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Published on: November 5, 2019
Growth hormone deficiency in patients with sickle cell disease and growth failure
Gail Nunlee-Bland1, Sohail R Rana, Patricia E Houston-Yu
1Department of Pediatrics, Howard University College of Medicine, Washington, DC 20060, USA. gnunlee-bland@howard.edu
Insights
Growth hormone (GH) deficiency may contribute to growth failure in children with sickle cell disease (SCD). Treatment with GH significantly improved height in GH-deficient pediatric patients with SCD.
Area of Science:
- Pediatric Endocrinology
- Hematology
- Growth Disorders
Background:
- Growth disorders are prevalent in children with sickle cell disease (SCD).
- The exact causes of growth disturbances in SCD are multifactorial.
- Abnormalities in the growth hormone (GH)/insulin-like growth factor-I (IGF-I) axis are implicated.
Purpose of the Study:
- To assess GH levels via provocative testing in children with SCD experiencing growth failure.
- To evaluate the efficacy of GH treatment in this cohort.
Main Methods:
- Reviewed growth records of 79 children with sickle cell hemoglobinopathies.
- Measured GH levels using arginine and L-Dopa stimulation tests in patients with and without growth failure.
- Longitudinally followed GH-treated, GH-deficient SCD patients for 5 years.
Main Results:
- 16.5% of patients (13/79) exhibited growth failure (height SDS < -2 or growth velocity SDS < -2).
- Five of seven participating children with growth failure received GH treatment for over 3 years, showing significant height SDS improvement.
- One patient who declined GH treatment experienced worsening height SDS, while another was lost to follow-up.
Conclusions:
- GH deficiency is a potential contributor to growth failure in some children with SCD.
- GH therapy may be beneficial for improving growth outcomes in GH-deficient pediatric patients with SCD.
Background:
Growth disorders are common in children with sickle cell disease (SCD). The etiology for growth disturbances in this population appears to be multifactorial. Recent evidence suggests abnormalities in the growth hormone (GH)/insulin-like growth factor-I (IGF-I) and IGF binding protein-3 (IGFBP-3) axis may play a role.
Objective:
To measure GH levels through provocative stimulation in a group of patients with SCD with growth failure, and to evaluate response to treatment.
Patients And Methods:
Growth records were reviewed of 79 children with sickle cell hemoglobinopathies to identify children with growth failure. GH levels were measured in patients with SCD with and without growth failure using arginine and L-Dopa as provocative stimulation tests. Treatment with GH was offered to GH-deficient children with SCD and these patients were followed longitudinally over 5 years.
Results:
Of the 79 patients, 13 (16.5%, all SS) had heights less than -2 SD below the mean or a growth velocity < -2 SD below the mean for age. Seven of the 13 children with growth failure participated in this study. Five patients received GH for 3 or more years and demonstrated significant improvement in their height SDS. One of the two who declined treatment was lost to follow-up and the other had significant worsening of height SDS score.
Conclusion:
GH deficiency may be associated with growth failure in some patients with SCD. These patients may benefit from treatment with GH.
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