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Acta Paediatr: MPS IH儿童具有进行性视力功能障碍的风险

2018-05-11 cuiguizhong MedSci原创

瑞典斯德哥尔摩St Erik眼科医院小儿眼科的Te?rFahnehjelm K近日在Acta Paediatr发表了一篇文章,在这项前瞻性研究中,他们评估了接受造血干细胞移植(HSCT)治疗的粘多糖类I型Hurler综合征(MPS IH)儿童的长期眼部视力结果。

瑞典斯德哥尔摩St Erik眼科医院小儿眼科的Te?rFahnehjelm K近日在Acta Paediatr发表了一篇文章,在这项前瞻性研究中,他们评估了接受造血干细胞移植(HSCT)治疗的粘多糖类I型Hurler综合征(MPS IH)儿童的长期眼部视力结果。

他们对2001年至2018年在瑞典斯德哥尔摩St Erik眼科医院治疗的8名患者进行临床眼科评估。患者被诊断是的中位年龄分别为12.2个月(范围5.0-16.4),造血干细胞移植(HSCT)治疗的时间为16.7(8.0-20.4)个月。最后一次进行眼睛检查的中位年龄为13.4(6.3-19.0)岁,随访持续的中位数为12.0(5.0-17.4)年。

调查结果发现,六名儿童出现视力不佳、夜间视力不良或畏光的现象。最后一次就诊的最佳矫正视力在右眼和左眼的中位数分别为0.4和0.5,并且在两位患者中具有显着的下降现象。5个患者自接受造血干细胞移植(HSCT)治疗以来,角膜混浊度增加。所有患者均出现高度远视的症状,中位数为+6屈光度。同时出现角膜僵硬的症状。患者的眼压正常。一名患者发现视网膜变性。

因此,他们认为,尽管接受造血干细胞移植(HSCT)治疗,但粘多糖类I型Hurler综合征(MPS IH)患者的长期随访记录显示,由于角膜混浊或视网膜变性等,引起视力下降。

原文出处:

Tear Fahnehjelm, K., et al., Children with mucopolysaccharidosis risk progressive visual dysfunction despite haematopoietic stem cell transplants. Acta Paediatr, 2018.

本文系梅斯医学(MedSci)原创编译整理,转载需授权!

 

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    2018-11-15 windight
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    2018-05-17 半夏微凉

    学习了谢谢分享

    0

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status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=314151, encodeId=b4813141510d, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=82311653708, createdName=医者仁心5538, createdTime=Fri May 11 13:36:30 CST 2018, time=2018-05-11, status=1, ipAttribution=)]
    2018-05-13 makuansheng
  8. 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status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=314151, encodeId=b4813141510d, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=82311653708, createdName=医者仁心5538, createdTime=Fri May 11 13:36:30 CST 2018, time=2018-05-11, status=1, ipAttribution=)]
    2018-05-11 王秀

    学习了.涨知识了!

    0

  9. 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status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=314151, encodeId=b4813141510d, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=82311653708, createdName=医者仁心5538, createdTime=Fri May 11 13:36:30 CST 2018, time=2018-05-11, status=1, ipAttribution=)]
    2018-05-11 1ddf0692m34(暂无匿称)

    学习了.长知识

    0

  10. 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status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=314151, encodeId=b4813141510d, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=61, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=82311653708, createdName=医者仁心5538, createdTime=Fri May 11 13:36:30 CST 2018, time=2018-05-11, status=1, ipAttribution=)]
    2018-05-11 医者仁心5538

    学习了

    0

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大多数霍奇金淋巴瘤(HL)患者可通过初试治疗得到治愈。另外高剂量的自体造血干细胞移植(AHCT)可治愈额外部分的复发性或难治性HL(rrHL)患者。PET阴性的完全缓解(CR)优于AHCT,对长期病程控制至关重要。有多种补救选择可供对比缓解率,而且可根据合并症和具体情况进行选择。放射治疗也可提高缓解率,对特定的患者来说也是一种重要的治疗选择。AHCT后用BV维持治疗有助于稳定高复发风险患者的病情,

Blood:移植非允许的HLA-DPB1不匹配的供体的造血干细胞,竟可降低中危患者疾病进展的风险

现有研究人员对供体-受体HLA-DPB1匹配情况对进行异体造血干细胞移植的用ATG进行活体内T细胞耗竭的血液恶性肿瘤患者的预后的影响进行深入研究。所有的供体-受体配对(HLA-A、-B、-C、-DRB1、-DQB1和DRB3/4/5)均可明确分辨,并且在HLA-A、-B、-C和DRB1水平相匹配。HLA-DPB1不匹配通过DPB1匹配的免疫组化(利用DPB T细胞抗原表位工具)来进行分类。1004

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