Bone mineral density in children with sickle cell anemia

Lal, Ashutosh; Fung, Ellen B.; Pakbaz, Zahra; Hackney-Stephens, Ekua; Vichinsky, Elliott

doi:10.1002/pbc.20681

Cited by 83 publications

(106 citation statements)

References 26 publications

(33 reference statements)

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“…Some [17,18], but not all [1,16] reported elevations in markers of bone formation (BSAP, C-terminal propeptide of type I collagen) in SCA compared to black controls. Reports of markers of bone resorption in pediatric subjects with SCA are equally divided: one reported increased urinary pyridinium [17] and the other reported decreased serum N-terminal telopeptide of type 1 collagen in serum compared to controls [16].…”

Section: Discussionmentioning

confidence: 92%

“…Few studies have assessed bone turnover in patients with SCA and explored its relation to DXA [1], ultrasound [16], or protein metabolism [17]. Some [17,18], but not all [1,16] reported elevations in markers of bone formation (BSAP, C-terminal propeptide of type I collagen) in SCA compared to black controls.…”

Section: Discussionmentioning

confidence: 99%

“…Reports of markers of bone resorption in pediatric subjects with SCA are equally divided: one reported increased urinary pyridinium [17] and the other reported decreased serum N-terminal telopeptide of type 1 collagen in serum compared to controls [16]. The only report in SCA relating DXA bone densitometry to bone markers found no significant relationships [1].…”

Section: Discussionmentioning

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%

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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: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 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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“…25 However, current information is inconclusive for a possible correlation between bone mineral density and iron stores as reflected by serum ferritin concentration in patients with sickle cell disease. 20,[26][27][28][29] In our study of sickle cell disease patients, BUN, interleukin-8 and interleukin-6 concentrations were associated with higher osteoclast activity as reflected by TRACP 5b concentration while PDGF and CCL5 were associated with lower osteoclast activity. Higher osteoclast activity, as reflected by serum TRACP 5b concentration, was also associated with higher tricuspid regurgitation velocity.…”

Section: Beta (95% Ci) P Standardized Betamentioning

confidence: 96%

Predictors of osteoclast activity in patients with sickle cell disease

Nouraie¹,

Cheng²,

Niu³

et al. 2011

Haematologica

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BackgroundBone changes are common in sickle cell disease, but the pathogenesis is not fully understood. Tartrate-resistant acid phosphatase (TRACP) type 5b is produced by bone-resorbing osteoclasts. In other forms of hemolytic anemia, increased iron stores are associated with osteoporosis. We hypothesized that transfusional iron overload would be associated with increased osteoclast activity in patients with sickle cell disease. Design and MethodsWe examined tartrate-resistant acid phosphatase 5b concentrations in patients with sickle cell disease and normal controls of similar age and sex distribution at steady state. Serum tartrateresistant acid phosphatase 5b concentration was measured using an immunocapture enzyme assay and plasma concentrations of other cytokines were assayed using the Bio-Plex suspension array system. Tricuspid regurgitation velocity, an indirect measure of systolic pulmonary artery pressure, was determined by echocardiography. ResultsTartrate-resistant acid phosphatase 5b concentrations were higher in 58 adults with sickle cell disease than in 22 controls (medians of 4.4 versus 2.4 U/L, respectively; P=0.0001). Among the patients with sickle cell disease, tartrate-resistant acid phosphatase 5b independently correlated with blood urea nitrogen (standardized beta=0.40, P=0.003), interleukin-8 (standardized beta=0.30, P=0.020), and chemokine C-C motif ligand 5 (standardized beta=-0.28, P=0.031) concentrations, but not with serum ferritin concentration. Frequent blood transfusions (>10 units in life time) were not associated with higher tartrate-resistant acid phosphatase 5b levels in multivariate analysis. There were strong correlations among tartrate-resistant acid phosphatase 5b, alkaline phosphatase and tricuspid regurgitation velocity (r>0.35, P<0.001). ConclusionsPatients with sickle cell disease have increased osteoclast activity as reflected by serum tartrateresistant acid phosphatase 5b concentrations. Our results may support a potential role of inflammation rather than increased iron stores in stimulating osteoclast activity in sickle cell disease. The positive relationships among tartrate-resistant acid phosphatase 5b, alkaline phosphatase and tricuspid regurgitation velocity raise the possibility of a common pathway in the pulmonary and bone complications of sickle cell disease.Key words: sickle cell disease, osteoclast activity, bone turnover, inflammation, TRACP 5b, pulmonary complications.Citation: Nouraie M, Cheng K, Niu X, Moore-King E, Fadojutimi-Akinsi MF, Minniti CP, Sable C, Rana S, Dham N, Campbell A, Ensing G, Kato GJ, Gladwin MT, Castro OL, and Gordeuk VR. Predictors of osteoclast activity in sickle cell disease patients. Haematologica 2011;96(8):1092-1098. doi:10.3324/haematol.2011 This is an open-access paper. Predictors of osteoclast activity in patients with sickle cell disease

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Sickle cell bone disease: Response to vitamin D and calcium

Adewoye

Chen

et al. 2007

American J Hematol

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Bone disease with osteoporosis and osteomalacia are common in sickle cell disease (SCD). Some patients have vitamin D deficiency and low bone mineral density (BMD). The role of vitamin D and calcium supplementation to restore bone health in SCD has not been well studied. In 14 adults with SCD, we measured 25(OH)D (25-hydroxyvitamin D) and BMD at the femoral neck, lumbar spine, and distal third of the ulna plus radius, along with markers of bone resorption (CTx; C-terminal component of pro-collagen type I) and bone formation (osteocalcin) before and after 12 months of vitamin D 2 and calcium carbonate treatment. Pretreatment, all patients were vitamin D deficient with a mean 25(OH)D level of 10.7 ± 4.7 ng/ml, had low BMD at the lumbar spine (L-spine), 0.87 ± 0.11 g/cm 2 (mean Z-score of -2.6 3 ± 0.71 SD and T score of -2.31 ± 0.75 SD), femoral neck, 0.8 ± 0.18 g/cm 2 (mean Z-score -1.36 ± 0.84, T-score -1.14 ± 0.75), and the distal radius and ulna, 0.6 ± 0.17 g/cm 2 (mean Z-score -1.18 ± 0.79, T-score -1.01 ± 0.74) and had elevated CTx (0.87 ± 0.5 ng/ml) and osteocalcin levels (12.3 ± 3.7 ng/ll). After treatment, all patients corrected their 25(OH)D level (38.8 ± 13.9 ng/ml) (P < 0.001) with a 3.6% ± 3.9% increase in BMD at the L-spine (P 5 0.009), 4.6% ± 8.5% increase at the femoral neck (P 5 0.05) and 6.5% ± 12.6% increase at the distal radius plus ulna (P 5 0.09). CTx, osteocalcin, and PTH(i) levels were unchanged. Treatment of adult SCD with vitamin D and calcium can restore 25(OH)D levels to normal and improve BMD, but, markers of bone resorption remained unchanged. Screening for vitamin D deficiency and BMD in SCD patients seems warranted. Am. J. Hematol. 83:271-274, 2008. V

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Bone mineral density in children with sickle cell anemia

Abstract: Our results suggest that children with severe manifestations of SCA have low BMD, and possess significant deficits in dietary calcium and circulating vitamin D.

Cited by 83 publications

References 26 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

Predictors of osteoclast activity in patients with sickle cell disease

Sickle cell bone disease: Response to vitamin D and calcium

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