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Jeanclaude Carel - One of the best experts on this subject based on the ideXlab platform.
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long term mortality after Recombinant Growth Hormone treatment for isolated Growth Hormone deficiency or childhood short stature preliminary report of the french saghe study
The Journal of Clinical Endocrinology and Metabolism, 2012Co-Authors: Jeanclaude Carel, Emmanuel Ecosse, Fabienne Landier, Djamila Meguellatihakkas, Florentia Kaguelidou, Gregoire Rey, Joel CosteAbstract:Context: Little is known about the long-term health of subjects treated with GH in childhood, and Safety and Appropriateness of Growth Hormone treatments in Europe (SAGhE) is a study addressing this question. Objective: The objective of the study was to evaluate the long-term mortality of patients treated with Recombinant GH in childhood in France. Design: This was a population-based cohort study. Setting: The setting of the study was a French population-based register. Participants: A total of 6928 children with idiopathic isolated GH deficiency (n = 5162), neurosecretory dysfunction (n = 534), idiopathic short stature (n = 871), or born short for gestational age (n = 335) who started treatment between 1985 and 1996 participated in the study. Follow-up data on vital status were available in September 2009 for 94.7% of the patients. Main outcome measures: All-cause and cause-specific mortality was measured in the study. Results: All-cause mortality was increased in treated subjects [standardized mortality...
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long term mortality after Recombinant Growth Hormone treatment for isolated Growth Hormone deficiency or childhood short stature preliminary report of the french saghe study
The Journal of Clinical Endocrinology and Metabolism, 2012Co-Authors: Jeanclaude Carel, Emmanuel Ecosse, Fabienne Landier, Djamila Meguellatihakkas, Florentia Kaguelidou, Joel CosteAbstract:CONTEXT: Little is known about the long-term health of subjects treated with GH in childhood, and Safety and Appropriateness of Growth Hormone treatments in Europe (SAGhE) is a study addressing this question. OBJECTIVE: The objective of the study was to evaluate the long-term mortality of patients treated with Recombinant GH in childhood in France. DESIGN: This was a population-based cohort study. SETTING: The setting of the study was a French population-based register. PARTICIPANTS: A total of 6928 children with idiopathic isolated GH deficiency (n = 5162), neurosecretory dysfunction (n = 534), idiopathic short stature (n = 871), or born short for gestational age (n = 335) who started treatment between 1985 and 1996 participated in the study. Follow-up data on vital status were available in September 2009 for 94.7% of the patients. MAIN OUTCOME MEASURES: All-cause and cause-specific mortality was measured in the study. RESULTS: All-cause mortality was increased in treated subjects [standardized mortality ratio (SMR) 1.33, 95% confidence interval (CI) 1.08-1.64]. In a multivariate analysis adjusted for height, the use of GH doses greater than 50 μg/kg · d was associated with mortality rates using external and internal references (SMR 2.94, 95% CI 1.22-7.07, hazard ratio 2.79, 95% CI 1.14-6.82). All type cancer-related mortality was not increased. Bone tumor-related mortality was increased (SMR 5.00, 95% CI 1.01-14.63). An increase in mortality due to diseases of the circulatory system (SMR 3.07, 95% CI 1.40-5.83) or subarachnoid or intracerebral hemorrhage (SMR 6.66, 95% CI 1.79-17.05) was observed. CONCLUSIONS: Mortality rates were increased in this population of adults treated as children with Recombinant GH, particularly in those who had received the highest doses. Specific effects were detected in terms of death due to bone tumors or cerebral hemorrhage but not for all cancers. These results highlight the need for additional studies of long-term mortality and morbidity after GH treatment in childhood.
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long term mortality and causes of death in isolated ghd iss and sga patients treated with Recombinant Growth Hormone during childhood in belgium the netherlands and sweden preliminary report of 3 countries participating in the eu saghe study
The Journal of Clinical Endocrinology and Metabolism, 2012Co-Authors: Lars Savendahl, Kerstin Albertssonwikland, Jeanclaude Carel, Birgit Borgström, Marc Maes, Severine Henrard, Niko Speybroeck, Muriel Thomas, Gladys R J Zandwijken, Anita C S HokkenkoelegaAbstract:CONTEXT: The long-term mortality in adults treated with Recombinant GH during childhood has been poorly investigated. Recently released data from the French part of the European Union Safety and Appropriateness of GH treatments in Europe (EU SAGhE) study have raised concerns on the long-term safety of GH treatment. OBJECTIVE: To report preliminary data on long-term vital status and causes of death in patients with isolated GH deficiency or idiopathic short stature or born small for gestational age treated with GH during childhood, in Belgium, The Netherlands, and Sweden. DESIGN: Data were retrieved from national registries of GH-treated patients and vital status from National Population Registries. Causes of death were retrieved from a National Cause of Death Register (Sweden), Federal and Regional Death Registries (Belgium), or individual patient records (The Netherlands). PATIENTS: All patients diagnosed with isolated GH deficiency or idiopathic short stature or born small for gestational age started on Recombinant GH during childhood from 1985-1997 and who had attained 18 yr of age by the end of 2010 were included. Vital status was available for approximately 98% of these 2,543 patients, corresponding to 46,556 person-years of observation. MAIN OUTCOME MEASURE: Vital status, causes of death, age at death, year of death, duration of GH treatment, and mean GH dose during treatment were assessed. RESULTS: Among 21 deaths identified, 12 were due to accidents, four were suicides, and one patient each died from pneumonia, endocrine dysfunction, primary cardiomyopathy, deficiency of humoral immunity, and coagulation defect. CONCLUSIONS: In these cohorts, the majority of deaths (76%) were caused by accidents or suicides. Importantly, none of the patients died from cancer or from a cardiovascular disease.
