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Effects of delayed pubertal development, nutritional status, and disease severity on longitudinal patterns of growth

Babette S Zemel1, Deborah A Kawchak, Kwaku Ohene-Frempong

  • 1Division of Gastroenterology, Hepatology, and Nutrition, The Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA. zemel@email.chop.edu

Pediatric Research
|April 7, 2007
PubMed

Insights

Children with sickle cell disease (SCD) experience significant growth failure and delayed maturation. These issues are linked to disease severity and nutritional status, highlighting potential areas for intervention.

Area of Science:

  • Pediatrics
  • Hematology
  • Human Growth and Development

Background:

  • Previous research indicates poor growth in children with sickle cell disease (SCD).
  • However, the prevalence, extent, and contributing factors of suboptimal growth in pediatric SCD populations require further investigation.

Purpose of the Study:

  • To prospectively assess the impact of disease severity and nutritional status on growth in children with SCD-SS.
  • To identify correlates of growth and maturation in this population.

Main Methods:

  • A 4-year longitudinal study evaluated 148 children (birth to 18 years) with SCD-SS annually.
  • Assessments included growth parameters, nutritional status, skeletal and sexual maturation, disease severity, dietary intake, and maternal education.

Main Results:

  • Eighty-four percent of subjects showed declining height, weight, or BMI; 38% fell below the 5th percentile.
  • Puberty was delayed by 1-2 years, with a median age at menarche of 13.2 years.
  • Height status decline correlated with puberty and hematological measures in girls, and nutritional status in both sexes.

Conclusions:

  • Growth failure and maturational delay are persistent challenges for children with sickle cell disease (SCD-SS).
  • These outcomes are associated with modifiable factors, particularly nutritional status, suggesting avenues for therapeutic intervention.

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