Evaluation of bone mineral density in children with sickle-cell anemia and its associated factors in the south of Iran: a case-control study

Bordbar, Mohammadreza; Haghpanah, Sezaneh; Zarei, Tahereh; Dabbaghmanesh, Mohammad Hossein; Omrani, Gholamhossein Ranjbar; Saki, Fatemeh

doi:10.1007/s11657-017-0364-x

Cited by 10 publications

(8 citation statements)

References 37 publications

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“…In our study, low BMD prevalence increased from early childhood to adolescence, as previously reported. 10,24,35,36 The association between adolescent age category and low BMD likely reflects the length of time required for bone deficits to arise from the cumulative vascular insults of SCD. In the univariate logistic regression, any hydroxyurea exposure or hydroxyurea exposure for 5 to 10 years significantly associated with low BMD (supplemental Table 4).…”

Section: Low Bmd Correlates With Older Age Hemolysis and Chronic Painmentioning

confidence: 99%

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Height-corrected low bone density associates with severe outcomes in sickle cell disease: SCCRIP cohort study results

et al. 2019

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Low bone mineral density (BMD) disproportionately affects people with sickle cell disease (SCD). Growth faltering is common in SCD, but most BMD studies in pediatric SCD cohorts fail to adjust for short stature. We examined low BMD prevalence in 6- to 18-year-olds enrolled in the Sickle Cell Clinical Research and Intervention Program (SCCRIP), an ongoing multicenter life span SCD cohort study initiated in 2014. We calculated areal BMD for chronological age and height-adjusted areal BMD (Ht-aBMD) z scores for the SCCRIP cohort, using reference data from healthy African American children and adolescents enrolled in the Bone Mineral Density in Childhood Study. We defined low BMD as Ht-aBMD z scores less than or equal to –2 and evaluated its associations with demographic and clinical characteristics by using logistic regression analyses. Of the 306 children and adolescents in our study cohort (mean age, 12.5 years; 50% female; 64% HbSS/Sβ0-thalassemia genotype; 99% African American), 31% had low areal BMD for chronological age z scores and 18% had low Ht-aBMD z scores. In multivariate analyses, low Ht-aBMD z scores associated with adolescence (odds ratio [OR], 7.7; 95% confidence interval [CI], 1.94-30.20), hip osteonecrosis (OR, 4.0; 95% CI, 1.02-15.63), chronic pain (OR, 10.4; 95% CI, 1.51-71.24), and hemoglobin (OR, 0.74; 95% CI, 0.57-0.96). Despite adjusting for height, nearly 20% of this pediatric SCD cohort still had very low BMD. As the SCCRIP cohort matures, we plan to prospectively evaluate the longitudinal relationship between Ht-aBMD z scores and markers of SCD severity and morbidity.

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Section: Low Bmd Correlates With Older Age Hemolysis and Chronic Painmentioning

confidence: 99%

“…Vitamin D deficiency in SCD has been well described [46][47][48][49][50][51][52][53][54][55] ; some studies have further investigated its association with low BMD. [56][57][58][59][60] Bordbar et al 35…”

Section: Sex Differences With Vitamin D Deficiency and Low Bmdmentioning

confidence: 99%

Height-corrected low bone density associates with severe outcomes in sickle cell disease: SCCRIP cohort study results

et al. 2019

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“…Indeed, vitamin D supplementation has already been demonstrated to be useful in the treatment of inflammation in other diseases, including acute congestive cardiac failure and autoimmune cytopenias (16,36). However, vitamin D supplementation is difficult in SCD patients compared with otherwise healthy, vitamin D-deficient individuals, and SCD patients generally need a higher dose to reach sufficiency (3,43).…”

Section: Discussionmentioning

confidence: 99%

Hemoglobin alters vitamin carrier uptake and vitamin D metabolism in proximal tubule cells: implications for sickle cell disease