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adult height and pubertal Growth in turner syndrome after treatment with Recombinant Growth Hormone
The Journal of Clinical Endocrinology and Metabolism, 2005Co-Authors: Leandro Sorianoguillen, Emmanuel Ecosse, Marc Nicolino, Maite Tauber, Juliane Leger, Sylvie Cabrol, J L Chaussain, Joel Coste, R Brauner, Jeanclaude CarelAbstract:Objective: The objective of this study was to evaluate factors affecting adult height (AH) in patients with Turner syndrome treated with GH. Design: The study design was a population-based cohort study. Setting: The setting was The StaTur Study, a register of patients treated in France between 1986 and 1997, followed for a mean of 9.3 yr. Patients: We followed 704 of the 891 eligible patients (79%) to AH. Intervention: GH (0.8 0.2 IU/kgwk; 0.26 0.06 mg/kgwk; mean SD) was administered for 5.0 2.2 yr. Puberty was classified as spontaneous (10%), spontaneous with secondary estrogens (13%), or induced (77%). Estrogen treatment was initiated at 15.0 1.9 yr of age in those with induced puberty. Main Outcome Measure: The main outcome measure was multivariate analysis of AH after grouping potential predictors. Results: The mean AH was 149.9 6.1 cm, 8.5 cm above projected height. The model explained 90% of the variance, with major effects of age at initiation and duration of treatment. Other factors included birth length, target height, bone age delay and weight at initiation of treatment, age at pubertal onset, GH dose, and number of injections per week. Age at introduction of estrogens was not a predictor, and the use of percutaneousvs. oral estrogens was associated with greater height (2.1 cm; 95% confidence interval, 1.00 –3.25). Conclusions: Our results support the early initiation of GH treatment and induction of puberty at a physiological age to achieve optimal AH. They suggest that GH should be injected daily, and percutaneous estrogens used. These results should be considered in the context of the lack of demonstrable influence of AH on psycho-social outcomes, uncertainties regarding long-term safety, and treatment cost. (J Clin Endocrinol Metab 90: 5197–5204, 2005)
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adult height after long term treatment with Recombinant Growth Hormone for idiopathic isolated Growth Hormone deficiency observational follow up study of the french population based registry
BMJ, 2002Co-Authors: Jeanclaude Carel, Emmanuel Ecosse, Marc Nicolino, Maite Tauber, Juliane Leger, Sylvie Cabrol, Irene Bastiesigeac, J L Chaussain, Joel CosteAbstract:Abstract Objective: To evaluate the efficacy of Recombinant Growth Hormone for increasing adult height in children treated for idiopathic isolated Growth Hormone deficiency. Design: Observational follow up study. Setting: Population based registry. Participants: All 2852 French children diagnosed as having isolated idiopathic Growth Hormone deficiency whose treatment started between 1987 and 1992 and ended before 1996. Main outcome measures: Change in height between the start of treatment and adulthood; classification of patients according to whether treatment was completed as scheduled or stopped early. Results: Adult height was obtained for 2165 (76%) patients. The mean dose of Growth Hormone at start of treatment was 0.42 IU/kg/week. Height gain was 1.1 (SD 0.9) standard deviation (SD) scores, resulting in an adult height of −1.6 (0.9) SD score (girls, 154 (5) cm; boys, 167 (6) cm). Patients who completed the treatment gained 1.0 (0.7) SD score of height in 3.6 (1.4) years. Patients with treatments stopped early gained 0.6 (0.6) SD score in 2.7 (1.4) years while receiving treatment and a further 0.4 (0.9) SD score after the end of treatment. Most of the variation in height gain was explained by regression towards the mean, patients9 characteristics, and delay in starting puberty. Severe Growth Hormone deficiency was associated with better outcome. Each year of treatment was associated with a gain of 0.2 SD score(1.3 cm). Conclusion: The effect of Growth Hormone is unclear in many patients treated for so called idiopathic isolated Growth Hormone deficiency. Most of the patients have pubertal delay and a spontaneous Growth potential, which must be taken into account when measuring the effect and cost effectiveness of treatments. Growth Hormone deficiency should be clearly distinguished from pubertal delay, and criteria should restrict the definition to patients with severely and permanently altered Growth Hormone secretion as our results support the use of Growth Hormone in such patients. Long term trials are required for most patients currently treated. What is already known on this topic Large numbers of children are treated with Recombinant Growth Hormone for so called idiopathic isolated Growth Hormone deficiency The effect on adult height is unclear because of a lack of controlled trials and analysis, and that subgroups, rather than entire populations, are analysed. What this study adds Half the patients treated for idiopathic isolated Growth Hormone deficiency stop treatment before reaching adult height and achieve adult heights similar to those of patients who complete their treatment Many patients diagnosed as having Growth Hormone deficiency actually have pubertal delay A small proportion of patients with severe Growth Hormone deficiency respond better to treatment than patients with less severe Growth Hormone deficiency
Joel Coste - One of the best experts on this subject based on the ideXlab platform.
