Growth Hormone (GH)-Binding Protein in Prepubertal Short Children Born Small for Gestational Age: Effects of Growth Hormone Treatment 1 (original) (raw)
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THE SIGNIFICANCE OF LABORATORY CLASSIFICATION OF ISOLATED GROWTH HORMONE DEFICIENCY.
International Journal of Advanced Research (IJAR), 2019
Background:Short Stature (SS) is defined as a standing height more than 2 standard deviations (SDs) below the mean (or below the 2.5 percentile) for sex. Causes of short stature include familial short stature, constitutional delay, endocrinopathies (e.g. Growth Hormone Deficiency), chronic systemic diseases and syndromic causes of SS. Aim of the study:To show the significance of the laboratory classification of isolated GHD to help for adjustment of dosages and duration of GH treatment. Methods:A retrospective study of children with isolated Growth Hormone Deficiency (GHD) who have been registered in GH Unit in the Al-Zahraa teaching hospital for Maternity and Children at Al Najaf city, Iraq from March 2012 to September 2016. A total number of 122 patients included in this study all of them on GH replacement .The population study were classified according to post-stimulation peak serum GH level into Mild GHD (>8 − <10ng/ml ) ,Moderate GHD (≥5 − ≤ 8ng/ml) and Severe GHD (< 5ng/ml). The cases also grouped according to age into 3 age groups (Group A: < 6 years, Group B: ≥ 6 − <11 years and Group C: ≥11years), sex and residency. In each of these age groups, comparison done between Mild, Moderate and severe isolated GHD. Results:This study showed that in Group A (35 cases) there was no statistically significant difference between Mild, Moderate and Severe isolated GHD regarding pre-treatment height and rate of the height velocity after the first year of GH treatment (p-value=0.6 for pre-treatment height and 0.74 for rate of height velocity H.V.). In Group B (50 cases) and Group C (37 cases) also no statistically significant difference, Group B (p-value=0.53 for pre-treatment height and p-value= 0.92 for H.V.) and Group C (p-value=0.63 for pre-treatment height, p-value= 0.92 for H.V.) Conclusion:No significance of classification of short stature children with isolated GHD according to stimulated peak GH value with cut-off less 10 ng/ml.
Journal of Endocrinological Investigation, 2012
Human GH (hGH) is a heterogeneous protein hormone consisting of several isoforms. This heterogeneity is the consequence of multiple hGH genes, mRNA splicing, post-translational modifications, and peripheral metabolism, and it represents one important reason for the disparity among GH assay results from different laboratories. However, other factors are involved: a) interference from endogenous GH binding proteins; b) different specificities of anti-GH (monoclonal and polyclonal) antibodies; c) different ma-trix effects among the calibrators; d) the use of different calibrators. The measurement of GH levels in response to provocative testing is an essential part of the diagnosis of GH deficiency. For this purpose, an accurate, reproducible and universally valid GH measurement would be highly desirable, but, despite a huge number of efforts in clinical biochemistry, this goal remains elusive. (J. Endocrinol. Invest. 35: 937-944, 2012) © 2012, Editrice Kurtis
European Journal of Endocrinology, 2000
Objective: To investigate which pretreatment variables most significantly affect long-term growth response to GH therapy in children with apparently idiopathic GH deficiency (GHD) treated from a similar and very young age (less than 2 years), for the same period (7 years) and with the same therapeutic protocol. Design and methods: Twelve children with either isolated GHD or multiple pituitary hormone deficiency were treated with biosynthetic human GH (0.7 IU/kg per week) and were examined every 6 months. Height measurements were performed by Harpenden stadiometers. Bone age was evaluated every 12 months. Results: The onset of therapy was followed in all patients by an important height gain, which attained its zenith during the first year of treatment and became progressively less evident during the next 4 years. Cumulative height gain was 3.0 Ϯ 1.7 SDS. Thanks to the therapy, at the end of the 7-year treatment period, average height in the entire series was not significantly far from mean target height (TH) (¹0.7 Ϯ 1.3 vs ¹0.3 Ϯ 0.4 SDS) and average predicted height (PH) (¹0.2 Ϯ 1.4 SDS) was very close to TH. A stepwise regression analysis showed that both catch-up growth under therapy and PH at the end of the 7-year treatment period were positively influenced by birth weight (BW). Conclusions: a) Our 7-year prospective study on GHD infants treated with GH from less than 2 years of age confirmed the importance of early diagnosis and treatment of GHD in childhood. b) The influence of BW on growth response to GH therapy in GHD children persists over time, at least when treatment is begun from less than 2 years of age.
