Over the last 10 years there have been a number of reports of the endocrinological events that occur during normal puberty. These studies, some cross-sectional and some longitudinal in design, have recently been the subject of reviews by... more
Over the last 10 years there have been a number of reports of the endocrinological events that occur during normal puberty. These studies, some cross-sectional and some longitudinal in design, have recently been the subject of reviews by Sizonenko (1978) and Tanner (1980). In the majority of these studies a variety of different hormones have been the subject of the subject of the investigation but they have usually included luteinising hormone (LH), follicle stimulating hormone (FSH) and prolactin (PRL) representing the pituitary peptide hormones, and a great variety of adrenal and gonadal steroids. Mostly the changes in hormone concentrations have been related to chronological age and pubertal stages as formalised by Tanner (1962). Only rarely have bone-age data been available. The auxological data for comparison with the hormonal data has, in general, been skimpy, depending mostly upon the puberty stages and measurement of height or weight.
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The risks of death from coronary heart disease among heterozygotes for familial hypercholesterolaemia are well known and are so high that attempts at prevention are usually undisputed. The earlier diagnosis can be established the sooner... more
The risks of death from coronary heart disease among heterozygotes for familial hypercholesterolaemia are well known and are so high that attempts at prevention are usually undisputed. The earlier diagnosis can be established the sooner treatment can be started and the ideal must be to diagnose the heterozygous state during childhood. However, where the diagnosis carries such a serious prognosis and treatment will be instigated for life it is important that the diagnosis should be correct. The best chance of making a correct diagnosis is among the children of known heterozygotes in whom there is a 1 in 2 chance that they will be affected; but among these children measurement of total serum cholesterol as well as low density lipoprotein cholesterol segregates into two overlapping G-aussian distributions of normal and heterogous individuals.
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The application of the powerful tool molecular biology has made it possible to ask questions not only about hormone production and action but also to characterize many of the receptor molecules that initiate responses to the hormones. We... more
The application of the powerful tool molecular biology has made it possible to ask questions not only about hormone production and action but also to characterize many of the receptor molecules that initiate responses to the hormones. We are beginning to understand how cells may regulate the expression of genes and how hormones intervene in regulatory processes to adjust the expression of individual genes. In addition, great strides have been made in understanding how individual cells talk to each other through locally released factors to coordinate growth, differentiation, secretion, and other responses within a tissue. In this review I (1) focus on developmental aspects of the pituitary gland, (2) focus on the different components of the growth hormone axis and (3) examine the different altered genes and their related growth factors and/or regulatory systems that play an important physiological and pathophysiological role in growth. Further, as we have already entered the ‘post-ge...
Research Interests: Molecular Biology, Biology, Cell Biology, Medicine, Growth Hormone, and 15 moreHumans, Human Growth Hormone, Mice, Animals, Growth and development, Gene, Clinical Sciences, Pituitary Gland, Hormone, Base Sequence, Growth Factor, Secretion, Molecular Sequence Data, Genetic control, and Paediatrics and reproductive medicine
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info:eu-repo/semantics/publishe
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This chapter explains normal childhood growth and clinical disorders associated with abnormal growth, including growth hormone deficiency (growth hormone insufficiency), hypopituitarism, growth hormone receptor deficiency, gigantism and... more
This chapter explains normal childhood growth and clinical disorders associated with abnormal growth, including growth hormone deficiency (growth hormone insufficiency), hypopituitarism, growth hormone receptor deficiency, gigantism and acromegaly. Immunoassays relevant to the investigation and diagnosis of growth hormone disorders are reviewed: growth hormone, growth hormone-binding protein, insulin-like growth factors IGF1 and IGF2 and insulin-like growth factor binding protein. A number of dynamic tests are described: insulin tolerance test, insulin stress test, glucagon tolerance test, clonidine test, arginine test and IGF1 generation test.