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Description of the SAGhE Cohort: A Large European Study of Mortality and Cancer Incidence Risks after Childhood Treatment with Recombinant Growth Hormone
Hormone Research in Paediatrics, 2015Co-Authors: Anthony Swerdlow, Joel Coste, Rosie Cooke, Kerstin Albertsson-wikland, Birgit Borgström, Gary Butler, Stefano Cianfarani, Peter Clayton, Annalisa Deodati, Emmanuel EcosseAbstract:BACKGROUND: The long-term safety of Growth Hormone treatment is uncertain. Raised risks of death and certain cancers have been reported inconsistently, based on limited data or short-term follow-up by pharmaceutical companies. PATIENTS AND METHODS: The SAGhE (Safety and Appropriateness of Growth Hormone Treatments in Europe) study assembled cohorts of patients treated in childhood with Recombinant human Growth Hormone (r-hGH) in 8 European countries since the first use of this treatment in 1984 and followed them for cause-specific mortality and cancer incidence. Expected rates were obtained from national and local general population data. The cohort consisted of 24,232 patients, most commonly treated for isolated Growth failure (53%), Turner syndrome (13%) and Growth Hormone deficiency linked to neoplasia (12%). This paper describes in detail the study design, methods and data collection and discusses the strengths, biases and weaknesses consequent on this. CONCLUSION: The SAGhE cohort is the largest and longest follow-up cohort study of Growth Hormone-treated patients with follow-up and analysis independent of industry. It forms a major resource for investigating cancer and mortality risks in r-hGH patients. The interpretation of SAGhE results, however, will need to take account of the methods of cohort assembly and follow-up in each country.
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long term mortality after Recombinant Growth Hormone treatment for isolated Growth Hormone deficiency or childhood short stature preliminary report of the french saghe study
The Journal of Clinical Endocrinology and Metabolism, 2012Co-Authors: Jeanclaude Carel, Emmanuel Ecosse, Fabienne Landier, Djamila Meguellatihakkas, Florentia Kaguelidou, Gregoire Rey, Joel CosteAbstract:Context: Little is known about the long-term health of subjects treated with GH in childhood, and Safety and Appropriateness of Growth Hormone treatments in Europe (SAGhE) is a study addressing this question. Objective: The objective of the study was to evaluate the long-term mortality of patients treated with Recombinant GH in childhood in France. Design: This was a population-based cohort study. Setting: The setting of the study was a French population-based register. Participants: A total of 6928 children with idiopathic isolated GH deficiency (n = 5162), neurosecretory dysfunction (n = 534), idiopathic short stature (n = 871), or born short for gestational age (n = 335) who started treatment between 1985 and 1996 participated in the study. Follow-up data on vital status were available in September 2009 for 94.7% of the patients. Main outcome measures: All-cause and cause-specific mortality was measured in the study. Results: All-cause mortality was increased in treated subjects [standardized mortality...
