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J Clin Endocrinol Metab：持续生长激素治疗普拉德-威利综合征

2016-08-24 Moon 译 MedSci原创

普拉德-威利综合征生长激素肥胖
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普拉德-威利综合征 (Prader-Willi syndrome；PWS)又称低肌张力-低智能-性腺发育底下-肥胖综合征；病因源于第15号染色体基因缺陷，患儿拥有正常语言能力，但实际智商低于普通人。患有这种病的人在新生儿期喂养困难、生长缓慢，一般自2岁左右开始无节制饮食，导致脂肪量(FM)的增加和瘦体重（LBM）的下降，进而导致体重持续增加及严重肥胖，需预防因肥胖导致的糖尿病、高血脂、高血压、脊柱

普拉德-威利综合征 (Prader-Willi syndrome；PWS)又称低肌张力-低智能-性腺发育底下-肥胖综合征；病因源于第15号染色体基因缺陷，患儿拥有正常语言能力，但实际智商低于普通人。

患有这种病的人在新生儿期喂养困难、生长缓慢，一般自2岁左右开始无节制饮食，导致脂肪量(FM)的增加和瘦体重（LBM）的下降，进而导致体重持续增加及严重肥胖，需预防因肥胖导致的糖尿病、高血脂、高血压、脊柱侧弯等症状。

在儿童时期，生长激素（GH）治疗能对抗增加肥胖的自然过程；当达到成年身高（AH）后停止GH治疗可能让改善的临床情况恶化，持续GH治疗可能会让患者受益。

有研究者对从儿童期开始，进行许多年GH治疗的27名PWS年轻成年患者进行了随机双盲安慰剂对照交叉研究，调查患者达到AH后，继续GH治疗或安慰剂对成体成分的影响。研究长达2年。在第一年，GH (0.67 mg/m2/天)和安慰剂进交叉干预。使用DXA测量身体组成。

在安慰剂期间，FM增加（相对变化+ 21.5%，P＜0.001）。与安慰剂组相比，GH治疗导致较低的FM(-2.9 kg, p=0.004)和较高的LBM(+1.5 kg, p=0.005)，相对变化分别为-17.3% FM和+3.5% LBM。GH组与安慰剂组相比，不管是四肢FM%还是躯干FM%均下降（相对变化分别为+17.3%和+15.6%, p值分别为p<0.001和p=0.007）。研究期间没有GH相关不良事件发生。

一直使用GH治疗的PWS成年患者达到AH后，持续使用GH治疗，能让患者受益。在安慰剂期间，FM增加；GH与安慰剂相比可降低FM，增加LBM。因此GH治疗可以维持身体组成的改善，并且没有安全问题。

原始出处：

Kuppens RJ.et al.Beneficial effects of growth hormone in young adults with Prader-Willi syndrome: a 2-year cross-over trial.J Clin Endocrinol Metab. 2016 Aug 23:jc20162594. [Epub ahead of print]


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    2017-05-02 guojianrong

    #Meta#

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    2017-05-05 智智灵药

    #MET#

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CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120309, encodeId=175c12030980, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=)]
    2017-04-20 wenjie

    #普拉德-威利综合征#

    0

  4. 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    2016-09-20 1e10c84am36(暂无匿称)

    文章很好，非常有益

    0

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    2016-09-12 舒心和人

    学习了新知识，感谢无私奉献

    0

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content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120311, encodeId=b20212031169, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=32, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120309, encodeId=175c12030980, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=)]
    2016-09-10 1def6e10m76(暂无匿称)

    很好的课程，学到很多，继续学习

    0

  7. 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topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:59:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120313, encodeId=a6af1203133f, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120319, encodeId=56b4120319d1, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120311, encodeId=b20212031169, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=32, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120309, encodeId=175c12030980, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=)]
    2016-09-10 1def6e10m76(暂无匿称)

    很好的课程，学到很多，继续学习

    0

  8. 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CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120309, encodeId=175c12030980, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=)]
    2016-09-10 1def6e10m76(暂无匿称)

    很好的课程，学到很多，继续学习

    0

  9. 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topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:59:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120313, encodeId=a6af1203133f, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120319, encodeId=56b4120319d1, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120311, encodeId=b20212031169, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=32, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120309, encodeId=175c12030980, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=26, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=)]
    2016-09-10 1def6e10m76(暂无匿称)

    很好的课程，学到很多，继续学习

    0

  10. 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createdName=智智灵药, createdTime=Fri May 05 17:15:00 CST 2017, time=2017-05-05, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1685325, encodeId=83851685325a9, content=<a href='/topic/show?id=3082599307e' target=_blank style='color:#2F92EE;'>#普拉德-威利综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=39, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=59930, encryptionId=3082599307e, topicName=普拉德-威利综合征)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=4cf828108646, createdName=wenjie, createdTime=Thu Apr 20 07:15:00 CST 2017, time=2017-04-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=131643, encodeId=163713164304, content=文章很好，非常有益, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzdAIB7UwTkHt6fQkBP8ictliaXtc5gCUmYdAW1sAETC2nhO3q6YjRHkUibyOCtP8ibmXNObTyd63A076/0, createdBy=d4111948983, createdName=1e10c84am36(暂无匿称), createdTime=Tue Sep 20 22:12:00 CST 2016, time=2016-09-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=123949, encodeId=58b41239493e, content=学习了新知识，感谢无私奉献, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160907/IMG57D00CF1965701770.jpg, createdBy=565c1948251, createdName=舒心和人, createdTime=Mon Sep 12 19:56:00 CST 2016, time=2016-09-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120326, encodeId=fc65120326d7, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:59:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120313, encodeId=a6af1203133f, content=很好的课程，学到很多，继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=29, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTInOuicIVN8Y32NO4Q53Zpd86ObmNp2WIotichCD0qQHaUBeHj7hgwQBCzX7ce4aEw5ZP8icQQfLH9Zw/132, createdBy=4ec21734756, createdName=1def6e10m76(暂无匿称), createdTime=Sat Sep 10 18:58:00 CST 2016, time=2016-09-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=120319, encodeId=56b4120319d1, 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    2016-09-10 1def6e10m76(暂无匿称)

    很好的课程，学到很多，继续学习

    0

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