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The processes of growth and development imply increases in cell size and number with all the accompanying requirements for protein and nucleic acid synthesis. For orderly growth, the availability of appropriate nutrients as the building... more
The processes of growth and development imply increases in cell size and number with all the accompanying requirements for protein and nucleic acid synthesis. For orderly growth, the availability of appropriate nutrients as the building blocks of macromolecular synthesis is fundamental. It has become clear over recent years that there is also a requirement for a network of supervising processes to control many or all of these metabolic steps. Most of the supervisory processes are controlled by the endocrine system. This network of interrelating hormones links the activity of the neuroendocrine system with cellular operations both in controlling relatively unchanging processes, which are little affected by the aging process, and in entraining processes that subserve changes and occur at specific times of life such as the development of puberty.
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SilverRussell syndrome (SRS) (OMIM 180860) has been recognised as a clinical entity since the 1950s; however, a molecular genetic cause remained unknown until 1997 when maternal uniparental disomy for chromosome 7 (mUPD7) was found in... more
SilverRussell syndrome (SRS) (OMIM 180860) has been recognised as a clinical entity since the 1950s; however, a molecular genetic cause remained unknown until 1997 when maternal uniparental disomy for chromosome 7 (mUPD7) was found in approximately 10% of ...
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Research Interests: International Cooperation, Public Health, Health Policy, Medicine, Humans, and 11 moreChild, Public Health Administration, Data Collection, Mental Disorders, Clinical Sciences, Public health systems and services research, Public Health Surveillance, Child Health Services, mass Screening, Paediatrics and reproductive medicine, and Population surveillance
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Plasma concentrations of somatomedin C/insulinlike growth factor I (SMC/IGF-I) are modulated by a variety of factors. Cardinal among these are growth hormone (GH) and nutritional status. In this chapter we review current understanding of... more
Plasma concentrations of somatomedin C/insulinlike growth factor I (SMC/IGF-I) are modulated by a variety of factors. Cardinal among these are growth hormone (GH) and nutritional status. In this chapter we review current understanding of the influence of GH and nutrition and SMC/IGF-I. We also present nomative data on SMC/IGF-I in over 800 normal infants and children and our assessment of the value of measuring this peptide in growth diagnosis and other conditions.
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ABSTRACT The Silver-Russell syndrome (SRS) is generally sporadic, but with sufficient reported cases of dominant and recessive patterns of inheritance to suggest a genetic cause in some cases, at least. No consistent cytogenetic... more
ABSTRACT The Silver-Russell syndrome (SRS) is generally sporadic, but with sufficient reported cases of dominant and recessive patterns of inheritance to suggest a genetic cause in some cases, at least. No consistent cytogenetic abnormalities have been found although some features of the syndrome have been reported to be associated with structural abnormalities of distal 15q. More recently it has been shown that about 10% of SRS patients have maternal uniparental disomy of chromosome 7 which suggests the presence of a maternally imprinted gene on chromosome 7 that is associated with SRS. In the majority of patients with normal biparental inheritance of chromosome 7 the same gene could be involved if the paternal copy were deleted or mutated so that it is disabled and the maternal copy is silent because of the imprinting.
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Pineal indoles have been shown to affect the release of anterior pituitary hormones but details of the interrelationships are lacking. Using a new gas chromatography–mass spectrometry (g.c.–m.s.) assay the concentration of... more
Pineal indoles have been shown to affect the release of anterior pituitary hormones but details of the interrelationships are lacking. Using a new gas chromatography–mass spectrometry (g.c.–m.s.) assay the concentration of 5-methoxytryptophol (ML) was measured in plasma samples obtained from 16 children undergoing investigation of pituitary function for delayed growth. All the children received an insulin tolerance test (ITT) to study their endocrine response to stress. Some children received luteinizing hormone releasing hormone (LH-RH) and/or thyrotrophin releasing hormone (TRH). The change in concentration of ML during an ITT was similar to the change in concentration of blood sugar; a drop at 20 min followed by a rise at 30 min. This was not significantly altered by the administration of LH-RH or TRH, nor was there a different pattern of response in children who were deficient in growth hormone as opposed to those with idiopathic delayed growth. The fall in concentration of ML w...
