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Response to growth hormone in children with chondrodysplasia
1Department of Pediatrics, Medical University of South Carolina, Charleston 29425, USA.
Insights
Children with chondrodysplasia show increased growth velocity during growth hormone (GH) therapy. This suggests abnormal growth cartilage can respond to GH treatment, offering potential benefits for growth.
Area of Science:
- Pediatric Endocrinology
- Skeletal Dysplasias
- Growth Hormone Therapy
Background:
- Children with chondrodysplasia face theoretical concerns regarding growth response to growth hormone (GH) therapy due to abnormal growth cartilage.
- Previous observations suggest potential benefits from increased growth velocity, particularly in the initial year of treatment.
Purpose of the Study:
- To evaluate the response of abnormal growth cartilage in children with chondrodysplasia to growth hormone (GH) therapy.
- To assess the impact of GH therapy on growth velocity in patients with achondroplasia and hypochondroplasia.
Main Methods:
- Analysis of data from the National Cooperative Growth Study.
- Inclusion of 14 patients with achondroplasia and 20 patients with hypochondroplasia.
- Treatment with an average GH dose of 0.317 mg/kg per week for an average of 2.6 years.
Main Results:
- Patients with achondroplasia gained an average of 0.7 SD in height.
- Patients with hypochondroplasia gained an average of 0.7 SD in height.
- Observed growth increase during early phases of GH therapy in both patient groups.
Conclusions:
- The findings suggest that abnormal growth cartilage in chondrodysplasia patients can respond to GH therapy.
- Current data indicates a positive short-term effect on growth velocity.
- The long-term effect on final adult height remains undetermined.
Abstract:
Theoretic concerns exist that children with chondrodysplasia will not grow in response to growth hormone (GH) therapy because of an inability of the abnormal growth cartilage to respond. Experience to date, however, suggests that there is an increase in growth velocity, especially during the first year of treatment, which may be beneficial. Growth has increased during the early phases of GH therapy in both patients with achondroplasia and patients with hypochondroplasia. Fourteen patients with achondroplasia in the National Cooperative Growth Study have been treated with an average dose of GH of 0.317 mg/kg per week for an average of 2.6 years and have gained an average of 0.7 SD in height. Twenty patients with hypochondroplasia in the National Cooperative Growth Study have been treated with an average dose of GH of 0.317 mg/kg per week for an average of 2.6 years and have gained an average of 0.7 SD in height. These data suggest that the abnormal growth cartilage in patients with chondrodysplasia responds to GH therapy. The effect on final height cannot be predicted with the currently available data.