少年佩吉特病的骨膜蛋白和硬化蛋白水平。

Q3 Medicine Clinical Cases in Mineral and Bone Metabolism Pub Date : 2017-05-01 Epub Date: 2017-10-25 DOI:10.11138/ccmbm/2017.14.2.269
Stergios A Polyzos, Polyzois Makras, Athanasios D Anastasilakis, Gesthimani Mintziori, Marina Kita, Athanasios Papatheodorou, Panagiotis Kokkoris, Evangelos Terpos
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引用次数: 3

摘要

少年佩吉特病(JPD)是一种罕见的常染色体隐性遗传病,其特征是血清碱性磷酸酶活性显著升高,表明整个骨骼的骨转换大大加速。本研究的主要目的是评估两名患有轻度JPD(由于“巴尔干”突变)的成年患者的循环骨膜蛋白和硬化蛋白水平。我们测量了一名先前描述的女性和一名新诊断的JPD男性的骨膜蛋白和硬化蛋白水平,以及10名明显健康的个体,在性别、年龄和体重指数上与JPD患者匹配(1:5)。JPD患者和对照组之间的硬化蛋白水平相似。JPD患者的Periostin水平高出约2.5倍。骨膜蛋白与硬化蛋白水平呈负相关(rs= -0.63;p = 0.03)。总之,在JPD患者中观察到高骨膜蛋白水平的趋势,而硬化蛋白水平与对照组相似。
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Periostin and sclerostin levels in juvenile Paget's disease.

Juvenile Paget's disease (JPD) is a rare, autosomal recessive disorder featuring markedly increased serum alkaline phosphatase activity, indicative of greatly accelerated bone turnover throughout the skeleton. The main aim of this study was to evaluate circulating periostin and sclerostin levels in two adult patients with mild JPD (due to "Balkan" mutation). We measured periostin and sclerostin levels in a previously described woman and a newly diagnosed man with JPD, and 10 apparently healthy individuals, matched (1:5) to JPD patients for gender, age and body mass index. Sclerostin levels were similar between JPD patients and controls. Periostin levels were about 2.5 times higher in JPD patients. Periostin and sclerostin levels were negatively correlated (rs= -0.63; p=0.03). In conclusion, a trend towards higher periostin levels was observed in JPD patients, whereas sclerostin levels were similar to controls.

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来源期刊
Clinical Cases in Mineral and Bone Metabolism
Clinical Cases in Mineral and Bone Metabolism ENDOCRINOLOGY & METABOLISM-
CiteScore
2.60
自引率
0.00%
发文量
0
期刊介绍: The Journal encourages the submission of case reports and clinical vignettes that provide new and exciting insights into the pathophysiology and characteristics of disorders related to skeletal function and mineral metabolism and/or highlight pratical diagnostic and /or therapeutic considerations.
期刊最新文献
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