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Bone area and bone mineral content deficits in children with sickle cell disease

Anne M Buison1, Deborah A Kawchak, Joan I Schall

  • 1Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

Pediatrics
|October 4, 2005
PubMed

Insights

Children with sickle cell disease (SCD) have lower bone mineral content (BMC) despite accounting for growth and lean mass. This bone deficit may increase fracture risk and hinder peak bone mass development in these children.

Area of Science:

  • Pediatric Endocrinology
  • Hematology
  • Bone Metabolism

Background:

  • Children with sickle cell disease (SCD) often exhibit poor growth, altered body composition, and delayed maturation.
  • Bone mineral content (BMC) and bone area (BA) deficits in pediatric SCD are not well understood.

Purpose of the Study:

  • To assess whole-body BMC (WBBMC) and WBBA in children with SCD, type SS (SCD-SS), compared to healthy controls, adjusted for growth and body composition.
  • To determine the relationship between WBBMC, WBBA, bone age, and hematologic parameters in children with SCD-SS.

Main Methods:

  • Dual-energy x-ray absorptiometry (DXA) was used to measure WBBMC, WBBA, and lean mass in children aged 4–19 years.
  • Growth, sexual development, and bone age were assessed.
  • Gender-specific z scores for WBBMC relative to age and height were derived from control data.

Main Results:

  • Ninety children with SCD-SS and 198 controls were evaluated; SCD-SS was linked to poor growth.
  • WBBMC was significantly lower in SCD-SS compared to controls, even after adjusting for age, height, pubertal status, and lean mass.
  • WBBMC z scores were associated with hemoglobin, hematocrit levels, and a history of delayed bone age.

Conclusions:

  • Children with SCD-SS demonstrate significant WBBMC deficits that persist despite adjustments for growth and lean mass.
  • These findings suggest children with SCD-SS may face an elevated risk of fragility fractures.
  • There is a potential for suboptimal peak bone mass attainment in pediatric SCD-SS.
Abstract

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