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long term mortality after Recombinant Growth Hormone treatment for isolated Growth Hormone deficiency or childhood short stature preliminary report of the french saghe study
The Journal of Clinical Endocrinology and Metabolism, 2012Co-Authors: Jeanclaude Carel, Emmanuel Ecosse, Fabienne Landier, Djamila Meguellatihakkas, Florentia Kaguelidou, Joel CosteAbstract:CONTEXT: Little is known about the long-term health of subjects treated with GH in childhood, and Safety and Appropriateness of Growth Hormone treatments in Europe (SAGhE) is a study addressing this question. OBJECTIVE: The objective of the study was to evaluate the long-term mortality of patients treated with Recombinant GH in childhood in France. DESIGN: This was a population-based cohort study. SETTING: The setting of the study was a French population-based register. PARTICIPANTS: A total of 6928 children with idiopathic isolated GH deficiency (n = 5162), neurosecretory dysfunction (n = 534), idiopathic short stature (n = 871), or born short for gestational age (n = 335) who started treatment between 1985 and 1996 participated in the study. Follow-up data on vital status were available in September 2009 for 94.7% of the patients. MAIN OUTCOME MEASURES: All-cause and cause-specific mortality was measured in the study. RESULTS: All-cause mortality was increased in treated subjects [standardized mortality ratio (SMR) 1.33, 95% confidence interval (CI) 1.08-1.64]. In a multivariate analysis adjusted for height, the use of GH doses greater than 50 μg/kg · d was associated with mortality rates using external and internal references (SMR 2.94, 95% CI 1.22-7.07, hazard ratio 2.79, 95% CI 1.14-6.82). All type cancer-related mortality was not increased. Bone tumor-related mortality was increased (SMR 5.00, 95% CI 1.01-14.63). An increase in mortality due to diseases of the circulatory system (SMR 3.07, 95% CI 1.40-5.83) or subarachnoid or intracerebral hemorrhage (SMR 6.66, 95% CI 1.79-17.05) was observed. CONCLUSIONS: Mortality rates were increased in this population of adults treated as children with Recombinant GH, particularly in those who had received the highest doses. Specific effects were detected in terms of death due to bone tumors or cerebral hemorrhage but not for all cancers. These results highlight the need for additional studies of long-term mortality and morbidity after GH treatment in childhood.
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adult height and pubertal Growth in turner syndrome after treatment with Recombinant Growth Hormone
The Journal of Clinical Endocrinology and Metabolism, 2005Co-Authors: Leandro Sorianoguillen, Emmanuel Ecosse, Marc Nicolino, Maite Tauber, Juliane Leger, Sylvie Cabrol, J L Chaussain, Joel Coste, R Brauner, Jeanclaude CarelAbstract:Objective: The objective of this study was to evaluate factors affecting adult height (AH) in patients with Turner syndrome treated with GH. Design: The study design was a population-based cohort study. Setting: The setting was The StaTur Study, a register of patients treated in France between 1986 and 1997, followed for a mean of 9.3 yr. Patients: We followed 704 of the 891 eligible patients (79%) to AH. Intervention: GH (0.8 0.2 IU/kgwk; 0.26 0.06 mg/kgwk; mean SD) was administered for 5.0 2.2 yr. Puberty was classified as spontaneous (10%), spontaneous with secondary estrogens (13%), or induced (77%). Estrogen treatment was initiated at 15.0 1.9 yr of age in those with induced puberty. Main Outcome Measure: The main outcome measure was multivariate analysis of AH after grouping potential predictors. Results: The mean AH was 149.9 6.1 cm, 8.5 cm above projected height. The model explained 90% of the variance, with major effects of age at initiation and duration of treatment. Other factors included birth length, target height, bone age delay and weight at initiation of treatment, age at pubertal onset, GH dose, and number of injections per week. Age at introduction of estrogens was not a predictor, and the use of percutaneousvs. oral estrogens was associated with greater height (2.1 cm; 95% confidence interval, 1.00 –3.25). Conclusions: Our results support the early initiation of GH treatment and induction of puberty at a physiological age to achieve optimal AH. They suggest that GH should be injected daily, and percutaneous estrogens used. These results should be considered in the context of the lack of demonstrable influence of AH on psycho-social outcomes, uncertainties regarding long-term safety, and treatment cost. (J Clin Endocrinol Metab 90: 5197–5204, 2005)
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adult height after long term treatment with Recombinant Growth Hormone for idiopathic isolated Growth Hormone deficiency observational follow up study of the french population based registry
