Treatment for growth hormone disorder.
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Acromegaly↗
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DNA mapping in growth and developmental disorders.
DNA mapping techniques are being increasingly applied to familial and acquired disorders affecting growth and development. To understand the potential applications of these techniques, one must first have a good understanding of the components and basic structure of DNA, the genetic code, the basic structure of genes and the roles that various components of gene structure play in regulating gene expression. Following review of this basic information, a variety of DNA mapping techniques including in situ hybridization, Southern blotting, polymerase chain reaction amplification, DNA sequencing and linkage analysis will be covered. Applications of these techniques can enable mapping of unknown genes by detection of loss of allelic heterozygosity or use of linkage analysis and genetic maps. With increasing ease, mutations such as deletions, expansions, rearrangements and point mutations can be detected in diseases such as congenital adrenal hyperplasia, cystic fibrosis, diabetes insipidus, growth hormone deficiency, fragile X syndrome, Laron dwarfism and Turner syndrome. From this discussion, a better understanding of methods of gene localization, uses of genetic maps and rapid, convenient methods to detect a variety of molecular derangements causing familial disorders affecting growth and development will be gained.
Blotting, Southern↗
[Neurological and growth follow-up of cardiac patients operated on before 45 days of life].
OBJECTIVE: Our aim was to study the neurological and growth disorders in patients undergoing cardiac surgery during the neonatal period. PATIENTS AND METHODS: Ninety clinical records of newborns with congenital heart disease that were operated between 1985 and 1996 and were under the age of 45 days with a minimum follow-up period of 12 months were reviewed. RESULTS: Of the 37 patient records considered, 33 (89.1%) were neurosensorially normal, 2 had minor handicaps and 2 moderate handicaps. There were 15 (40.5%) with the weight and 9 (24.3%) with the length below the 10th percentile. Although exitus, handicaps and growth disorders were more frequent in those patients operated in the extracorporal circulation, there were no significant differences. The growth disorders were greater in complex heart diseases and in pulmonary atresia, but significant differences were only related to the weight. CONCLUSIONS: Because at least 11% of these patients showed neurological disorders, a neurological follow-up is necessary. The number of post-operated patients with growth disorders was high, but catch-up growth resulted in recovery. A greater number of neurological and growth disorder has been found in complex heart disease, where only a palliative treatment could be employed. Although the complete correction of the cardiopathy may imply an early mortality, long time prognosis for survivors is much better.
Follow-Up Studies↗
[Growth & developmental disorders in children].
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Child↗
Complications and failures of the ESIN technique.
Only knowledge of the possible failures and complications of a method allows its correct application. This also applies to ESIN (Elastic Stable Intramedullary Nailing), which has been promoted as a simple procedure. Most failures occur through neglect of the important biomechanical principles, and by obvious technical errors. The combination of both causes of failure can lead to serious complications in this method, which is generally regarded as "benign". These complications include serious rotational deformity, leg shortening, and growth disorders from growth plate injuries. A fundamental distinction must be made between intraoperative and postoperative problems and true complications. The present paper is intended to contribute towards avoiding such errors and detecting errors as early as possible during surgery in order to correct them.
Adult↗
Identification and characterization of specific growth hormone receptors in cultured human fibroblasts.
A significant impediment to studies of growth disorders in man has been the lack of an easily accessible target tissue for studies of GH action. As human fibroblasts have been shown to produce a somatomedin-C-like peptide in culture, the aim of this study was to investigate the interaction of GH with cultured fibroblasts. GH binding to human skin fibroblasts and to lung fibroblasts was examined. Specific binding of [125I]iodo human GH [( 125I]iodo hGH) was rapid, reversible, and time and temperature dependent. Maximal binding was achieved within 2 h at 30 C and specifically bound [125I]iodo-hGH was rapidly dissociable at this temperature. A linear relationship between specific binding of [125I]iodo-hGH and cell number was found and negligible degradation of labeled hormone occurred during the binding studies. Half-maximal inhibition of specific binding occurred at 30 ng/ml hGH. Lactogenic and nonprimate somatogenic hormones did not displace [125I]iodo-hGH at the concentrations tested. Scatchard analysis of [125I]iodo-hGH binding to human skin fibroblasts demonstrated a single class of binding sites with an affinity constant of 1.07 +/- 0.07 (SEM, n = 5) X 10(9) M-1 and a capacity of 8355 +/- 1880 site per cell. Similar GH-receptor characteristics were found on each of the fibroblast cell lines examined irrespective of the site of origin (skin or lung) or age of donor. These findings demonstrate for the first time specific GH receptors in cultured human fibroblast cell lines. The demonstration of GH receptors in human fibroblasts should encourage the search for similar receptors in tissues not previously considered to be target tissues for GH action. On the basis of these studies we suggest that cultured skin fibroblasts may be a suitable tissue for the study of GH-receptor status in patients with disorders of growth.
