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Factors affecting prepubertal growth in homozygous sickle cell disease
A Singhal1, J Morris, P Thomas
1Medical Research Council Laboratories, University of the West Indies, Kingston, Jamaica.
Insights
Fetal hemoglobin concentration positively impacts growth in prepubertal boys with sickle cell (SS) disease, suggesting gender-specific management is crucial. Low fetal hemoglobin may increase hemolysis and metabolic demands, hindering growth in boys.
Area of Science:
- Pediatric Hematology
- Growth and Development
- Sickle Cell Disease
Background:
- Homozygous sickle cell (SS) disease significantly affects child development.
- Understanding factors influencing prepubertal growth in SS disease is critical for intervention.
Purpose of the Study:
- To investigate the influence of hematological indices, socioeconomic status, and morbidity on prepubertal growth in children with SS disease.
Main Methods:
- A cohort study involving 315 children with SS disease was conducted from birth to 9 years.
- Serial measurements of height, weight, and hematological parameters were recorded.
- Statistical analyses, including correlation and partial correlation, were employed.
Main Results:
- Height and weight at age 7 years correlated positively with hemoglobin and fetal hemoglobin in boys, but not girls.
- Fetal hemoglobin concentration, specifically F reticulocyte count, showed a positive correlation with height in both sexes.
- Growth was not significantly associated with mean red cell volume, reticulocyte count, alpha-thalassemia, socioeconomic status, or morbidity.
Conclusions:
- Higher fetal hemoglobin concentrations are linked to enhanced linear growth in prepubertal boys with SS disease.
- Lower fetal hemoglobin may increase hemolysis and metabolic demands, contributing to poorer growth in boys.
- Gender-specific differences in hematology-growth relationships necessitate considering sex in future analyses and management strategies for SS disease.
Objective:
To investigate the role of haematological indices, socioeconomic status, and morbidity in prepubertal growth in homozygous sickle cell (SS) disease.
Method:
Height, weight, and haematology were serially recorded in a cohort study of 315 children with SS disease from birth to 9 years at the sickle cell clinic of the University Hospital of the West Indies, Kingston, Jamaica.
Results:
Height increment between 3 and 9 years correlated positively with total haemoglobin at age 7 years in boys but not girls. Attained height and weight at age 7 years correlated positively with haemoglobin and fetal haemoglobin in boys but not girls. Only the correlation between haemoglobin and weight showed a significant gender difference. Partial correlation analysis suggested that the effect of haemoglobin was accounted for by the effect of fetal haemolglobin and further analysis indicated that height correlated with F reticulocyte count (a measure of fetal haemoglobin production) in both sexes but not with the ratio of F cells to F reticulocytes (a measure of F cell enrichment). Growth was not significantly related to mean red cell volume, proportional reticulocyte count, alpha thalassaemia, socioeconomic status, or morbidity.
Conclusion:
A high concentration of fetal haemoglobin in boys with SS disease is associated with greater linear growth. It is postulated that in boys, low concentrations of fetal haemoglobin increase haemolysis and hence metabolic requirements for erythropoiesis, putting them at greater risk of poor growth. Differences in the relationship of haematology and growth between boys and girls with SS disease dictate that future analyses of growth take gender into account.
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