BMJ, 2002Co-Authors: Jeanclaude Carel, Emmanuel Ecosse, Marc Nicolino, Maite Tauber, Juliane Leger, Sylvie Cabrol, Irene Bastiesigeac, J L Chaussain, Joel CosteAbstract:Abstract Objective: To evaluate the efficacy of Recombinant Growth Hormone for increasing adult height in children treated for idiopathic isolated Growth Hormone deficiency. Design: Observational follow up study. Setting: Population based registry. Participants: All 2852 French children diagnosed as having isolated idiopathic Growth Hormone deficiency whose treatment started between 1987 and 1992 and ended before 1996. Main outcome measures: Change in height between the start of treatment and adulthood; classification of patients according to whether treatment was completed as scheduled or stopped early. Results: Adult height was obtained for 2165 (76%) patients. The mean dose of Growth Hormone at start of treatment was 0.42 IU/kg/week. Height gain was 1.1 (SD 0.9) standard deviation (SD) scores, resulting in an adult height of −1.6 (0.9) SD score (girls, 154 (5) cm; boys, 167 (6) cm). Patients who completed the treatment gained 1.0 (0.7) SD score of height in 3.6 (1.4) years. Patients with treatments stopped early gained 0.6 (0.6) SD score in 2.7 (1.4) years while receiving treatment and a further 0.4 (0.9) SD score after the end of treatment. Most of the variation in height gain was explained by regression towards the mean, patients9 characteristics, and delay in starting puberty. Severe Growth Hormone deficiency was associated with better outcome. Each year of treatment was associated with a gain of 0.2 SD score(1.3 cm). Conclusion: The effect of Growth Hormone is unclear in many patients treated for so called idiopathic isolated Growth Hormone deficiency. Most of the patients have pubertal delay and a spontaneous Growth potential, which must be taken into account when measuring the effect and cost effectiveness of treatments. Growth Hormone deficiency should be clearly distinguished from pubertal delay, and criteria should restrict the definition to patients with severely and permanently altered Growth Hormone secretion as our results support the use of Growth Hormone in such patients. Long term trials are required for most patients currently treated. What is already known on this topic Large numbers of children are treated with Recombinant Growth Hormone for so called idiopathic isolated Growth Hormone deficiency The effect on adult height is unclear because of a lack of controlled trials and analysis, and that subgroups, rather than entire populations, are analysed. What this study adds Half the patients treated for idiopathic isolated Growth Hormone deficiency stop treatment before reaching adult height and achieve adult heights similar to those of patients who complete their treatment Many patients diagnosed as having Growth Hormone deficiency actually have pubertal delay A small proportion of patients with severe Growth Hormone deficiency respond better to treatment than patients with less severe Growth Hormone deficiency
Emmanuel Ecosse - One of the best experts on this subject based on the ideXlab platform.
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Description of the SAGhE Cohort: A Large European Study of Mortality and Cancer Incidence Risks after Childhood Treatment with Recombinant Growth Hormone
Hormone Research in Paediatrics, 2015Co-Authors: Anthony Swerdlow, Joel Coste, Rosie Cooke, Kerstin Albertsson-wikland, Birgit Borgström, Gary Butler, Stefano Cianfarani, Peter Clayton, Annalisa Deodati, Emmanuel EcosseAbstract:BACKGROUND: The long-term safety of Growth Hormone treatment is uncertain. Raised risks of death and certain cancers have been reported inconsistently, based on limited data or short-term follow-up by pharmaceutical companies. PATIENTS AND METHODS: The SAGhE (Safety and Appropriateness of Growth Hormone Treatments in Europe) study assembled cohorts of patients treated in childhood with Recombinant human Growth Hormone (r-hGH) in 8 European countries since the first use of this treatment in 1984 and followed them for cause-specific mortality and cancer incidence. Expected rates were obtained from national and local general population data. The cohort consisted of 24,232 patients, most commonly treated for isolated Growth failure (53%), Turner syndrome (13%) and Growth Hormone deficiency linked to neoplasia (12%). This paper describes in detail the study design, methods and data collection and discusses the strengths, biases and weaknesses consequent on this. CONCLUSION: The SAGhE cohort is the largest and longest follow-up cohort study of Growth Hormone-treated patients with follow-up and analysis independent of industry. It forms a major resource for investigating cancer and mortality risks in r-hGH patients. The interpretation of SAGhE results, however, will need to take account of the methods of cohort assembly and follow-up in each country.
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long term mortality after Recombinant Growth Hormone treatment for isolated Growth Hormone deficiency or childhood short stature preliminary report of the french saghe study
The Journal of Clinical Endocrinology and Metabolism, 2012Co-Authors: Jeanclaude Carel, Emmanuel Ecosse, Fabienne Landier, Djamila Meguellatihakkas, Florentia Kaguelidou, Gregoire Rey, Joel CosteAbstract:Context: Little is known about the long-term health of subjects treated with GH in childhood, and Safety and Appropriateness of Growth Hormone treatments in Europe (SAGhE) is a study addressing this question. Objective: The objective of the study was to evaluate the long-term mortality of patients treated with Recombinant GH in childhood in France. Design: This was a population-based cohort study. Setting: The setting of the study was a French population-based register. Participants: A total of 6928 children with idiopathic isolated GH deficiency (n = 5162), neurosecretory dysfunction (n = 534), idiopathic short stature (n = 871), or born short for gestational age (n = 335) who started treatment between 1985 and 1996 participated in the study. Follow-up data on vital status were available in September 2009 for 94.7% of the patients. Main outcome measures: All-cause and cause-specific mortality was measured in the study. Results: All-cause mortality was increased in treated subjects [standardized mortality...
