Abstract
Previous studies of children with sickle cell disease (SCD) reported poor growth and delayed maturation. However, the prevalence, magnitude, and correlates of suboptimal growth remain poorly understood. A prospective longitudinal study was undertaken to determine the effects of disease severity and nutritional status on growth, an indicator of childhood well-being. Children, birth to 18 y of age, with SCD-SS were evaluated annually for 4 y. Growth, nutritional status, skeletal and sexual maturation, disease severity, dietary intake, and maternal education were assessed. In this sample of 148 children (78 females), growth in height, weight, or body mass index declined in 84% of subjects; 38% fell below the 5th percentile in one or more measures. Puberty was delayed 1 to 2 y, and median age at menarche was 13.2 y. Skeletal age was delayed by 0.7 ± 1.4 y overall and by 1.3 ± 1.5 y in children 10 to 15 y old. Height status declined over time and was positively associated with advancing puberty and hematological measures in girls, and nutritional status in girls and boys. Growth failure and maturational delay remain significant chronic problems in children with SCD-SS and are related to potentially modifiable factors such as nutritional status.
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Abbreviations
- CSSCD:
-
Comprehensive Study of Sickle Cell Disease
- Hct:
-
hematocrit
- Hgb:
-
hemoglobin
- NHANES III:
-
Third National Health and Nutrition Examination Survey
- SCD:
-
sickle cell disease
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Acknowledgements
The authors are grateful to the children and their families for participating in this study and thank the staff of the Comprehensive Sickle Cell Center, the General Clinical Research Center, and the Nutrition and Growth Laboratory of The Children's Hospital of Philadelphia for their time and effort.
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Supported by the General Clinical Research Center (NIH/NCRR grant M01-RR00240), the Comprehensive Sickle Cell Center (NIH HL 38633), and the Nutrition Center of the Children's Hospital of Philadelphia.
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Zemel, B., Kawchak, D., Ohene-Frempong, K. et al. Effects of Delayed Pubertal Development, Nutritional Status, and Disease Severity on Longitudinal Patterns of Growth Failure in Children With Sickle Cell Disease. Pediatr Res 61, 607–613 (2007). https://doi.org/10.1203/pdr.0b013e318045bdca
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DOI: https://doi.org/10.1203/pdr.0b013e318045bdca
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