Gliozzi

Rbaibi

Long

et al. 2019

American Journal of Physiology-Cell Physiology

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Kidney disease, including proximal tubule (PT) dysfunction, and vitamin D deficiency are among the most prevalent complications in sickle cell disease (SCD) patients. Although these two comorbidities have never been linked in SCD, the PT is the primary site for activation of vitamin D. Precursor 25-hydroxyvitamin D [25(OH)D] bound to vitamin D-binding protein (DBP) is taken up by PT cells via megalin/cubilin receptors, hydroxylated to the active 1,25-dihydroxyvitamin D [1,25(OH)2D] form, and released into the bloodstream. We tested the hypothesis that cell-free hemoglobin (Hb) filtered into the PT lumen impairs vitamin D uptake and metabolism. Hb at concentrations expected to be chronically present in the ultrafiltrate of SCD patients competed directly with DBP for apical uptake by PT cells. By contrast, uptake of retinol binding protein was impaired only at considerably higher Hb concentrations. Prolonged exposure to Hb led to increased oxidative stress in PT cells and to a selective increase in mRNA levels of the CYP27B1 hydroxylase, although protein levels were unchanged. Hb exposure also impaired vitamin D metabolism in PT cells, resulting in reduced ratio of 1,25(OH)2D:25(OH)D. Moreover, plasma levels of 1,25(OH)2D were reduced in a mouse model of SCD. Together, our data suggest that Hb released by chronic hemolysis has multiple effects on PT function that contribute to vitamin D deficiency in SCD patients.

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“…Full texts of 40 studies were screened, and 15 studies were subsequently excluded because of insufficient data to estimate the outcomes of interest (Figure 1). A total of 25 studies published from 1993 to 2019 that met the eligibility for data were used for qualitative and quantitative syntheses: 10 studies from the Americas [12,[28][29][30][31][32][33][34][35][36]; six studies from Asia [37][38][39][40][41][42]; five studies from Europe [43][44][45][46][47]; two studies from Africa [48,49], one study that used samples from both Jamaica and West Africa [50], and one study that used samples from both Brazil and Nigeria [51].…”

Section: Studies Includedmentioning

confidence: 99%

A Meta-analysis on Vitamin D Deficiency in Patients with Sickle Cell Disease

Mohamed¹,

MohamedElmugadam²,

Awadalla³

et al. 2020

DSAHMJ

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Vitamin D Deficiency (VDD) is reported to be more frequent with serious clinical outcomes in patients with Sickle Cell Disease (SCD). There is a wide disparity in data in the existing literature regarding the prevalence and risk of VDD in patients with SCD. These data require further summary and analyses for better accuracy. This review aimed to assess the association between VDD and SCD, and was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Medline/ PubMed, World Health Organization Virtual Health Library, ScienceDirect, and Google Scholar were used for the systematic search. A random effects model was used to estimate the pooled prevalence, Risk Ratio (RR), and Standardized Mean Difference (SMD) estimates with the corresponding 95% Confidence Interval (CI) using OpenMeta Analyst software version 10.10 (Tufts Medical Center, Boston, MA, USA). Twenty-five studies fulfilled the eligibility criteria. The prevalence of VDD among patients with SCD was 63.8% (95% CI, 52.5-75.1). The risk of VDD among patients with SCD was more than two times that of the general population (RR = 2.129; 95% CI, 1.024-4.423; p < 0.001). Serum vitamin D levels were significantly lower in SCD patients than in their controls (SMD = −1.883: 95% CI, −3.006 to −0.760; p < 0.001). This review provides a comprehensive view of the association between vitamin D status and SCD.

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Evaluation of bone mineral density in children with sickle-cell anemia and its associated factors in the south of Iran: a case-control study

Cited by 10 publications

References 37 publications

Height-corrected low bone density associates with severe outcomes in sickle cell disease: SCCRIP cohort study results

Height-corrected low bone density associates with severe outcomes in sickle cell disease: SCCRIP cohort study results

Hemoglobin alters vitamin carrier uptake and vitamin D metabolism in proximal tubule cells: implications for sickle cell disease

A Meta-analysis on Vitamin D Deficiency in Patients with Sickle Cell Disease

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