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long term mortality after Recombinant Growth Hormone treatment for isolated Growth Hormone deficiency or childhood short stature preliminary report of the french saghe study
The Journal of Clinical Endocrinology and Metabolism, 2012Co-Authors: Jeanclaude Carel, Emmanuel Ecosse, Fabienne Landier, Djamila Meguellatihakkas, Florentia Kaguelidou, Joel CosteAbstract:CONTEXT: Little is known about the long-term health of subjects treated with GH in childhood, and Safety and Appropriateness of Growth Hormone treatments in Europe (SAGhE) is a study addressing this question. OBJECTIVE: The objective of the study was to evaluate the long-term mortality of patients treated with Recombinant GH in childhood in France. DESIGN: This was a population-based cohort study. SETTING: The setting of the study was a French population-based register. PARTICIPANTS: A total of 6928 children with idiopathic isolated GH deficiency (n = 5162), neurosecretory dysfunction (n = 534), idiopathic short stature (n = 871), or born short for gestational age (n = 335) who started treatment between 1985 and 1996 participated in the study. Follow-up data on vital status were available in September 2009 for 94.7% of the patients. MAIN OUTCOME MEASURES: All-cause and cause-specific mortality was measured in the study. RESULTS: All-cause mortality was increased in treated subjects [standardized mortality ratio (SMR) 1.33, 95% confidence interval (CI) 1.08-1.64]. In a multivariate analysis adjusted for height, the use of GH doses greater than 50 μg/kg · d was associated with mortality rates using external and internal references (SMR 2.94, 95% CI 1.22-7.07, hazard ratio 2.79, 95% CI 1.14-6.82). All type cancer-related mortality was not increased. Bone tumor-related mortality was increased (SMR 5.00, 95% CI 1.01-14.63). An increase in mortality due to diseases of the circulatory system (SMR 3.07, 95% CI 1.40-5.83) or subarachnoid or intracerebral hemorrhage (SMR 6.66, 95% CI 1.79-17.05) was observed. CONCLUSIONS: Mortality rates were increased in this population of adults treated as children with Recombinant GH, particularly in those who had received the highest doses. Specific effects were detected in terms of death due to bone tumors or cerebral hemorrhage but not for all cancers. These results highlight the need for additional studies of long-term mortality and morbidity after GH treatment in childhood.
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adult height and pubertal Growth in turner syndrome after treatment with Recombinant Growth Hormone
The Journal of Clinical Endocrinology and Metabolism, 2005Co-Authors: Leandro Sorianoguillen, Emmanuel Ecosse, Marc Nicolino, Maite Tauber, Juliane Leger, Sylvie Cabrol, J L Chaussain, Joel Coste, R Brauner, Jeanclaude CarelAbstract:Objective: The objective of this study was to evaluate factors affecting adult height (AH) in patients with Turner syndrome treated with GH. Design: The study design was a population-based cohort study. Setting: The setting was The StaTur Study, a register of patients treated in France between 1986 and 1997, followed for a mean of 9.3 yr. Patients: We followed 704 of the 891 eligible patients (79%) to AH. Intervention: GH (0.8 0.2 IU/kgwk; 0.26 0.06 mg/kgwk; mean SD) was administered for 5.0 2.2 yr. Puberty was classified as spontaneous (10%), spontaneous with secondary estrogens (13%), or induced (77%). Estrogen treatment was initiated at 15.0 1.9 yr of age in those with induced puberty. Main Outcome Measure: The main outcome measure was multivariate analysis of AH after grouping potential predictors. Results: The mean AH was 149.9 6.1 cm, 8.5 cm above projected height. The model explained 90% of the variance, with major effects of age at initiation and duration of treatment. Other factors included birth length, target height, bone age delay and weight at initiation of treatment, age at pubertal onset, GH dose, and number of injections per week. Age at introduction of estrogens was not a predictor, and the use of percutaneousvs. oral estrogens was associated with greater height (2.1 cm; 95% confidence interval, 1.00 –3.25). Conclusions: Our results support the early initiation of GH treatment and induction of puberty at a physiological age to achieve optimal AH. They suggest that GH should be injected daily, and percutaneous estrogens used. These results should be considered in the context of the lack of demonstrable influence of AH on psycho-social outcomes, uncertainties regarding long-term safety, and treatment cost. (J Clin Endocrinol Metab 90: 5197–5204, 2005)
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adult height after long term treatment with Recombinant Growth Hormone for idiopathic isolated Growth Hormone deficiency observational follow up study of the french population based registry
