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

Buison, Anne; Kawchak, Deborah A.; Schall, Joan I.; Ohene‐Frempong, Kwaku; Stallings, Virginia A.; Leonard, Mary B.; Zemel, Babette S.

doi:10.1542/peds.2004-2582

Cited by 64 publications

(65 citation statements)

References 34 publications

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“…Children with homozygous type-SS SCA were recruited from The Children's Hospital of Philadelphia to participate in a longitudinal study of nutrition and growth as previously described [3]. Height and weight were measured, and pubertal development (Tanner stage) determined by a validated self-assessment question-naire [11] at two annual visits (years 4 and 5).…”

Section: Methodsmentioning

confidence: 99%

“…Height and weight were measured, and pubertal development (Tanner stage) determined by a validated self-assessment question-naire [11] at two annual visits (years 4 and 5). Whole body BMD, bone area (BA), and bone mineral content (BMC) were also determined twice by dual energy X-ray absorptiometry (DXA: Hologic QDR 2000, Bedford, MA) [3] in a subset of subjects (n = 46). There were no differences in age, gender, or growth between those who had DXA measured at one versus both time points.…”

Section: Methodsmentioning

confidence: 99%

“…Reference values were obtained from the literature which utilized similar analytical techniques [12][13][14]. Bone mineral and growth Z-scores were calculated as previously described [3]. Data were analyzed using standardized techniques (STATA version 9.0, Stata Corp, College Station, TX).…”

Section: Methodsmentioning

confidence: 99%

“…Children with sickle cell anemia (SCA) have low bone mass compared to healthy children [1][2][3]. Low bone mass in these children is apparent even after adjusting for age, height, pubertal development, and lean body mass, suggesting that the deficits cannot be fully explained by short stature, delayed puberty, or altered body composition [3].…”

Section: Introductionmentioning

confidence: 99%

“…Low bone mass in these children is apparent even after adjusting for age, height, pubertal development, and lean body mass, suggesting that the deficits cannot be fully explained by short stature, delayed puberty, or altered body composition [3]. Chronic hemolytic anemia and the resulting erythroblastic hyperplasia may contribute to bone demineralization in SCA [4,5].…”

Section: Introductionmentioning

confidence: 99%

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Markers of bone turnover are associated with growth and development in young subjects with sickle cell anemia

Fung¹,

Kawchak

Zemel

et al. 2008

Pediatric Blood & Cancer

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Children with sickle cell anemia (SCA) have low bone mass though bone turnover has not been well described. In this study, growth and pubertal development were assessed twice, 1 year apart, in 80 young subjects with type-SS SCA, while whole body bone mineral content (BMC) and density where measured in a subset (n = 46). Markers of bone turnover were measured at the second visit. Bone formation (alkaline phosphatase) was elevated, whereas bone resorption (deoxypryidinoline) was decreased compared to published data in healthy children. Markers of bone turnover correlated with growth velocity and pubertal development but not with changes in bone mass.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Markers of bone turnover are associated with growth and development in young subjects with sickle cell anemia

Fung¹,

Kawchak

Zemel

et al. 2008

Pediatric Blood & Cancer

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Bone Health in Patients With Hematopoietic Disorders of Bone Marrow Origin: Systematic Review and Meta- Analysis

Steer

Stavnichuk

Morris

et al. 2016

Journal of Bone and Mineral Research

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Blood cell production and bone homeostasis are physically interlinked systems that exhibit active cross-talk. We examined how bone health is affected in patients with hematopoietic disorders due to abnormal proliferation of bone marrow cells. The electronic databases Medline, Embase, PubMed, BIOSIS Previews, Web of Science, and Cochrane were searched for studies presenting numerical values for trabecular bone volume or bone mineral density in control and patients with hematopoietic disorders. We identified 5 studies for beta-thalassemia, 6 for sickle cell anemia, 2 for polycythemia vera and essential thrombocythemia, 3 for chronic myelogenous leukemia, 6 for myelofibrosis, 5 for multiple myeloma, and 4 studies each for systemic mastocytosis, lymphocytic leukemia, and hemochromatosis. The effect of the disease state on bone density was significant and negative for beta-thalassemia (r = -2.00; 95% confidence interval [CI] -3.41, -0.58; p < 0.005), sickle cell anemia (-0.91; -1.36, -0.47; p < 0.00005), chronic myelogenous leukemia (-0.55; -0.88, -0.22; p < 0005), mastocytosis (-0.99; -1.16, -0.82; p < 0.00001), lymphoblastic leukemia (-0.69; -0.98, -0.40; p < 0.00001), multiple myeloma (-0.67; -0.99, -0.35; p < 0.00005), and hemochromatosis (-1.15; -1.64, -0.66; p < 0.00001). The changes were negative but not significant for polycythemia vera (-0.16; -0.38, 0.05; p = 0.069) and essential thrombocythemia (-0.33; -0.92, 0.26; p = 0.14). In myelofibrosis, disease state was associated with increased bone density (0.74; 0.12, 1.36; p < 0.05). Bone density change significantly and negatively correlated with the level of ferritin and bone marrow cellularity but not with hemoglobin or erythropoietin. Thus, independent of hematopoietic lineage, abnormal proliferation of bone marrow cells appears to be associated with bone loss. Iron metabolism may independently contribute to bone homeostasis. © 2016 American Society for Bone and Mineral Research.

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Prevalence of abnormal bone density of pediatric patients prior to blood or marrow transplant

Klopfenstein

Clayton

Rosselet

et al. 2009

Pediatric Blood & Cancer

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Osteoporosis and osteopenia are long-term side effects of bone marrow transplant (BMT). The purpose of this study was to determine the prevalence of bone mineral density (BMD) abnormalities in pediatric patients prior to BMT. Forty-four pediatric patients were evaluated with DEXA scans. The average Z-score was -0.37. Thirty-six percent had abnormal BMD. Sixty-seven percent of ALL patients had abnormal BMD. Patients with non-malignant diseases were significantly more likely to have abnormal BMD. Patients with ALL had more defects than solid tumor patients. Females had more defects than males. These results demonstrate BMD defects are common in children prior to BMT, especially in patients with ALL.

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

Abstract: 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.

Cited by 64 publications

References 34 publications

Markers of bone turnover are associated with growth and development in young subjects with sickle cell anemia

Markers of bone turnover are associated with growth and development in young subjects with sickle cell anemia

Bone Health in Patients With Hematopoietic Disorders of Bone Marrow Origin: Systematic Review and Meta- Analysis

Prevalence of abnormal bone density of pediatric patients prior to blood or marrow transplant

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