KIDNEY INT:有“高原红细胞增多症”的藏族居民,他们的肾脏临床病理特征表现如何?

2022-06-30 liangying MedSci原创

慢性缺氧本身和继发的血流动力学变化都可能参与HAPC相关肾病的发病机制。进一步研究该病的病理机制和治疗至关重要。

慢性高原病(CMS)是世界山区的一个重大公共卫生问题,影响着数百万高海拔居民。CMS发生于长期居住在海拔2500米以上的居民中,其特点是红细胞增多(女性患者,血红蛋白高于190g/L;男性患者,血红蛋白高于210 g/L)和严重低氧血症。CMS在高海拔地区的发病率估计为5%-10%,被认为是对生活在高海拔地区的一种异常反应。术语“高海拔红细胞增多症”(HAPC)被专门用来指Hb水平。

HAPC是一种临床综合征,发生于当地居民或长期居住在高原的居民。肾脏是受影响最严重的器官之一。然而,HAPC相关肾脏疾病的临床和肾脏组织病理学特征鲜有报道。本研究报告了以肾活检为基础的HAPC藏族居民肾脏疾病的临床病理特征。

2016年1月1日至2020年11月31日,共有416名藏族患者接受了肾活检。其中17名患者符合HAPC相关肾病的诊断标准。临床上,这些患者的平均尿蛋白水平为2.5g/24小时(范围1.81-6.85)。12名患者患有高尿酸血症,9名患者患有高血压,3名患者患有肾功能不全。

组织病理学以肾小球肥大、肾小球基底膜增厚、足细胞足突消失、节段性肾小球硬化和全局性肾小球硬化为主要特征。肾小球外动脉/小动脉病变常见,表现为内膜纤维化、透明质增生和内皮细胞肿胀/内膜下水肿。以平滑肌细胞增殖为特征的动脉/小动脉内侧壁区域扩张明显,可能提示血管重塑。缺氧诱导因子2α在这些患者的肾组织中表达。HAPC相关肾脏疾病的病理变化包括肾小球和肾小球外血管病变,表明慢性缺氧本身和继发性血流动力学变化在该疾病的发病机制中起着关键作用。

高原红细胞增多症(HAPC)相关肾脏疾病患者动脉/小动脉病变的代表性光镜表现如箭头所示

本研究首次对HAPC相关肾病患者的肾脏组织病理学和临床特征进行了活检描述。该队列中的疾病表现为中度至重度蛋白尿,伴有相对较高频率的高尿酸血症和高血压。肾功能不全可发生在晚期。肾活检的病理特征包括肾小球和肾小球外血管病变。在肾组织中观察到HIF-2a而非HIF-1a的表达。本研究表明,慢性缺氧本身和继发的血流动力学变化都可能参与HAPC相关肾病的发病机制。进一步研究该病的病理机制和治疗至关重要。

参考文献:Hui Wang, Chen Tang, Zonghui Dang, A Yong, Lijun Liu, Suxia Wang, Minghui Zhao,
Clinicopathological characteristics of high-altitude polycythemia-related kidney disease in Tibetan inhabitants, Kidney International, Volume 102, Issue 1, 2022, Pages 196-206, ISSN 0085-2538, https://doi.org/10.1016/j.kint.2022.03.027.

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    2023-04-04 weiz
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  7. [GetPortalCommentsPageByObjectIdResponse(id=1797499, encodeId=54021e9749974, content=<a href='/topic/show?id=96eb22906b9' target=_blank style='color:#2F92EE;'>#临床病理#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=22906, encryptionId=96eb22906b9, topicName=临床病理)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Wed Apr 26 18:55:24 CST 2023, time=2023-04-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2033215, encodeId=37f5203321537, content=<a href='/topic/show?id=c097e104497' target=_blank style='color:#2F92EE;'>#病理特征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=40, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71044, encryptionId=c097e104497, topicName=病理特征)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c279140, createdName=vividelife, createdTime=Sun Mar 12 10:55:24 CST 2023, time=2023-03-12, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1809023, encodeId=7e7818090232c, content=<a href='/topic/show?id=6d2e82668f0' target=_blank style='color:#2F92EE;'>#肾脏临床#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=27, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=82668, encryptionId=6d2e82668f0, topicName=肾脏临床)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=1c77441, createdName=weiz, createdTime=Tue Apr 04 03:55:24 CST 2023, time=2023-04-04, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1282109, encodeId=25981282109ad, content=<a href='/topic/show?id=3dece71531e' target=_blank style='color:#2F92EE;'>#红细胞#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=77153, encryptionId=3dece71531e, topicName=红细胞)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c22f171, createdName=kord1982, createdTime=Sat Jun 25 07:55:24 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1441483, encodeId=9e2814414833b, content=<a href='/topic/show?id=9bdc884e39d' target=_blank style='color:#2F92EE;'>#藏族#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=39, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=88473, encryptionId=9bdc884e39d, topicName=藏族)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=3de94846329, createdName=ms49895894946433513521, createdTime=Sat Jun 25 07:55:24 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1555475, encodeId=c3fb15554e58a, content=<a href='/topic/show?id=f016e71581b' target=_blank style='color:#2F92EE;'>#红细胞增多症#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=42, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=77158, encryptionId=f016e71581b, topicName=红细胞增多症)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=4a1014606284, createdName=linlin2317, createdTime=Sat Jun 25 07:55:24 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1574873, encodeId=f25315e48731c, content=<a href='/topic/show?id=6d6210228935' target=_blank style='color:#2F92EE;'>#高原#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=102289, encryptionId=6d6210228935, topicName=高原)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=687116029883, createdName=jmtang, createdTime=Sat Jun 25 07:55:24 CST 2022, time=2022-06-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1603175, encodeId=93c716031e50b, content=<a href='/topic/show?id=81b91049067' target=_blank style='color:#2F92EE;'>#kidney#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=10490, encryptionId=81b91049067, topicName=kidney)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=c36e18990621, createdName=gwc389, createdTime=Sat Jun 25 07:55:24 CST 2022, time=2022-06-25, status=1, ipAttribution=)]
    2022-06-25 jmtang
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    2022-06-25 gwc389