Research Interests: Endocrinology, Adolescent, Animal Production, Growth Hormone, Humans, and 14 moreChild, Blood Glucose, Insulin, Puberty, Female, Male, Hypoglycemia, Clinical Sciences, Gonadotropin Releasing Hormone, Gas Chromatography/mass Spectrometry, Veterinary Sciences, Luteinizing Hormone, Follicle stimulating hormone, and hydrocortisone
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Research Interests: Mathematics, Adolescent, Growth, Humans, Child, and 4 moreFemale, Male, Age Factors, and Body Height
Research Interests: Epilepsy, Calcium, Adolescent, Humans, Child, and 12 moreMagnesium, Female, Male, Alkaline phosphatase, Aged, Middle Aged, Adult, Time Factors, Phosphates, Hydroxylation, Bone and Bones, and Wrist
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Highly sensitive assays have been developed that enable 25-hydroxycholecalciferol (25-hydroxyvitamin D3) and 25-hydroxyergocalciferol (25-hydroxyvitamin D2) to be measured in the same serum sample. With these assays it has been shown that... more
Highly sensitive assays have been developed that enable 25-hydroxycholecalciferol (25-hydroxyvitamin D3) and 25-hydroxyergocalciferol (25-hydroxyvitamin D2) to be measured in the same serum sample. With these assays it has been shown that endogenously produced cholecalciferol (vitamin D3) is important in man; the findings further emphasize the role of vitamin D metabolites as hormones rather than vitamins in the traditional sense. Dietary sources of vitamin D appear to be inadequate and vitamin D deficiency has been shown to the cause of rickets and osteomalacia in Asian immigrants to Britain. This condition may be readily treated with small doses of vitamin D. In addition, sub-clinical deficiency was found in the Asian community. In the elderly, also, vitamin D deficiency was established as an important cause of osteomalacia and again evidence for the existence of a sub-clinical deficiency state was found. It is therefore suggested that the present prophylactic practices should be ...
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The assumption that enterocystoplasty in children has a detrimental effect on linear growth has been based almost exclusively upon a chance finding in a retrospective study 10 years ago. We re-evaluated the same research question in a... more
The assumption that enterocystoplasty in children has a detrimental effect on linear growth has been based almost exclusively upon a chance finding in a retrospective study 10 years ago. We re-evaluated the same research question in a larger cohort and with a longer follow-up. Between 1982 and 1997, 242 children and adolescents underwent enterocystoplasty. Patients with conditions involving organ systems apart from the urinary tract, and those with myelomeningocele, malignant diseases, reduced glomerular filtration rate and incomplete notes were excluded. In the definitive study cohort (123; mean age at operation 8.6 years; mean age at investigation 16.8 years), enterocystoplasty had been undertaken using colon in 70, ileum in 37, a combination of both in 11, ileocaecal segments in three and stomach in two patients. In all, 1215 height and weight measurements had been recorded. The distribution of percentile positions before and after enterocystoplasty showed a normal configuration,...
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Growth is far too complex to be described only in terms of height and weight in relation to age. In addition to addressing multiple factors that have clinical value in the assessment of growth in adolescence, this chapter discusses the... more
Growth is far too complex to be described only in terms of height and weight in relation to age. In addition to addressing multiple factors that have clinical value in the assessment of growth in adolescence, this chapter discusses the interpretation of growth data, growth at puberty, and methods of predicting height at maturity.
