Growth hormone deficiency.
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ABSTRACT: Growth Hormone (GH) is secreted from the anterior pituitary gland. It binds to receptors on the surface of target cells, stimulates production of Insulin-like growth factor-I (IGF-I) leading to growth of almost all tissues of the body capable of growing. Growth failure (height below 3rd centile) occurs in children who do not secrete sufficient amount of GH. In some children, however, short stature is present in the presence of high levels of GH in their blood and they also secrete normal to increased amounts of GH in response to stimulation tests when tested for possible deficiency of GH. This condition is known as GH resistance syndrome or Larons syndrome (LS). All patients after a thorough clinical evaluation underwent GH evaluation protocol as follows. On arrival in the lab a blood sample was collected for basal GH level in each patient. Screening was performed by subjecting the patients to exercise stimulation test and/or L-dopa stimulation test. Patients with GH deficiency underwent insulin tolerance test (ITT) after one week for confirmation. All the basal and post-stimulation samples were analyzed for GH levels. A level below 10mIU/L indicated GH deficiency, between 10-20mIU/L as borderline and an adequate response was defined as a GH >20mIU/L. Patients with a basal GH level of >20mIU/L and/or a post-stimulation level of >40mIU/L were arbitrarily considered as having exaggerated GH levels. This article evaluates the high plasma growth hormone levels among clinically short stature children undergoing growth hormone stimulation tests. Two hundred ninty-three patients reported for GH evaluation. Twenty were excluded for various reasons. Thus 273 patients were included for GH evaluation out of which 66(24.2%) showed GH deficiency, 89(32.6%) were borderline while 118(43.2%) patients exhibited adequate response, with GH levels of >20mIU/L. A number of patients unexpectedly showed very high GH levels on screening tests. Out of 118 patients, 21 showed either very high basal levels of >20mIU/L and/or a much-exaggerated response to stimulation tests with levels more than about 40mIU/L. Close consanguinity was found in 67% of patients showing very high GH levels. Some children with idiopathic short stature may show high levels of GH during their evaluation for GH deficiency. We identified a considerable number of such patients. These patients require further investigations.Journal of Ayub Medical College, Abbottabad: JAMC 18(2):29-33.
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ABSTRACT: Advances in molecular biology have led to the identification of mutations within several novel genes associated with the phenotype of isolated growth hormone deficiency, combined pituitary hormone deficiency, and syndromes such as septo-optic dysplasia. Progress has also been made in terms of the optimum diagnosis of disorders of stature and their treatment. The use of growth hormone for the treatment of adults with growth hormone deficiency and conditions such as Turner's syndrome, Prader-Willi syndrome, intrauterine growth restriction, and chronic renal failure has changed the practice of endocrinology, although cost-benefit implications remain to be established.The Lancet 07/2004; 363(9425):1977-87. DOI:10.1016/S0140-6736(04)16413-1 · 45.22 Impact Factor
Article: Use of growth hormone in children.[Show abstract] [Hide abstract]
ABSTRACT: The introduction of recombinant DNA-derived human growth hormone (rhGH) in the mid-1980s allowed studies to be undertaken in a number of growth disorders other than the classic indication--growth-hormone deficiency (GHD). In patients with GHD, optimizing the dose and frequency of rhGH administration, and early instigation of therapy, has led to near-normalization of final height. The use of rhGH in the treatment of Turner syndrome, Prader-Willi syndrome, intrauterine growth restriction, and chronic renal failure demonstrated the efficacy of therapy, although the increase in final height (5-7 cm) is less than that achieved in GHD. Cost-benefit implications need to be considered in the next phases of evaluating the role of rhGH therapy in these indications. To date, rhGH has only received approval for the management of idiopathic short stature in the US; as with the other wider growth indications, the lack of formal randomized, controlled trials hampers the full evaluation of efficacy, and a cautious approach should, therefore, be adopted for this particular indication. rhGH has a good safety record, although there are current concerns about the possible long-term increased risk of colonic and lymphatic malignancy, which will require monitoring through national cancer registries.Nature Clinical Practice Endocrinology & Metabolism 06/2006; 2(5):260-8. DOI:10.1038/ncpendmet0169 · 7.55 Impact Factor