Sustained Benefits of Growth Hormone on Body Composition, Fat Utilization, Physical Strength and Agility, and Growth in Prader-Willi Syndrome are Dose-Dependent
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Aaron L. Carrel
,
Susan E. Myers
Abstract
Background: Obesity and hypotonia in children with Prader-Willi syndrome (PWS) are accompanied by abnormal body composition resembling a growth hormone (GH) deficient state. Hypothalamic dysfunction in PWS includes decreased GH secretion, suggesting a possible therapeutic role for GH treatment. While recent studies have demonstrated short-term benefits of treatment with GH, a critical question is whether beneficial changes persist or wane with prolonged therapy, and whether these effects on body composition are dose-dependent as seen in adult GH deficiency.
Objectives and Methods: After 24 months of GH theapy at a dose of 1 mg/m2/day (“standard dose”), the effects of 12 additional months of GH treatment at varying doses (0.3-1.5 mg/m2/day) on growth, body composition, strength and agility, pulmonary function, resting energy expenditure (REE), and fat utilization were assessed in 46 children with PWS. Percent body fat, lean muscle mass, and bone mineral density (BMD) were measured by dual X-ray absorptiometry (DXA). Indirect calorimetry was used to determine REE and to calculate respiratory quotient (RQ).
Results: During months 24-36 of GH therapy, further changes in body composition (decrease in fat mass, and increase in lean body mass), growth velocity, and REE occurred with standard and higher-dose GH therapy (1.5 mg/m2/day), but not with lower dose GH (0.3 mg/m2/day). Prior improvements in BMD, and strength and agility, which occurred during the initial 24 months, were sustained during the additional 12 months (to 36 months) regardless of dose.
Conclusions: Salutary and sustained . GH- induced changes in growth, body composition, and physical function in children with PWS require GH doses of >0.3 mg/m2/day. Conversely, BMD increased during the additional 12 months of therapy regardless of GH dose. Lower doses of GH, effective in improving body composition in adults with GHD, do not appear to be effective in children with PWS.
© 2016 by Walter de Gruyter Berlin/Boston
Articles in the same Issue
- Titelei
- Table of Contents
- Doping with Growth Hormone
- Intrauterine Diabetic Environment Confers Risks for Type 2 Diabetes Mellitus and Obesity in the Offspring, in Addition to Genetic Susceptibility
- Variable Presentation of X-linked Adrenal Hypoplasia Congenita
- Sustained Benefits of Growth Hormone on Body Composition, Fat Utilization, Physical Strength and Agility, and Growth in Prader-Willi Syndrome are Dose-Dependent
- Insufficient Adrenarche in Patients with Combined Pituitary Hormone Deficiency Caused by a PROP A Gene Defect
- Syndrome of Congenital Adrenocortical Unresponsiveness to ACTH. Report of Six Patients
- Maternal and Fetal Serum Insulin-like Growth Factor-I (IGF-I), IGF Binding Protein-3 (IGFBP-3), Leptin Levels and Early Postnatal Growth in Infants Born Asymmetrically Small for Gestational Age
- Relationships Between Levels of Leptin and Hematological Parameters in Healthy Term Infants
- Recombinant Growth Hormone Treatment in Short Patients with Thalassemia Major: Results after 24 and 36 Months
- Assessment of the Current Status of Iodine Prophylaxis in Bosnia and Herzegovina Federation
- Profiles of Obese Children Presenting for Metabolic Evaluation
- Effect of Growth Hormone Treatment on Hypoglycemia in a Patient with both Hepatic Glycogen Synthase and Isolated Growth Hormone Deficiencies
- Gliclazide-Induced Hepatitis, Hemiplegia and Dysphasia in a Suicide Attempt
- Neonatal Goiter Caused by Expectorant Usage
- Idiopathic Hypothalamic Dysfunction with Precocious Puberty and Adipsic Hypernatremia First Presenting in Adolescence
- Neonatal Hyperthyroidism m Infants of Mothers Previously Thyroidectomized due to Graves’ Disease
- Primary Hyperparathyroidism in an Infant with Three Parathyroid Glands and Pulmonary Calcinosis
- Asymmetric Crying Facies and Congenital Hypothyroidism: Report of Two Patients
- Obituary. Professor Dr. Andrea Prader (1919–2001)
- Meetings Calendar
- Author Index
Articles in the same Issue
- Titelei
- Table of Contents
- Doping with Growth Hormone
- Intrauterine Diabetic Environment Confers Risks for Type 2 Diabetes Mellitus and Obesity in the Offspring, in Addition to Genetic Susceptibility
- Variable Presentation of X-linked Adrenal Hypoplasia Congenita
- Sustained Benefits of Growth Hormone on Body Composition, Fat Utilization, Physical Strength and Agility, and Growth in Prader-Willi Syndrome are Dose-Dependent
- Insufficient Adrenarche in Patients with Combined Pituitary Hormone Deficiency Caused by a PROP A Gene Defect
- Syndrome of Congenital Adrenocortical Unresponsiveness to ACTH. Report of Six Patients
- Maternal and Fetal Serum Insulin-like Growth Factor-I (IGF-I), IGF Binding Protein-3 (IGFBP-3), Leptin Levels and Early Postnatal Growth in Infants Born Asymmetrically Small for Gestational Age
- Relationships Between Levels of Leptin and Hematological Parameters in Healthy Term Infants
- Recombinant Growth Hormone Treatment in Short Patients with Thalassemia Major: Results after 24 and 36 Months
- Assessment of the Current Status of Iodine Prophylaxis in Bosnia and Herzegovina Federation
- Profiles of Obese Children Presenting for Metabolic Evaluation
- Effect of Growth Hormone Treatment on Hypoglycemia in a Patient with both Hepatic Glycogen Synthase and Isolated Growth Hormone Deficiencies
- Gliclazide-Induced Hepatitis, Hemiplegia and Dysphasia in a Suicide Attempt
- Neonatal Goiter Caused by Expectorant Usage
- Idiopathic Hypothalamic Dysfunction with Precocious Puberty and Adipsic Hypernatremia First Presenting in Adolescence
- Neonatal Hyperthyroidism m Infants of Mothers Previously Thyroidectomized due to Graves’ Disease
- Primary Hyperparathyroidism in an Infant with Three Parathyroid Glands and Pulmonary Calcinosis
- Asymmetric Crying Facies and Congenital Hypothyroidism: Report of Two Patients
- Obituary. Professor Dr. Andrea Prader (1919–2001)
- Meetings Calendar
- Author Index
