De Franceschi Lucia, Gabbiani Daniele, Giusti Andrea, Forni Gianluca, Stefanoni Filippo, Pinto Valeria Maria, Sartori Giulia, Balocco Manuela, Dal Zotto Chiara, Valenti Maria Teresa, Dalle Carbonare Luca
Section of Internal Medicine, Department of Medicine, University of Verona, 37134 Verona, Italy.
Rheumatology Unit, Department of Locomotor System, La Colletta Hospital, 16011 Arenzano, Italy.
J Clin Med. 2020 May 25;9(5):1601. doi: 10.3390/jcm9051601.
Sickle-cell disease (SCD) is a worldwide distributed hemoglobinopathy, characterized by hemolytic anemia associated with vaso-occlusive events. These result in acute and chronic multiorgan damage. Bone is early involved, leading to long-term disability, chronic pain and fractures. Here, we carried out a retrospective study to evaluate sickle bone disease (SBD) in a cohort of adults with SCD. We assessed bone density, metabolism and turnover. We also evaluated the presence of fractures and the correlation between SCD severity and skeletal manifestations. A total of 71 patients with SCD were analyzed. The mean age of population was 39 ± 10 years, 56% of which were females. We found osteoporosis in a range between 7% and 18% with a high incidence of vertebral fractures. LDH and AST were predictive for the severity of vertebral fractures, while bone density was not. Noteworthy, we identified -1.4 Standard Deviations -score as the cutoff for detecting the presence of fractures in patients with SCD. Collectively our data allowed us to develop an algorithm for the management of SBD, which may be useful in daily clinical practice to early intersect and treat SBD.
镰状细胞病（SCD）是一种全球分布的血红蛋白病，其特征是与血管闭塞事件相关的溶血性贫血。这些会导致急性和慢性多器官损伤。骨骼早期受累，导致长期残疾、慢性疼痛和骨折。在此，我们进行了一项回顾性研究，以评估一组成年SCD患者的镰状骨病（SBD）。我们评估了骨密度、代谢和转换。我们还评估了骨折的存在情况以及SCD严重程度与骨骼表现之间的相关性。共分析了71例SCD患者。人群的平均年龄为39±10岁，其中56%为女性。我们发现骨质疏松症的发生率在7%至18%之间，椎体骨折的发生率很高。乳酸脱氢酶（LDH）和天冬氨酸转氨酶（AST）可预测椎体骨折的严重程度，而骨密度则不能。值得注意的是，我们将-1.4标准差评分确定为检测SCD患者骨折存在情况的临界值。总体而言，我们的数据使我们能够制定一种SBD管理算法，这在日常临床实践中可能有助于早期干预和治疗SBD。
