Pattern of Bone Mineral Density in Sickle Cell Disease Patients with the High-Hb F Phenotype

Gupta, Renu; Marouf, Rajaa; Adekile, Adekunle

doi:10.1159/000262319

Cited by 14 publications

(12 citation statements)

References 39 publications

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“…These results are accompanied by a significant reduction in PTH and serum Ca concentrations and increased concentration of bone turnover parameters (OC, b-ALP and CTX). These results are in accordance with those of previous literature [1, 23], and suggest a relation between low BMD and increased bone turnover, as previously reported [24]. …”

Section: Discussionsupporting

confidence: 94%

“…Sickle-cell disease (SCD) is an autosomal recessive genetic disease with an increase in the adhesion of sickled erythrocytes, and it is a potential cause of vaso-occlusive episodes, which are related to clinical manifestations, morbidity and mortality [1, 2]. The SCA often coincides with osteoporosis or osteopenia in children and young adults [3].…”

Section: Introductionmentioning

confidence: 99%

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The association of bone mineral density and parathyroid hormone with serum magnesium in adult patients with sickle-cell anaemia

Elshal¹,

Bernawi²,

Al-Ghamdy³

et al. 2012

aoms

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IntroductionBone disorders including osteopenia and osteoporosis are a frequent cause of morbidity in sickle-cell disease (SCD). Magnesium (Mg) regulates some biological processes important in bone remodelling. We aimed to investigate whether serum Mg levels (sMg) may have an impact on bone mineral density (BMD) in sickle-cell anaemia (SCA).Material and methodsSixty adults with SCA in steady-state and 20 age- and race-matched healthy blood donors were included in the study. The BMD was evaluated with respect to minerals and biochemical indices of bone metabolism. Multivariate analysis was performed to determine the factors influencing BMD.ResultsThe mean sMg concentration was 0.64 ±0.06 (reference range 0.7-1.2 mmol/l) for 34% of the population, and 0.86 ±0.08 mmol/l for 66%. There were significant differences between Mg groups and controls in BMD, phosphorus (PO4), parathyroid hormone (PTH) (p = 0.011, p = 0.011 and p = 0.0001 respectively) and osteocalcin (OC) (p = 0.030) levels. The sMg was found to be associated positively with serum calcium (Ca), PTH and OC (r = 0.585; r = 0.436; r = 0.351 respectively, all at p < 0.05), and negatively with PO4 (r = –0.312; p < 0.05). Multivariate analysis demonstrated that only PTH (p < 0.05) was an independent factor for BMD. Moreover, it identified sMg, OC, and CTX as independent factors for PTH (all p < 0.05).ConclusionsThese results indicate that serum Mg may be a co-contributing factor in causing low BMD. However, other possible aetiologies including decreased PTH and increased bone turnover certainly play a role. Based on the present data, it is prudent to monitor sMg routinely in this patient population and treat the condition whenever possible.

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Section: Discussionsupporting

confidence: 94%

Section: Introductionmentioning

confidence: 99%

The association of bone mineral density and parathyroid hormone with serum magnesium in adult patients with sickle-cell anaemia

Elshal¹,

Bernawi²,

Al-Ghamdy³

et al. 2012

aoms

View full text Add to dashboard Cite

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“…The infarction of vertebral bone marrow and the presence of osteoporosis may produce collapse of the vertebrae with the typical fish mouth appearance. Several studies have shown an overall reduction in BMD in children and adults with SCD (9,(12)(13)(14). In recent studies, according to WHO criteria, 70-80% of SCD patients were osteoporotic or osteopenic (15)(16)(17).…”

Section: Sickle Cell Disease and The Bonesmentioning

confidence: 99%

Bone Disease in Haemoglobin Disorders

Voskaridou

Terpos

2013

Thalassemia Reports

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Bone disease represents a prominent cause of morbidity in patients with thalassaemia and other haemoglobin disorders. The delay in sexual maturation, the presence of diabetes and hypothyroidism, the parathyroid gland dysfunction, the haemolytic anaemia, the progressive marrow expansion, the iron toxicity on osteoblasts, the iron chelators, and the deficiency of growth hormone or insulin growth factors have been identified as major causes of osteoporosis in thalassaemia. Adequate hormonal replacement, effective iron chelation, improvement of hemoglobin levels, calcium and vitamin D administration, physical activity, and smoking cessation are the main to-date measures for the management of the disease. During the last decade, novel pathogenetic data suggest that the reduced osteoblastic activity, which is believed to be the basic mechanism of bone loss in thalassemia, is accompanied by a comparable or even greater increase in bone resorption. Therefore, potent inhibitors of osteoclast activation, such as the aminobisphosphonates, arise as key drugs for the management of osteoporosis in thalassaemia patients and other haemoglobin disorders.

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“…However, the unreliable interpretations of DXA results have led to numerous misdiagnoses due to overestimating and underestimating BMD levels in general [Gafni and Baron, 2004; Wren et al, 2005]. Additionally, variations in the BMD of sickle patients are based on age, gender, and ethnicity, further minimizing the usefulness of BMD to monitor sickle bone by itself [Almedia and Roberts, 2005; Gupta et al, 2009; Lal et al, 2006; Sarrai et al, 2007]. …”

Section: Introductionmentioning

confidence: 99%

Microarchitectural and mechanical characterization of the sickle bone

Green

Akinsami

Lin

et al. 2015

Journal of the Mechanical Behavior of Biomedical Materials

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Individuals with sickle cell disease often experience acute and chronic bone pain due to occlusive events within the tissue vasculature that result in ischemia, necrosis, and organ degeneration. Macroscopically, sickle bone is identified in clinical radiographs by its reduced mineral density, widening of the marrow cavity, and thinning of the cortical bone due to the elevated erythroid hyperplasia accompanying the disease. However, the microstructural architecture of sickle bone and its role in mechanical functionality is largely unknown. This study utilized micro-CT and biomechanical testing to determine the relationship between the bone morphology, tissue mineral density, and trabecular and cortical microarchitecture of 10-and 21-week-old femurs from transgenic sickle male mice and littermates with sickle trait, as well as a wild-type control. While bone tissue mineral density did not vary among the genotypes at either age, variation in bone microstructure were observed. At 10 weeks, healthy and trait mice exhibited similar morphology within the cortical and trabecular bone, while sickle mice exhibited highly connected trabeculae. Within older femurs, sickle and trait specimens displayed significantly fewer trabeculae, and the remaining trabeculae had a more deteriorated geometry based on the structure model index. Thinning of the cortical region in sickle femurs contributed to the displayed flexibility with a significantly lower elastic modulus than the controls at both 10- and 21-weeks old. Wild-type and trait femurs generally demonstrated similar mechanical properties; however, trait femurs had a significantly higher modulus than sickle and wild-type control at 21-weeks. Overall, these data indicate that the progressive damage to the microvasculature caused by sickle cell disease, results in deleterious structural changes in the bone tissue's microarchitecture and mechanics.

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Pattern of Bone Mineral Density in Sickle Cell Disease Patients with the High-Hb F Phenotype

Cited by 14 publications

References 39 publications

The association of bone mineral density and parathyroid hormone with serum magnesium in adult patients with sickle-cell anaemia

The association of bone mineral density and parathyroid hormone with serum magnesium in adult patients with sickle-cell anaemia

Bone Disease in Haemoglobin Disorders

Microarchitectural and mechanical characterization of the sickle bone

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