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Osteoporos Int：成骨不全患儿的健康相关生活质量的调查研究

2018-12-23 MedSci MedSci原创

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成骨不全症（OI）是一种遗传性疾病，其特征在于多个脆性骨折和进行性骨骼畸形。目前尚没有在大量OI患者样本中进行关于生活质量（QoL）的详细调查。本研究中，研究人员在一个大而且特征明确的OI队列中评估了QoL及其影响因素。使用经过验证的PedsQL 4.0调查问卷来评估患有OI的儿童和青少年的健康相关生活质量（HRQoL）。比较I型，III型和IV型OI患者的HRQoL。研究了HRQoL与候选OI基

成骨不全症（OI）是一种遗传性疾病，其特征在于多个脆性骨折和进行性骨骼畸形。目前尚没有在大量OI患者样本中进行关于生活质量（QoL）的详细调查。本研究中，研究人员在一个大而且特征明确的OI队列中评估了QoL及其影响因素。

使用经过验证的PedsQL 4.0调查问卷来评估患有OI的儿童和青少年的健康相关生活质量（HRQoL）。比较I型，III型和IV型OI患者的HRQoL。研究了HRQoL与候选OI基因中致病突变之间的关系。此外，研究人员还评估了OI患者HRQoL的影响因素。

结果，共有138名患有OI的儿童和138名健康对照参加了这项研究。OI患者的HRQoL评分在体格，情感，社交，学校功能和总分上分别为64.4±30.0,71.9±22.2,75.7±24.8,63.7±24.5和68.9±22.0，显著低于健康儿童（86.5±12.7,83.3±16.0,92.1±11.8,87.5±11.8和87.3±10.7，均p <0.01）。中度和重度OI（III/IV型）患者的HRQoL评分低于轻度OI患者（I型）。除了情绪功能外，诱导I型胶原蛋白定性缺陷的基因突变导致HRQoL评分比I型胶原蛋白定量缺陷的评分更差。HRQoL总分与家庭收入、腰椎和股骨骨密度（BMD）Z评分呈正相关，与疾病严重程度和骨折频率呈负相关。

综上所述，该研究结果表明，HRIoL在OI患者中显着受损，并且具有更严重OI的患者具有较差的HRQoL评分。我们第一次发现，I型胶原蛋白质定性缺陷的儿童HRQoL评分低于I型胶原蛋白定量缺陷患儿。

原始出处：

Song Y, Zhao D, et al., Health-related quality of life in children with osteogenesis imperfecta: a large-sample study. Osteoporos Int. 2018 Dec 19. doi: 10.1007/s00198-018-4801-5.

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    2018-12-25 zhaojie88

    #成骨#

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=1351922, encodeId=1433135192298, content=<a href='/topic/show?id=e95954093cd' target=_blank style='color:#2F92EE;'>#成骨#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=54093, encryptionId=e95954093cd, topicName=成骨)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4c37265, createdName=zhaojie88, createdTime=Tue Dec 25 04:59:00 CST 2018, time=2018-12-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1364431, encodeId=75bc136443103, content=<a href='/topic/show?id=7c5615628ad' target=_blank style='color:#2F92EE;'>#ROS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=15628, encryptionId=7c5615628ad, topicName=ROS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=acc3210, createdName=lsndxfj, createdTime=Tue Dec 25 04:59:00 CST 2018, time=2018-12-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1522196, encodeId=364715221960a, content=<a href='/topic/show?id=d08e2e894e7' target=_blank style='color:#2F92EE;'>#健康相关生活质量#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=27894, encryptionId=d08e2e894e7, topicName=健康相关生活质量)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=cd0411427202, createdName=yilong5287546, createdTime=Tue Dec 25 04:59:00 CST 2018, time=2018-12-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=356200, encodeId=274735620010, content=感谢小编为我们准备了如此丰盛的精神大餐，同时也向作者致谢！认真学习了，点赞！, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=30251637888, createdName=lietome15, createdTime=Sun Dec 23 14:27:55 CST 2018, time=2018-12-23, status=1, ipAttribution=)]
    2018-12-25 lsndxfj

    #ROS#

    0

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    2018-12-25 yilong5287546

    #健康相关生活质量#

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=1351922, encodeId=1433135192298, content=<a href='/topic/show?id=e95954093cd' target=_blank style='color:#2F92EE;'>#成骨#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=54093, encryptionId=e95954093cd, topicName=成骨)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4c37265, createdName=zhaojie88, createdTime=Tue Dec 25 04:59:00 CST 2018, time=2018-12-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1364431, encodeId=75bc136443103, content=<a href='/topic/show?id=7c5615628ad' target=_blank style='color:#2F92EE;'>#ROS#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=20, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=15628, encryptionId=7c5615628ad, topicName=ROS)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=acc3210, createdName=lsndxfj, createdTime=Tue Dec 25 04:59:00 CST 2018, time=2018-12-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1522196, encodeId=364715221960a, content=<a href='/topic/show?id=d08e2e894e7' target=_blank style='color:#2F92EE;'>#健康相关生活质量#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=27894, encryptionId=d08e2e894e7, topicName=健康相关生活质量)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=cd0411427202, createdName=yilong5287546, createdTime=Tue Dec 25 04:59:00 CST 2018, time=2018-12-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=356200, encodeId=274735620010, content=感谢小编为我们准备了如此丰盛的精神大餐，同时也向作者致谢！认真学习了，点赞！, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=30251637888, createdName=lietome15, createdTime=Sun Dec 23 14:27:55 CST 2018, time=2018-12-23, status=1, ipAttribution=)]
    2018-12-23 lietome15

    感谢小编为我们准备了如此丰盛的精神大餐，同时也向作者致谢！认真学习了，点赞！

    0