Published online September 30, 2005
PEDIATRICS Vol. 116 No. 4 October 2005, pp. 943-949 (doi:10.1542/peds.2004-2582)
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via ISI Web of Science (4)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Buison, A. M.
Right arrow Articles by Zemel, B. S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Buison, A. M.
Right arrow Articles by Zemel, B. S.
Related Collections
Right arrow Blood

Bone Area and Bone Mineral Content Deficits in Children With Sickle Cell Disease

Anne M. Buison, PhD*, Deborah A. Kawchak, MS, RD*, Joan I. Schall, PhD*, Kwaku Ohene-Frempong, MD{ddagger}, Virginia A. Stallings, MD*, Mary B. Leonard, MD, MSCE§ and Babette S. Zemel, PhD*

* Gastroenterology and Nutrition
{ddagger} Divisions of Hematology
§ Divisions of Nephrology, Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania

Objective. Children with sickle cell disease (SCD) experience poor growth, altered body composition, and delayed maturation. Deficits in bone mineral content (BMC) and bone area (BA) have not been well characterized. The objectives of this study were to assess whole-body BMC (WBBMC) and WBBA in children with SCD, type SS (SCD-SS), compared with healthy control subjects, adjusted for growth and body composition, and to determine the relationships of WBBMC and WBBA to bone age and hematologic parameters in children with SCD-SS.

Methods. WBBMC, WBBA, and lean mass were measured by dual-energy x-ray absorptiometry in children who were aged 4 to 19 years. Growth, sexual development, and bone age were assessed. Gender-specific z scores for WBBMC relative to age and height were generated from control data.

Results. Ninety children with SCD-SS and 198 healthy control subjects were evaluated. SCD-SS was associated with poor growth. WBBMC was significantly decreased in SCD-SS compared with control subjects, adjusted for age, height, pubertal status, and lean mass. WBBMC relative to age and WBBMC relative to height z scores were –0.95 ± 0.99 and –0.54 ± 0.97, respectively, and were associated with hemoglobin and hematocrit levels and history of delayed bone age.

Conclusions. Children with SCD-SS have significant deficits in WBBMC that persist despite adjustment for poor growth and decreased lean mass. These children may be at increased risk for fragility fractures and suboptimal peak bone mass.

Key Words: bone health • growth and nutrition • sickle cell disease

Abbreviations: SCD, sickle cell disease • SCD-SS, sickle cell disease type SS • BMD, bone mineral density • DXA, dual-energy x-ray absorptiometry • WBBMC, whole-body bone mineral content • WBBA, whole-body bone area • CHOP, Children's Hospital of Philadelphia • WBBMC-age-z, WBBMC z score relative to age • WBBA-age-z, WBBA z score relative to age • WBBMC-ht-z, WBBMC z score relative to height • WBBA-ht-z, WBBA z score relative to height

Accepted May 5, 2005.