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Growth Hormone/Insulin-like Growth Factor 1 Axis Associated with Modifier Factors in Children with Sickle Cell Anemia
Domício Antônio da Costa-Júnior1, Ana Paula Pinho Santos2, Célia Maria da Silva3
1Department of Medicine, Life Sciences Institute, Federal University of Juiz de Fora, Governador Valadares, Minas Gerais, Brazil.
Insights
Sickle cell anemia affects growth in children. Factors like fetal hemoglobin and alpha-thalassemia influence growth hormone/IGF-1 axis, with alpha-thalassemia linked to shorter stature. Hydroxyurea treatment and higher fetal hemoglobin show positive associations.
Area of Science:
- Pediatric Endocrinology
- Hematology
- Genetics
Background:
- Sickle cell anemia (SCA) impacts the growth hormone/insulin-like growth factor 1 (GH/IGF-1) axis.
- Disease severity is modulated by factors including fetal hemoglobin (HbF) levels, alpha-thalassemia co-inheritance, and hydroxyurea treatment.
Purpose of the Study:
- To investigate the relationship between modifying factors and the GH/IGF-1 axis in children with SCA.
- To assess the impact of these factors on growth parameters and height potential.
Main Methods:
- Cross-sectional study of 39 children with SCA.
- Evaluated bone age, adult height prediction, target height, and laboratory parameters.
- Grouped children based on HbF levels, alpha-thalassemia status, and hydroxyurea therapy.
Main Results:
- Children on hydroxyurea or with HbF >10% showed higher adjusted IGF-1/IGFBP-3 levels.
- Absence of alpha-thalassemia was associated with higher adjusted IGF-1/IGFBP-3.
- Alpha-thalassemia co-inheritance correlated with reduced growth potential relative to parental height.
Conclusions:
- Modifying factors are associated with the GH/IGF-1 axis in pediatric SCA.
- Alpha-thalassemia co-inheritance is linked to decreased height in children with SCA, even after adjusting for parental height.
Background:
Sickle cell anemia is a disease that develops episodes of acute pain and multiple organ dysfunction that can affect the growth hormone/insulin-like growth factor 1 (GH/IGF-1) axis. The severity of sickle cell anemia is influenced by modifying factors, such as levels of fetal hemoglobin (HbF), the co-inheritance of alphathalassemia, or treatment with hydroxyurea.
Methods:
This cross-sectional study in children with sickle cell anemia evaluated bone age (BA), adult height prediction (AHP) using BA, a target height (TH) calculated as the mean SDS of the parents, and laboratory parameters. Children were grouped according to serum levels of HbF, co-inheritance of alpha-thalassemia, and hydroxyurea therapy..
Results:
The mean age of the 39 children was 8.2 ± 2.2 years old. The average height was -0.75 ± 0.30 SDS, and 10.3% (4/39) had short stature. Adjusted levels of IGF-1 or IGFBP- 3 were significantly higher in children with sickle cell anemia on hydroxyurea treatment, in children with HbF levels >10%, and in those without alpha-thalassemia. Using SDS, the growth potential of children with sickle cell anemia in relation to their parents calculated by the difference between AHP and TH as well as the difference between children's height and their TH, were lower in children with co-inheritance of alphathalassemia.
Conclusion:
The study showed an association between modifying factors and the GH/IGF-1 axis in children with sickle cell anemia. Additionally, the co-inheritance of alpha-thalassemia was associated with decreased height in these children when adjusted for their parents' height.
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