth rate was still present with final height being attained only in 2 girls. There was no significant change in height SDS for bone age. Multiple regression analysis showed only chronological age at the onset of GH treatment was a predictor of gain in height SDS during GH therapy. These findings suggest that early GH treatment improves long-term growth in children with NRSS/RSS. Moreover, final height attainment is required for a definitive assessment of the beneficial effect on adult stature. Bone age assessment is not a reliable parameter to predict final height outcome in these children.

Aging↗
PubMed1997

Persistent short stature in children with intrauterine growth retardation: use of growth hormone treatment.

Since the early 1970s, it has been demonstrated that growth hormone (GH) treatment improves the short-term growth rate of children with short stature due to intrauterine growth retardation (IUGR). However, it is only in recent years that studies have been conducted in which children with IUGR have been followed to final height. It is appreciated that pharmacological doses of GH are required for the treatment of short children with IUGR, and that the initial effect of rapid advancement of epiphyseal maturation is probably due more to the natural history of growth in children with IUGR than to the GH treatment. Initial studies are promising, showing that the rapid improvement in growth rate is eventually translated into an improvement in final height. However, studies involving a larger number of children with IUGR are necessary in order to quantify this improvement in final height.

Body Height↗