BMJ, 2002Co-Authors: Jeanclaude Carel, Emmanuel Ecosse, Marc Nicolino, Maite Tauber, Juliane Leger, Sylvie Cabrol, Irene Bastiesigeac, J L Chaussain, Joel CosteAbstract:Abstract Objective: To evaluate the efficacy of Recombinant Growth Hormone for increasing adult height in children treated for idiopathic isolated Growth Hormone deficiency. Design: Observational follow up study. Setting: Population based registry. Participants: All 2852 French children diagnosed as having isolated idiopathic Growth Hormone deficiency whose treatment started between 1987 and 1992 and ended before 1996. Main outcome measures: Change in height between the start of treatment and adulthood; classification of patients according to whether treatment was completed as scheduled or stopped early. Results: Adult height was obtained for 2165 (76%) patients. The mean dose of Growth Hormone at start of treatment was 0.42 IU/kg/week. Height gain was 1.1 (SD 0.9) standard deviation (SD) scores, resulting in an adult height of −1.6 (0.9) SD score (girls, 154 (5) cm; boys, 167 (6) cm). Patients who completed the treatment gained 1.0 (0.7) SD score of height in 3.6 (1.4) years. Patients with treatments stopped early gained 0.6 (0.6) SD score in 2.7 (1.4) years while receiving treatment and a further 0.4 (0.9) SD score after the end of treatment. Most of the variation in height gain was explained by regression towards the mean, patients9 characteristics, and delay in starting puberty. Severe Growth Hormone deficiency was associated with better outcome. Each year of treatment was associated with a gain of 0.2 SD score(1.3 cm). Conclusion: The effect of Growth Hormone is unclear in many patients treated for so called idiopathic isolated Growth Hormone deficiency. Most of the patients have pubertal delay and a spontaneous Growth potential, which must be taken into account when measuring the effect and cost effectiveness of treatments. Growth Hormone deficiency should be clearly distinguished from pubertal delay, and criteria should restrict the definition to patients with severely and permanently altered Growth Hormone secretion as our results support the use of Growth Hormone in such patients. Long term trials are required for most patients currently treated. What is already known on this topic Large numbers of children are treated with Recombinant Growth Hormone for so called idiopathic isolated Growth Hormone deficiency The effect on adult height is unclear because of a lack of controlled trials and analysis, and that subgroups, rather than entire populations, are analysed. What this study adds Half the patients treated for idiopathic isolated Growth Hormone deficiency stop treatment before reaching adult height and achieve adult heights similar to those of patients who complete their treatment Many patients diagnosed as having Growth Hormone deficiency actually have pubertal delay A small proportion of patients with severe Growth Hormone deficiency respond better to treatment than patients with less severe Growth Hormone deficiency
Lubaina M. Rangwala - One of the best experts on this subject based on the ideXlab platform.
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Pediatric idiopathic intracranial hypertension.
Survey of ophthalmology, 2007Co-Authors: Lubaina M. RangwalaAbstract:Our understanding of pediatric idiopathic intracranial hypertension has been refined since Dr. Simmons Lessell's review in 1992. The use of rigorous methodologies and standard definitions in recent studies has demonstrated distinct demographic trends. Specifically, the incidence of idiopathic intracranial hypertension seems to be increasing among adolescent children, and among older children its clinical picture is similar to that of adult idiopathic intracranial hypertension (female and obese). Within younger age groups there are more boys and nonobese children who may develop idiopathic intracranial hypertension. The pathogenesis of the disease has yet to be elucidated. Idiopathic intracranial hypertension among young children has been associated with several new etiologies, including Recombinant Growth Hormone and all-trans-retinoic acid. More modern neuroimaging techniques such as MRI and MRI-venograms are being used to exclude intracranial processes. Although most cases of pediatric idiopathic intracranial hypertension improve with medical treatment, those who have had visual progression despite medical treatment have undergone optic nerve sheath fenestration and lumboperitoneal shunting. Because idiopathic intracranial hypertension in young children appears to be a different disorder than in adolescents and adults, separate diagnostic criteria for younger children are warranted. We propose new criteria for pediatric idiopathic intracranial hypertension in which children should have signs or symptoms consistent with elevated intracranial pressure, be prepubertal, have normal sensorium, can have reversible cranial nerve palsies, and have an opening cerebrospinal fluid pressure greater than 180 mm H2O if less than age 8 and papilledema is present, but greater than 250 mm H20 if age 8 or above or less than 8 without papilledema. (Surv Ophthalmol 52:597--617, 2007. 2007 Elsevier Inc. All rights reserved.)
O V Bolshova - One of the best experts on this subject based on the ideXlab platform.