Adult↗
Serum somatomedin activity assayed by the enhancement of proteoglycan sulphation using chick embryo chondrocytes in normal children at various ages and children of short stature.
It was possible to obtain a sufficient amount of homogeneous chondrocytes from 13-day old chick embryo-sterna in an 8-day suspension culture. Serum somatomedin (SM) activity was measured on the basis of ability of serum to stimulate labelled sulphate uptake in these chondrocytes. Somatomedin A and multiplication stimulating activity (MSA) stimulated the sulphation in this assay. Serum SM activity in term cord sera and newborn sera was lower than that in normal adults. Serum SM activity in early infants increased rapidly and attained the levels of that in normal adults within 1 month following birth. Serum SM activity in infants (1 month - 2 years) and pubertal children (10-16 years), when the annual height increment is greatest, was higher than in normal adults. Serum SM activity in children with constituted dwarfism aged 6-10 years and over 10 years was normal and lower, respectively, compared to that in age-matched controls. Pituitary dwarfs had markedly low SM activity compared to age-matched controls. Diagnostic evaluation of serum SM activity in children requires comparison with age-matched controls. Assay for serum SM activity may provide a useful and simple procedure fo differentiating the cause of growth disorders from growth hormone deficiency.
Adolescent↗
Extra-colonic manifestations of familial adenomatous polyposis.
Familial adenomatous polyposis (FAP) is a genetic disease created by an abnormality of chromosome number five resulting in a generalized cellular growth disorder. The growth abnormality created by the genetic defect dictates the development of benign and malignant lesions in different organs of the body. Even the term familial adenomatous polyposis does not fully describe the defect because lesions other than polyps may occur in these patients. The major organ affected is the large intestine. Adenomas occur at an earlier age than normally seen in the general population, grow more numerously and more rapidly, and become malignant at an inordinately young age.
Adenomatous Polyposis Coli↗
[Burn injury of the child's hand].
The treatment of the burnt hand of a child requires careful consideration of both the physical and psychological aspects involved in this programme; measures such as physical therapy, which play an important part in the treatment of adults, are of less significance. Nevertheless, due to a very low incidence of complications eg. joint stiffness, the final results are very good. Generally the recommended approach to the treatment programme is to use the closed method (i. e. dressings), whereas in such cases care on a special unit employing the open method and a topical bactericidal agent is considered advisable. When the general condition of the child permits, the surgical treatment of a third degree burn should be carried out as early as possible, rather than waiting for spontaneous escharatomy and formation of granulation tissue. Immobilisation of a wound grafted with split-thickness skin should be obtained using KIRSCHNER wires or a hay-rake splint. An early date should also be set for secondary surgical procedures involving improvement of function. Depending upon the surgical findings, flaps or free grafts may be used. It is essential that flexor contractures in the region of the PIP joints be dealt with primarily, in order to prevent the secondary formation of button-hole deformities. During follow-up examinations, growth disorders of the phalanges may be seen. These may arise as sequel to arthrodesis, or trauma to epiphyses as a result of electrical current. On the other hand, disorders of growth may also be observed in purely thermal injuries -- these are mainly confined to growth in the length. Deviation from the central axis of the finger resulting from scar tissue contracture, was not observed among our group of patients.
Adolescent↗
[One hundred years of orthopedics in the Netherlands. VIII. Pediatric orthopedics].
Orthopaedic disorders in children differ in type from those in adults: most frequent are congenital anomalies and disorders of growth and development. The special nature and relative rarity of these conditions justify the separate development of this branch of the discipline. Fractures almost always heal normally after closed reduction and immobilization in a plaster cast; fractures close to epiphyseal discs and in joints require special attention. Slipping of the upper femoral epiphysis necessitates surgical fixation of the epiphysis. Benign bone tumours occur relatively often and mostly require no surgical intervention. The prognosis of solid malignant bone tumours has improved since the introduction of (neo)adjuvant chemotherapy and limb-sparing surgery. In case of difference in leg length, the length of both legs is predicted with the aid of roentgenological measurements. Inhibition of the growth of the longer leg gives rise to fewer complications than lengthening of the short leg. The essence of the treatment of growth disorders due to abnormal ossification of the cartilage is to monitor the natural repair process and to intervene if permanent malformation threatens.
