Baldan Alessandro, Giusti Andrea, Bosi Cristina, Malaventura Cristina, Musso Marco, Forni Gian Luca, Volpato Stefano, Zuliani Giovanni, Borgna-Pignatti Caterina
Department of Medical Sciences, Section of Pediatrics, University of Ferrara, Italy; Department of Life and Reproduction Sciences, Section of Biology and Genetics, University of Verona, Verona, Italy.
Bone Clinic, Department of Gerontology and Musculoskeletal Sciences, Galliera Hospital, Genoa, Italy.
Blood Cells Mol Dis. 2015 Dec;55(4):396-401. doi: 10.1016/j.bcmd.2015.08.004. Epub 2015 Aug 24.
Aim of this study was to compare plasma levels of the secreted protein Klotho in β-thalassemia major patients and in healthy controls. Also, we examined the existence of correlations between the protein level and osteoporosis, poor muscle strength and fractures. A total of 106 patients with β-thalassemia major and 95 healthy blood donors were enrolled. Klotho level in plasma was measured by mean of an ELISA test and the hand-grip strength using a dynamometer. Intact parathyroid hormone (PTH), 25-hydroxy vitamin D (Vitamin D), serum calcium (Ca), phosphate (P), total alkaline phosphatase (ALP), ferritin, creatinine were measured by standard clinical techniques. DXA was used to measure bone mineral density (BMD) at the lumbar spine (L2-L4), femoral neck and total hip. We found that the Klotho protein concentration was lower in the blood of patients with β-thalassemia major than in healthy controls, and it was directly correlated to the hand-grip strength. In β-thalassemia major patients, the secreted Klotho was lower than in healthy controls. The preliminary investigation into the correlation between markers of osteo- and sarcopenia and Klotho demonstrated a decreased Klotho concentration in β-TM patients and a higher probability of having had fragility fractures.
本研究的目的是比较重型β地中海贫血患者与健康对照者血浆中分泌蛋白α-klotho的水平。此外，我们还研究了该蛋白水平与骨质疏松症、肌肉力量差和骨折之间的相关性。总共招募了106例重型β地中海贫血患者和95名健康献血者。采用酶联免疫吸附测定（ELISA）法测定血浆中α-klotho水平，并用握力计测量握力。采用标准临床技术测定血清完整甲状旁腺激素（PTH）、25-羟基维生素D（维生素D）、血清钙（Ca）、磷（P）、总碱性磷酸酶（ALP）、铁蛋白、肌酐。采用双能X线吸收法（DXA）测量腰椎（L2-L4）、股骨颈和全髋部的骨密度（BMD）。我们发现，重型β地中海贫血患者血液中的α-klotho蛋白浓度低于健康对照者，且与握力直接相关。在重型β地中海贫血患者中，分泌的α-klotho低于健康对照者。对骨量减少和肌肉减少症标志物与α-klotho之间相关性的初步研究表明，重型β地中海贫血患者的α-klotho浓度降低，且发生脆性骨折的可能性更高。