To evaluate the effect of recombinant human growth hormone (rhGH) on the linear growth of children with persistently active juvenile chronic arthritis (JCA), most of whom were receiving steroid therapy. All of them were severely growth... more
To evaluate the effect of recombinant human growth hormone (rhGH) on the linear growth of children with persistently active juvenile chronic arthritis (JCA), most of whom were receiving steroid therapy. All of them were severely growth retarded, but had adequate GH secretion. After monitoring height velocity for one year, children were treated for the following year with either 12 IU/m2 or 24 IU/m2 of rhGH. During this period disease activity, drug treatment, dietary intake and bone maturation as well as linear growth were documented. There was a significant increase in height velocity in almost all children during the treatment period. Children with mild to moderate disease activity grew at a better rate than those with very active disease. Children with polyarticular disease responded better than those with systemic JCA. Those children receiving high dose rhGH grew significantly more than those on the low dose regimen. Bone maturation did not exceed chronological age. We conclude ...
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Significantly lower testosterone levels are common in male patients with homozygous sickle-cell (SS) disease and have been attributed to either abnormalities of the hypothalamo-pituitary axis or primary testicular failure. The mechanism... more
Significantly lower testosterone levels are common in male patients with homozygous sickle-cell (SS) disease and have been attributed to either abnormalities of the hypothalamo-pituitary axis or primary testicular failure. The mechanism has now been investigated by observing the response to gonadotropin-thyrotropin releasing hormones (GnRH-TRH) in 10 male patients with SS disease and in 10 matched male sibling controls without sickle-cell disease. Mean basal levels of luteinizing hormone (LH), follicular stimulating hormone (FSH) and thyrotropin (TSH) were significantly elevated but prolactin (PRL) levels were within the normal range in the SS group. All hormones increased following GnRH-TRH, and proportionate increases over baseline were similar for FSH and TSH in SS and AA subjects, but SS patients showed a lesser percentage increase in LH at 30 minutes, and a higher percentage increase in PRL at 60 minutes. These observations are more consistent with primary testicular failure th...
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Sixty patients with the diagnosis of idiopathic growth hormone deficiency have been followed till final height was reached, after hGH treatment lasting between 2 and 15 (average 5.4) years. Twenty-six had 'total'and 13... more
Sixty patients with the diagnosis of idiopathic growth hormone deficiency have been followed till final height was reached, after hGH treatment lasting between 2 and 15 (average 5.4) years. Twenty-six had 'total'and 13 'partial'isolated growth hormone ...
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Growth hormone deficiency embraces a heterogeneous group of disorders with multiple aetiologies. The biggest single division is between those children whose growth hormone deficiency is due to a structural lesion and those where it is... more
Growth hormone deficiency embraces a heterogeneous group of disorders with multiple aetiologies. The biggest single division is between those children whose growth hormone deficiency is due to a structural lesion and those where it is not. In both cases other pituitary hormones may, or may not, be affected, although when a structural lesion is present multiple pituitary hormone deficiency is the rule rather than the exception. In the absence of structural lesions the pathogenesis of the condition in still largely obscure, although some mechanisms, such as cranial irradiation, are now well recognized. Birth trauma is also now a strong candidate as a predisposing factor. The prevalence of growth hormone deficiency is still uncertain, but is probably about 1 in 4000 live births. The clinical features of growth hormone deficiency are usually fairly clear, with short stature, low growth velocity, excess subcutaneous fat and delayed skeletal maturation being the principal clinical feature...
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Twenty-seven patients with cystic fibrosis completed a controlled trial comparing the effects of an inhaled mucolytic drug, sodium-2-mercaptoethane sulphonate (Mistabron, UCB Pharmaceutical Division, Brussels, Belgium), with inhaled... more
Twenty-seven patients with cystic fibrosis completed a controlled trial comparing the effects of an inhaled mucolytic drug, sodium-2-mercaptoethane sulphonate (Mistabron, UCB Pharmaceutical Division, Brussels, Belgium), with inhaled iso-osmolar hypertonic saline. As a group the 22 patients with chronic sputum production showed small but statistically significant improvement in pulmonary function tests after Mistabron therapy, both when compared with a control period, and with iso-osmolar saline results. Subjective measurements by diary card failed to show any changes. No significant changes were found in five patients with no measurable sputum production. The inhalations were given after physiotherapy and were well tolerated. There were no significant side effects. The results suggest that Mistabron has a beneficial therapeutic effect unrelated to its high osmolality, and the intermittent inhalation of Mistabron may have a role in the treatment of selected patients with cystic fibro...