The Journal of Clinical Endocrinology & Metabolism, 2005
In children, GH secretion and sensitivity to GH are influenced by developmental changes. It is not clear whether the response to GH in very young children with GH deficiency (GHD) is the same as that in older, prepubertal children. A cohort of 265 children (180 males and 85 females) with idiopathic GHD from KIGS (Pfizer International Growth Database), with treatment started at less than 3 yr of age (mean age, 1.9 yr; group I) was compared with a cohort of 509 children (331 males and 178 females; group II) with treatment started at 7-8 yr of age (mean age, 7.5 yr). The following differences (P < 0.01) were found (given in mean values) between groups I and II at the start of GH treatment: 9% vs. 5% breech delivery, 38% vs. 14% multiple pituitary hormone deficiency, 4.2 vs. 5.9 ng/ml maximum GH in response to tests, ؊0.1 vs. ؊0.8 midparental height (MPH) SD score (SDS), ؊3.1 vs. ؊2.5 height SDS, 0.83 vs. 0.66 IU/kg⅐wk GH dose. After the first year of GH, the results were: 13.3 vs. 8.6 cm/yr height velocity, and 1.7 vs. 0.6 maximum change in height SDS. Using the previously developed growth prediction models for prepubertal children with idiopathic GHD more than 2 yr of age, our analysis revealed differences in the indexes of responsiveness in prediction models (Studentized residuals SDS, 0.7 vs.؊0.3) and strikingly higher responsiveness to treatment among the young cohort, but with large scatter. Thus, new prediction models of height velocity (centimeters per year) were derived by means of mul-
European Journal of Endocrinology, 2013
ObjectiveShort stature caused by biologically inactive GH is clinically characterized by lack of GH action despite normal-high secretion of GH, pathologically low IGF1 concentrations and marked catch-up growth on GH replacement therapy.Design and methodsAdopted siblings (girl and a boy) of unknown family history were referred for assessment of short stature (−4.5 and −5.6 SDS) at the age of 10 and 8.1 years respectively. They had delayed bone ages (6.8 and 4.5 years), normal GH peaks at stimulation tests, and severely reduced IGF1 concentrations (−3.5 and −4.0 SDS). Genetic analysis of the GH1 gene showed a heterozygous P59S mutation at position involved in binding to GH receptor (GHR).ResultsIsoelectric focusing analysis of secreted GH in patient serum revealed the presence of higher GH-P59S peak compared with that of wt-GH. Furthermore, computational simulation of GH-P59S binding to GHR suggested problems in correct binding of the mutant to the GHR. In vitro GHR binding studies re...
Final height of patients treated for isolated GH deficiency: examination of 83 patients
European Journal of Endocrinology, 1997
The aim of the present study was to evaluate retrospectively the influence of various auxological and laboratory parameters on final height in a group of GH-deficient children after replacement therapy and to compare their final height with that of a group of short children with normal GH secretion and hence not treated. The final height was evaluated of 83 patients (51 males and 32 females) affected by idiopathic isolated GH deficiency and treated with recombinant human GH (hGH) for 2-7 years. Inclusion criteria at the start of treatment were short stature (mean height for chronological age in standard deviation score (SDS) -2.21) due to idiopathic isolated GH deficiency (GH peak…