伊拉克al - najaf市Β-thalassemia患者血清硬化蛋白水平作为骨病相关生物标志物的评估

Sharba Intisar Razzaq, AL-DUJAILI Arshad Noori
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The information of beta-thalassemia patients was collected and records by the questioner. Results: A significantly increased serum sclerostin level (mean 26.80±0.91) pg/ml was showed in βT patients compared with the healthy controls (10.03±0.68, p  smaller than  0.001) pg/ml. Furthermore, a significant decrease (p smaller than 0.05) of the sclerostin level was observed in β-thalassemia major compared to intermedia β-thalassemia patients. Serum sclerostin level revealed a significant increase in progress age; it is highest in the age group (30-40) year as compared with age group (8-18) and (19-29) year respectively. Sclerostin showed no associations with the RBC, Hb, PCV, and significantly positively correlated (p smaller than 0.05) with serum iron, ferritin levels, WBC, and PLT count. Significantly higher sclerostin levels in splenectomized and underweight groups were observed compared to unsplenectomized and normal-weight groups (p smaller than 0.05) of βT patients. 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摘要

背景:β-地中海贫血是一种血液疾病,其中身体不能正常产生血红蛋白。目的:评价女性-地中海贫血患者血清硬化蛋白水平,并与健康对照进行比较,预测其与骨病理生理相关的并发症,为改善女性-地中海贫血患者的生活方式提供参考。材料与方法:对69例8 ~ 40岁依赖输血的女性β -地中海贫血(βT)患者(βT重度患者54例,βT中度患者15例)和20例健康对照者进行血清硬化蛋白测定,并检测其与血清学参数RBC、Hb、PCV、WBC、PLT、BMI、脾状态、铁和铁蛋白水平的关系。由提问者收集并记录β -地中海贫血患者的信息。结果:βT患者血清硬化蛋白水平(平均26.80±0.91)pg/ml明显高于健康对照组(10.03±0.68,p < 0.001) pg/ml。与中度β-地中海贫血患者相比,重度β-地中海贫血患者的硬化蛋白水平显著降低(p < 0.05)。血清硬化蛋白水平随着进展年龄的增加而显著升高;与8-18岁和19-29岁年龄组相比,30-40岁年龄组的发病率最高。硬化蛋白与红细胞、血红蛋白、PCV无相关性,与血清铁、铁蛋白水平、白细胞和血小板计数显著正相关(p < 0.05)。脾切除组和体重过轻组βT患者的硬化蛋白水平显著高于未脾切除组和体重正常组(p < 0.05)。结论:硬化蛋白在-地中海贫血患者中发挥着重要作用,可作为与骨病理生理相关的生物标志物和指标,预防这些患者因铁超载导致的严重疾病的继续发展。
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ASSESSMENT OF SERUM SCLEROSTIN LEVEL AS A BIOMARKER ASSOCIATED WITH BONE DISORDERS IN Β-THALASSEMIA PATIENTS IN AL- NAJAF CITY, IRAQ
Background: β-thalassemia is a blood disorder in which the body does not make hemoglobin normally. Aim: To assess serum sclerostin in female patients with beta-thalassemia and compare with the healthy controls and to predict its complication associated with the bone pathophysiology, for designed improvement the lifestyle goodliness for these patients. Material and methods: Sixty-nine female beta-thalassemia (βT) patients (54 βT major and 15 βT Intermedia), aged 8-40 years who dependent on transfused blood, and 20 healthy controls were evaluated serum sclerostin, and was examined the relationship with hematological parameters RBC, Hb, PCV, WBC, PLT, BMI, splenic status, iron, and ferritin levels. The information of beta-thalassemia patients was collected and records by the questioner. Results: A significantly increased serum sclerostin level (mean 26.80±0.91) pg/ml was showed in βT patients compared with the healthy controls (10.03±0.68, p  smaller than  0.001) pg/ml. Furthermore, a significant decrease (p smaller than 0.05) of the sclerostin level was observed in β-thalassemia major compared to intermedia β-thalassemia patients. Serum sclerostin level revealed a significant increase in progress age; it is highest in the age group (30-40) year as compared with age group (8-18) and (19-29) year respectively. Sclerostin showed no associations with the RBC, Hb, PCV, and significantly positively correlated (p smaller than 0.05) with serum iron, ferritin levels, WBC, and PLT count. Significantly higher sclerostin levels in splenectomized and underweight groups were observed compared to unsplenectomized and normal-weight groups (p smaller than 0.05) of βT patients. Conclusions: Sclerostin plays an important role in beta-thalassemia patients and can serve as a biomarker associated with the bone pathophysiology and indicator to prevent the continuation of such serious diseases caused by iron overload in these patients.
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