Research Interests: Cystic Fibrosis, Adolescent, Aerosols, Respiratory therapy, Humans, and 7 moreChild, Female, Thorax, Male, Lung, Clinical Sciences, and Sputum
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We describe the phenomena of catch-up and catch-down growth. These are normal physiological responses in both endocrine and non-endocrine diseases of childhood. Evidence suggests that catch-up growth is controlled by a centre in the... more
We describe the phenomena of catch-up and catch-down growth. These are normal physiological responses in both endocrine and non-endocrine diseases of childhood. Evidence suggests that catch-up growth is controlled by a centre in the central nervous system and that it is not dependent on increased growth hormone secretion.
Research Interests: Adolescent, Growth Hormone, Growth, Humans, Child, and 8 moreFemale, Animals, Male, Infant, Newborn Infant, Homeostasis, Age Factors, and Body Height
The three commonly used methods of height prediction employ various combinations of anthropometric data and bone age. In normal children, the regression type methods are preferable, though they do not perform well in the more severe... more
The three commonly used methods of height prediction employ various combinations of anthropometric data and bone age. In normal children, the regression type methods are preferable, though they do not perform well in the more severe disorders of growth. They are extremely dependent upon the limitations of the method of bone age determination that is used. A major source of error is the inability to predict the timing or the intensity of the adolescent growth spurt. Until this proves possible it is unlikely that significant improvements can be made.
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Research Interests: Humans()
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We present findings from a study of nutritional status amongst 1st year primary school children in Brazil. The study was based on a 10% stratified random sample of children in Campinas, Sao Paulo State. The primary schools in the city... more
We present findings from a study of nutritional status amongst 1st year primary school children in Brazil. The study was based on a 10% stratified random sample of children in Campinas, Sao Paulo State. The primary schools in the city were grouped into four socio-economic strata (high, medium, low and very low) based on the type of school maintenance (private or maintained by the local or by the State government) and the socio-economic characteristics of the school's catchment area. The nutritional status of 1942 children was assessed by looking at the distributions of z-scores of weight-for-age, height-for-age and weight-for-height in relation to growth charts of the National Center for Health Statistics reference population. In the overall population, 22% of the children were found to be stunted (z-score of height-for-age less than -1.0), 15% wasted (z-score of weight-for-height less than -1.0), 22% underweight (z-score of weight-for-age less than -1.0) and 5% overweight (z-sc...
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Beclomethasone dipropionate (BDP) is a synthetic glucocorticoid with great topical potency. It has previously been demonstrated to be an effective treatment for childhood atopic dermatitis (AD) when given orally. We have monitored linear... more
Beclomethasone dipropionate (BDP) is a synthetic glucocorticoid with great topical potency. It has previously been demonstrated to be an effective treatment for childhood atopic dermatitis (AD) when given orally. We have monitored linear growth and adrenal function in a group of children treated with oral BDP for severe AD. Stable control of disease was achieved in 10/14 patients (mean dose: 1000 micrograms/day, range 800-1800). At this maintenance dose, there was evidence of deceleration of linear growth in 7/10 patients. There was no significant difference between pre-treatment 8 a.m. plasma cortisol levels and those on the maintenance dose. However, there was a reduction in 24-hour urinary cortisol excretion during maintenance treatment, although this did not reach statistical significance. We regard oral BDP as a useful treatment in widespread childhood atopic AD that has not responded adequately to topical therapy. However, it is mandatory that growth be monitored carefully dur...