非缺失性血红蛋白H病青少年的骨矿物质密度和Dickkopf-1

IF 1.7 4区 医学 Q4 ENDOCRINOLOGY & METABOLISM
Pattara Wiromrat , Aree Rattanathongkom , Napat Laoaroon , Kunanya Suwannaying , Patcharee Komwilaisak , Ouyporn Panamonta , Nantaporn Wongsurawat , Nat Nasomyont
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引用次数: 0

摘要

背景:低骨密度(BMD)在β地中海贫血患者中普遍存在,并与循环dickkopf-1浓度增加有关。这些数据在α地中海贫血中是有限的。因此,我们旨在确定患有非缺失血红蛋白H疾病的青少年低BMD的患病率以及BMD与血清dickkopf-1之间的关系,非缺失血红蛋白H疾病是一种严重程度与中间型β地中海贫血相当的α-地中海贫血。方法:测量腰椎和全身骨密度,并将其转换为身高调整后的z评分。低BMD定义为BMD z评分≤-2。抽取参与者的血液以测量dickkopf-1和骨转换标志物的浓度。结果:37名患有非缺失性血红蛋白H疾病的参与者(59%为女性,平均年龄14.6±3.2岁,86%的Tanner分期≥2,95%定期输血,16%服用泼尼松)被纳入研究。在研究前一年,平均转化前血红蛋白、铁蛋白和25-羟基维生素D浓度分别为8.8±1.0 g/dL、958±513和26±6 ng/mL。当排除服用泼尼松的参与者时,腰椎和全身骨密度低的患病率分别为42%和17%。两个部位的BMD与体重指数z评分呈正相关,与dickkopf-1呈负相关(p值均<0.05)。Dickkopv-1、25-羟基维生素D、骨钙素和I型胶原C-末端肽之间没有相关性。多元回归分析显示,dickkopf-1与经性别、骨龄、体重指数、输血前血红蛋白、25-羟基维生素D、青春期延迟史、铁螯合剂类型和泼尼松龙使用调整后的全身BMD z评分呈负相关(p值 = 0.009)。结论:我们证明在患有非缺失血红蛋白H疾病的青少年中,低BMD的患病率很高。此外,dickkopf-1与全身BMD呈负相关,这表明它可能是该患者群体的骨生物标志物。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Bone Mineral Density and Dickkopf-1 in Adolescents with Non-Deletional Hemoglobin H Disease

Background: Low bone mineral density (BMD) is prevalent in individuals with β-thalassemia and is associated with increased circulating dickkopf-1 concentration. These data are limited in α-thalassemia. Therefore, we aimed to determine the prevalence of low BMD and the association between BMD and serum dickkopf-1 in adolescents with non-deletional hemoglobin H disease, a form of α-thalassemia whose severity is comparable to β-thalassemia intermedia. Methodology: The lumbar spine and total body BMD were measured and converted into height-adjusted z-scores. Low BMD was defined as BMD z-score ≤ -2. Participant blood was drawn for measurement of dickkopf-1 and bone turnover marker concentrations. Results: Thirty-seven participants with non-deletional hemoglobin H disease (59% female, mean age 14.6 ± 3.2 years, 86% Tanner stage ≥2, 95% regularly transfused, 16% taking prednisolone) were included. Over one year prior to the study, mean average pretransfusion hemoglobin, ferritin and 25-hydroxyvitamin D concentrations were 8.8 ± 1.0 g/dL, and 958 ± 513 and 26 ± 6 ng/mL, respectively. When participants taking prednisolone were excluded, the prevalence of low BMD at the lumbar spine and total body was 42% and 17%, respectively. BMD at both sites was correlated positively with body mass index z-score, and negatively with dickkopf-1 (all p-values <0.05). There were no correlations among dickkopf-1, 25-hydroxyvitamin D, osteocalcin and C-telopeptide of type-I collagen. Multiple regression analysis showed dickkopf-1 inversely associated with total body BMD z-score adjusting for sex, bone age, body mass index, pre-transfusion hemoglobin, 25-hydroxyvitamin D, history of delayed puberty, type of iron chelator and prednisolone use (p-value = 0.009). Conclusions: We demonstrated a high prevalence of low BMD in adolescents with non-deletional hemoglobin H disease. Moreover, dickkopf-1 inversely associated with total body BMD suggesting it may serve as a bone biomarker in this patient population.

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来源期刊
Journal of Clinical Densitometry
Journal of Clinical Densitometry 医学-内分泌学与代谢
CiteScore
4.90
自引率
8.00%
发文量
92
审稿时长
90 days
期刊介绍: The Journal is committed to serving ISCD''s mission - the education of heterogenous physician specialties and technologists who are involved in the clinical assessment of skeletal health. The focus of JCD is bone mass measurement, including epidemiology of bone mass, how drugs and diseases alter bone mass, new techniques and quality assurance in bone mass imaging technologies, and bone mass health/economics. Combining high quality research and review articles with sound, practice-oriented advice, JCD meets the diverse diagnostic and management needs of radiologists, endocrinologists, nephrologists, rheumatologists, gynecologists, family physicians, internists, and technologists whose patients require diagnostic clinical densitometry for therapeutic management.
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