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JCEM:X连锁性低磷血症的患病率和死亡率

2019-11-16 xing.T MedSci原创

由此可见,该研究保守估计了英国儿童和成人中XLH的患病率。这些患者死亡率出乎意料地增加,可能与其他FGF23相关的疾病有关。

X连锁性低磷酸盐血症(XLH)是一种罕见的多系统性疾病,具有明显的肌肉骨骼表型。近日,内分泌和代谢性疾病领域权威杂志Journal of Clinical Endocrinology & Metabolism上发表了一篇研究文章,研究人员旨在增进人们对整个生命过程中XLH患病率和整体生存率的了解。

这是一项基于人群的队列研究,研究人员使用了英国(UK)1995-2016年期间的大型初级保健数据库。XLH病例按年龄、性别和治疗进行了匹配,最多匹配四个对照者。研究人员估计了研究期间的患病率趋势(按年龄分层),并将病例之间的存活率与对照组进行比较。

在522例潜在病例中,至少有122例(23.4%)被诊断为XLH,而62例(11.9%)被分类为极有可能(保守定义)。在主要分析中,患病率(95%CI)从1995-1999年的百万分之3.1(1.5-6.7)增加到2012-2016年的百万分之14.0(10.8-18.1)。使用保守定义相应的估计值为3.0(1.4-6.5)到8.1(5.8-11.4)。随访期间有9例(7.4%)死亡,中位年龄为64岁。对照组中有14人(2.9%)死亡,年龄中位数为72.5岁。与对照组相比,可能患有XLH的个体死亡率显著增加(风险比[HR]为2.93,95%CI为1.24-6.91)。同样,在那些可能或非常可能患有XLH的人群中(HR为6.65,1.44-30.72)。

由此可见,该研究保守估计了英国儿童和成人中XLH的患病率。这些患者死亡率出乎意料地增加,可能与其他FGF23相关的疾病有关。 

原始出处:

Samuel Hawley,et al.Prevalence and mortality of individuals with X-linked hypophosphataemia: a United Kingdom real world data analysis.J Clin Endocrinol Metab.2019.https://doi.org/10.1210/clinem/dgz203

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    2020-01-19 achengzhao
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    2020-04-29 smallant2015
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