Bone Diseases, Developmental↗
Alpha-2-adrenoceptor sensitivity in heroin addicts with and without previous attention deficit disorder/hyperactivity and conduct disorder.
Growth hormone (GH) and beta-endorphin (beta-EP) responses to clonidine stimulation were examined in 18 male heroin addicts, 9 with and 9 without previous histories of attention deficit disorder with hyperactivity (ADD-H) and conduct disorder (CD). Ten psychophysically healthy volunteers were used as controls. ADD-H/CD addicts had blunted GH and beta-EP responses as compared to controls while those of non-ADD-H/CD addicts were normal. This suggests that postsynaptic adrenoceptor sensitivity is decreased and, possibly, that presynaptic noradrenaline secretion is increased in ADD-H/CD patients with heroin addiction.
Adolescent↗
[The question of midface growth inhibition following retinoblastoma treatment in early childhood].
99 patients treated for bilateral retinoblastomas in the University Hospital of Essen between 1965 and 1983 were followed up for midface growth inhibition for an average period of 15 1/2 years. Depending on the type of treatment employed the patients were divided into four groups: Local tumor treatment using cryotherapy or laser techniques, ophthalmectomy alone, radiotherapy alone, and combined ophthalmectomy and radiotherapy. Statistically significant differences between the treatment groups were found regarding such symptoms as hypoplasia of the zygomatic bone, hypoplasia of the transversal orbit, hypoplasia of the temporal fossa, hypoplasia of the root of the nose as well as hypotelorism and enophthalmos. Combined surgery and radiotherapy caused the most severe growth disorders, followed by radiotherapy alone and ophthalmectomy alone. With local treatment detectable growth disorders were observed, if at all, only as a result of contralateral radiation scatter. Orthovoltage radiotherapy was found to produce growth disorders of significantly higher severity than megavoltage radiotherapy.
Adolescent↗
IGFs and IGFBPs: role in health and disease.
The insulin-like growth factors (IGFs), IGF-binding proteins (IGFBPs), and IGFBP proteases are the main regulators of somatic growth and cellular proliferation. IGFs are involved in growth pre-natally and post-natally. Dysregulation of the IGF axis can lead to growth disorders such as growth hormone deficiency and acromegaly. Pre-natally, this dysregulation can lead to IUGR or macrosomia. IGFs also have an important mitogenic action and play a role in tumorigenesis and cancer. These actions are regulated by co-interactions with IGFBPs, especially IGFBP-3. In addition to somatic growth and mitogenic activity, IGFs have hypoglycaemic and insulin sensitizing actions, and their dysregulation is involved in diabetes and its complications. In this chapter, we examine the role of IGFs and IGFBPs in growth, tumorigenesis and diabetes, and discuss treatment modalities for each disease involving the GH-IGF-IGFBP axis, including discussion of current in vitro and in vivo investigations in this field.
Animals↗
Evidence for hypothalamo-growth hormone dysfunction in panic disorder: profile of growth hormone (GH) responses to clonidine, yohimbine, caffeine, glucose, GRF and TRH in panic disorder patients versus healthy volunteers.
Given the abrupt and time-limited nature of daytime-awake and nocturnal-sleep panic attacks, several chemical and neuroendocrine challenge tests have been employed to investigate the neurobiology of "spontaneous" panic attacks. Previously we demonstrated that panic disorder patients have blunted growth hormone (GH) responses to clonidine, an alpha 2-adrenergic agonist. However, the mechanism of this blunted response and the role of hypothalamic-GH dysfunction, if any, remains unclear. To further delineate the status of hypothalamic-GH function in panic disorder, we review the literature and present original data on the GH responses to a number of different chemical and neuroendocrine challenge paradigms. Although stress-mediated increases in GH are thought to be a common correlate of stress in humans, our findings indicate that panic disorder patients have significantly blunted GH responses to clonidine, yohimbine, growth-hormone releasing factor, and caffeine compared to normal control subjects. A similar trend was noted in the delayed rise in GH after glucose challenge. There was no difference in the rate of abnormal GH responses to thyrotropin-releasing hormone in panic disorder compared to normal control subjects. No drug or neuroendocrine challenge, even if associated with marked increases in anxiety, produced a significantly enhanced GH response compared to normal control subjects. These findings provide support for a hyporesponsive hypothalamic-GH system in panic disorder. These observations, combined with preliminary observations from our clinic of short stature in several cases of prepubescent children with anxiety disorders, also underscore the need for assessing early growth patterns in individuals with panic disorder. Strategies for investigating the site(s) of possible neurotransmitter or hypothalamic-GH-somatomedin dysfunction are discussed.
