Article

Growth hormone deficiency.

Department of Chemical Pathology, St James's University Hospital, Leeds, UK.
Annals of Clinical Biochemistry (Impact Factor: 1.92). 10/1987; 24 ( Pt 5):429-34. DOI: 10.1136/bmj.325.7355.58
Source: PubMed
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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.
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    ABSTRACT: Data on the GH-induced catch-up growth of severely GH-deficient children affected by monogenetic defects are missing. Catch-up growth of 21 prepubertal children (6 females, 15 males) affected with IGHD type II was analyzed in a retrospective chart review. At start of therapy, mean age was 6.2 years (range, 1.6-15.0), mean height SDS was -4.7 (-7.6 to -2.2), mean IGF-I SDS was -6.2 (-10.1 to -2.2). GH was substituted using a mean dose of 30.5microg/kg*d. Catch-up growth was characterized by a mean height gain of +0.92, +0.82, and +0.61 SDS after 1, 2, and 3 years of GH therapy, respectively. Mean height velocities were 10.7, 9.2 and 7.7cm/year during the first three years. Mean duration of complete catch-up growth was 6 years (3-9). Mean height SDS reached was -0.97 (-2.3 to +1.1), which was within the range of the estimated target height of -0.60 SDS (-1.20 to -0.15). The younger and shorter the children were at start of therapy the better they grew during the first year independent of the dose. Mean bone age was delayed at start by 2.1 years and progressed by 2.5 years during the first two years of therapy. Incomplete catch-up growth was caused by late initiation or irregular administration of GH in four cases. Our data suggest that GH-treated children with severe IGHD show a sustained catch-up growth over 6 years (mean) and reach their target height range. This response to GH is considered to be characteristic for young children with severe growth retardation due to IGHD.
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