1 Pediatric Research 2007 Vol: 0():607-613. DOI: 10.1203/pdr.0b013e318045bdca

Effects of Delayed Pubertal Development, Nutritional Status, and Disease Severity on Longitudinal Patterns of Growth Failure in Children With Sickle Cell Disease

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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Figures
Figure 1: Prevalence of growth failure was defined as a height, weight, or BMI value below the 5th percentile compared with the Centers for Disease Control and Prevention growth charts at any time during the 4-y observation period. Results are shown for all subjects (open columns) and for girls (solid columns) and boys (hatched columns) separately as follows: The final column shows the prevalence of growth failure in any of the measures combined. Figure 2: All observations for height, weight, and BMI Z score for each subject are shown. Children receiving long-term transfusion therapy are represented by filled circles; nontransfused children are represented by open circles. The figures illustrate the age-related decline in Z scores and that even children with relatively good growth status decline over time.
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References
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    • . . . Studies of children with SCD from the 1960s to 1990s described poor growth and delayed skeletal and sexual maturation (1–5), demonstrating low growth percentiles in height and weight among children older than 2 to 5 y of age, especially for children with type SCD-SS . . .
    • . . . Previous studies of children and adolescents with SCD were conducted up to several decades ago and demonstrated impaired growth in height and weight (1–4,14–16) . . .
    • . . . Although the sample of SCD-SS was small, Phebus et al. (1) also noted some improvement in growth status of females after age 12 y. . . .
    • . . . The age at menarche (13.2 y) was later than the reported age in the NHANES III survey . . .
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    • . . . Nutrient deficiencies based on biomarkers have been reported for vitamins B6 (33,34), D (35), and E (36,37); retinol (38); and zinc (39–41) . . .
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    • . . . Nutrient deficiencies based on biomarkers have been reported for vitamins B6 (33,34), D (35), and E (36,37); retinol (38); and zinc (39–41) . . .
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    • . . . Nutrient deficiencies based on biomarkers have been reported for vitamins B6 (33,34), D (35), and E (36,37); retinol (38); and zinc (39–41) . . .
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