Internal Medicine Department, Sickle Cell Referral Center, Georges Pompidou European Hospital, AP-HP, Paris, France; Faculté de Médecine Paris Descartes, Sorbonne Paris-Cité, Paris, France.
Internal Medicine Department, Sickle Cell Referral Center, Georges Pompidou European Hospital, AP-HP, Paris, France; Faculté de Médecine Paris Descartes, Sorbonne Paris-Cité, Paris, France; Laboratory of Excellence GR-Ex, Paris, France.
Bone. 2018 May;110:199-203. doi: 10.1016/j.bone.2018.02.003. Epub 2018 Feb 8.
Osteosclerosis (OSC) is a rarely studied complication of sickle cell disease (SCD). The objective of our study was to determine the prevalence and characteristics of high bone mineral density (BMD) and its radiological features in adult SCD patients.
This prospective observational study was conducted from May 2007 to May 2016 in consecutive patients with steady-state SCD at two university hospitals. The BMD of the lumbar spine (L1-L4) and right femoral neck was determined by dual energy X-ray absorptiometry. Clinical, laboratory and radiographic data were recorded. High BMD was defined as a BMD Z-score of at least +2.5 standard deviations at the lumbar spine or hip. The characteristics of the patients with high BMD were compared to those of individuals with low or middle BMD, using multivariate ordinal logistic regression.
135 patients (86 women and 49 men) with a median age of 27 (IQR 23-33) years were included. High BMD was diagnosed in 20 (15%) patients with a median age of 33.5 (IQR 28-45) years. The SCD genotypes of these patients were SS in 11, SC in 5, S/beta+ in 3, and S/beta0 in 1. High BMD patients more frequently harbored the S/beta SCD genotype (21% vs 5% in non-high BMD patients; p=0.047) and were older (p=0.0007). Compared to patients with low or middle BMD, after adjustment for age and SCD genotype, high BMD patients had a higher prevalence of avascular necrosis history (p=0.009), higher BMI (p=0.007), and lower serum resorption marker CTX (p=0.04), bilirubin (p=0.02) and parathyroid hormone levels (p=0.02). There were no differences between groups regarding fracture history, H-shaped vertebrae or other biological variables.
High-BMD values is a common manifestation in SCD patients, especially in those with the S/beta-thalassemia genotypes. The prevalence of high-BMD in SCD is associated with older age, suggesting that it will be more common in the future because the life span of patients with SCD is increasing thanks to significant progress in SCD treatment.
骨硬化症（OSC）是镰状细胞病（SCD）少见的并发症。本研究的目的是确定成人 SCD 患者中高骨密度（BMD）及其影像学特征的患病率和特点。
这是一项在两所大学医院进行的连续 SCD 稳定期患者的前瞻性观察性研究。通过双能 X 射线吸收法测定腰椎（L1-L4）和右侧股骨颈的 BMD。记录临床、实验室和影像学数据。高 BMD 定义为腰椎或髋部 BMD Z 评分至少为+2.5 个标准差。采用多变量有序逻辑回归比较高 BMD 患者与低或中 BMD 患者的特征。
共纳入 135 名患者（86 名女性和 49 名男性），中位年龄为 27 岁（IQR 23-33）。诊断为高 BMD 的 20 名患者的中位年龄为 33.5 岁（IQR 28-45）。这些患者的 SCD 基因型为 SS11、SC5、S/beta+3、S/beta01。高 BMD 患者更常携带 S/beta SCD 基因型（21% vs 非高 BMD 患者的 5%；p=0.047）且年龄更大（p=0.0007）。在调整年龄和 SCD 基因型后，与低或中 BMD 患者相比，高 BMD 患者有更高的血管坏死病史（p=0.009）、更高的 BMI（p=0.007）、更低的血清吸收标志物 CTX（p=0.04）、胆红素（p=0.02）和甲状旁腺激素水平（p=0.02）。两组间骨折史、H 形椎骨或其他生物学变量无差异。
高 BMD 值是 SCD 患者的常见表现，尤其是 S/beta 地中海贫血基因型患者。SCD 患者的高 BMD 患病率与年龄较大有关，这表明由于 SCD 治疗的显著进展，患者的寿命延长，因此未来这种情况将更为常见。