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Essential microelement contents in the blood plasma of children with biologically inactive Growth Hormone syndrome
Publishing House Zaslavsky, 2018Co-Authors: N.a. 1 Sprinchuk, V.g. Pakhomova, O V BolshovaAbstract:Background. The purpose was to increase the effectiveness of treatment in children and adolescents with biologically inactive Growth Hormone syndrome (BIGHS) based on studying the content of essential microelements (EM) in the blood plasma. Materials and methods. The study included 206 children and adolescents with different forms of short stature. Group with BIGHS consisted of 29 children (14.08 %): 21 boys (72.41 %) and 8 girls (27.59 %), with Growth retardation from –2.0 to –4.6 SD. The average age of children was 9.01 ± 0.60 years. Diagnosis of BIGHS was confirmed by the presence of normal/elevated Growth Hormone parameters against the background of stimulation tests, a sharp decrease in the level of insulinlike Growth factor1 (IGF1), and positive test for Growth Hormone sensitivity. The microelement status was assessed by determining the levels of EM (zinc, selenium, manganese, chromium, copper) in the blood plasma by Xray fluorescence spectrometry using the ElvaXmed spectrometer (Ukraine). Plasma levels of IGF1 were evaluated by immunoradiometric assay (IRMA) using standard IRMA IGF1 kits (Immunotech® kit, Czech Republic). Results. In the group of children with BIGHS, the levels of zinc and selenium were 0.57 ± 0.04 µg/ml and 0.05 ± 0.01 µg/ml, respectively, that is significantly lower than in the general group of children with short stature (p < 0.001 and p < 0.05, respectively). The average plasma levels of chromium and copper were lower than those in children with other forms of short stature and amounted to 0.040 ± 0.003 µg/ml and 0.79 ± 0.05 µg/ml, respectively, but were significantly lower than in the control group (р 0.01). Conclusions. Patients with BIGHS were found to have potentially lower levels of zinc, selenium, chromium and copper in the blood plasma. Thus, in our opinion, it is advisable to include the determination of EM levels in a comprehensive examination of children with short stature. When detecting lower levels of EM, a combined treatment with Recombinant Growth Hormone preparations and those containing appropriate EM is recommended that significantly increases the effectiveness of Growthcorrection therapy
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Essential microelement contents in the blood plasma of children with biologically inactive Growth Hormone syndrome
'Publishing House Zaslavsky', 2018Co-Authors: N.a. 1 Sprinchuk, V.g. Pakhomova, O V BolshovaAbstract:Background. The purpose was to increase the effectiveness of treatment in children and adolescents with biologically inactive Growth Hormone syndrome (BIGHS) based on studying the content of essential microelements (EM) in the blood plasma. Materials and methods. The study included 206 children and adolescents with different forms of short stature. Group with BIGHS consisted of 29 children (14.08 %): 21 boys (72.41 %) and 8 girls (27.59 %), with Growth retardation from –2.0 to –4.6 SD. The average age of children was 9.01 ± 0.60 years. Diagnosis of BIGHS was confirmed by the presence of normal/elevated Growth Hormone parameters against the background of stimulation tests, a sharp decrease in the level of insulinlike Growth factor1 (IGF1), and positive test for Growth Hormone sensitivity. The microelement status was assessed by determining the levels of EM (zinc, selenium, manganese, chromium, copper) in the blood plasma by Xray fluorescence spectrometry using the ElvaXmed spectrometer (Ukraine). Plasma levels of IGF1 were evaluated by immunoradiometric assay (IRMA) using standard IRMA IGF1 kits (Immunotech® kit, Czech Republic). Results. In the group of children with BIGHS, the levels of zinc and selenium were 0.57 ± 0.04 µg/ml and 0.05 ± 0.01 µg/ml, respectively, that is significantly lower than in the general group of children with short stature (p 0.01). Conclusions. Patients with BIGHS were found to have potentially lower levels of zinc, selenium, chromium and copper in the blood plasma. Thus, in our opinion, it is advisable to include the determination of EM levels in a comprehensive examination of children with short stature. When detecting lower levels of EM, a combined treatment with Recombinant Growth Hormone preparations and those containing appropriate EM is recommended that significantly increases the effectiveness of Growthcorrection therapy
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the level of c reactive protein in the blood plasma of young adults with childhood onset Growth Hormone deficiency
International Journal of Endocrinology, 2016Co-Authors: O V Bolshova, T O Tkachova, O A VyshnevskaAbstract:The article presents the data on the level of high-sensitivity C-reactive protein in the blood plasma of young adults with Growth Hormone deficiency that occurred in childhood and persists in adulthood. It was found that prolonged deficiency of endogenous Growth Hormone is associated with a significant increase of C-reactive protein level in the blood plasma of patients. This could significantly enhance the risk of early atherosclerosis and cardiovascular diseases in patients with Growth Hormone deficiency already at a young age. Therapy with Recombinant Growth Hormone preparations leads to normalization of C-reactive protein levels already in 3–6 months after therapy initiation/retreatment.