Caffeine↗
Serum levels of 20-kilodalton human growth hormone (GH) are parallel those of 22-kilodalton human GH in normal and short children.
Twenty-kilodalton human GH (20K), which is one of the human GH (hGH) variants, is thought to be produced by alternative premessenger ribonucleic acid splicing. However, its physiological role is still unclear due to the lack of a specific assay. We have measured serum 20K and 22-kDa hGH (22K) by specific ELISAs to investigate the physiological role of 20K in children. The subjects were 162 normal children, aged 1 month to 20 yr; 12 patients with GH deficiency (GHD), aged 11 months to 13 yr; 57 children with non-GHD short stature, aged 2-17 yr; and 13 girls with Turner's syndrome, aged 5 months to 15 yr. Samples were collected at random from normal children and were collected after hGH provocative tests and 3-h nocturnal sleep from GHD, non-GHD short stature, and Turner's syndrome children. The mean basal serum concentrations of 22K and 20K were 2.4 +/- 2.8 ng/mL and 152.3 +/- 184.0 pg/mL in normal boys and 2.5 +/- 3.1 ng/mL and 130.6 +/- 171.5 pg/mL in normal girls, respectively. The percentages of 20K (%20K) were 5.8 +/- 2.1% and 6.0 +/- 3.2% in 83 normal boys and 79 normal girls, respectively. There was no significant difference in %20K either among ages or between the prepubertal stage and the pubertal stage in normal boys and girls. The mean %20K values in basal samples of provocative tests in 12 patients with GHD, non-GHD short stature, and Turner's syndrome were 6.5 +/- 2.4%, 6.5 +/- 3.8%, and 5.9 +/- 3.2%, respectively. There was no significant difference in %20K among normal children and these growth disorders, and there was no significant difference in %20K throughout the hGH provocative tests and 3-h nocturnal sleep in these growth disorders. There was also no significant correlation between the percentage of 20K and the height SD score or body mass index in either normal children or subjects with these growth disorders. In conclusion, the %20K is constant, regardless of age, sex, puberty, height SD score, body mass index, and GH secretion status. The regulation of serum 20K levels remains to be established.
Adolescent↗
Physiology and disorders of the growth hormone receptor (GHR) and GH-GHR signal transduction.
GH exerts its actions through binding with two receptor molecules at the cell surface and interaction with Janus kinase and signal transducers and activators of transcription, and other likely effectors to stimulate metabolic effects and IGF synthesis. The circulating GH binding protein is the proteolytic product of the cell surface receptor and serves as a marker of receptor number and function. Thirty-six distinct mutations of the receptor in the extracellular and transmembrane domains cause a clinical picture of severe GH/IGF-I deficiency, whereas two dominant negative mutations of the intracellular domain result in a milder clinical syndrome. These mutations have provided insight into the physiology of the GH receptor. A few patients have been described with what appears to be primary GH insensitivity due to defective signal transduction by the GH-GH-receptor complex. Clinical and biochemical features of primary GH insensitivity are not a function of genotype, with as much variability in a genetically homogeneous population as in a heterogeneous one. Except for those dominant negative mutations where co-transfection of the mutant GH receptor gene with wild-type receptor gene has been informative, evidence for an effect of a single mutant allele remains speculative. Treatment of GH receptor deficiency with recombinant human IGF-I suggests that the absence of a direct effect of GH limits growth response.
Animals↗
Disorders of somatic growth.
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Anabolic Agents↗
Growth hormone therapy in short children without classical growth hormone deficiency.
The spontaneous secretion of GH which determines the growth in height as well as the GH increment in the provocation tests (the socalled pituitary reserve) is diminished in pituitary dwarfism. In numerous other growth disorders only the spontaneous GH secretion is impaired. Amongst others this is true of the syndrome of constitutional delay of growth and adolescence which is the most frequent growth disorder in childhood and of the growth retardation after X-ray irradiation of the skull. In all these cases treatment by hGH represents replacement therapy. In children with intrauterine growth retardation, familial short stature and skeletal dysplasias growth hormone deficiency cannot be proved. In most cases the growth velocity cannot be accelerated with hGH in the usual replacement doses. High doses of hGH may be successful. In endogenous and exogenous hypercortisolism the spontaneous GH secretion is suppressed and the effect of the somatomedins on the growing cartilage is inhibited. This is not only true of Cushing's Syndrome and corticosteroid therapy but also of cases of glycogenosis Type I. The resulting growth retardation can - at least partly - be overcome with hGH. Preliminary investigations show that the growth retardation in children with chronic renal failure can successfully be treated by hGH.
Child↗