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Anakinra is safe and effective in controlling hyperimmunoglobulinaemia D syndrome-associated febrile crisis.阿那白滞素在控制高免疫球蛋白D综合征相关发热危象方面安全有效。
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4
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Inefficacy of etanercept in a child with hyper-IgD syndrome and periodic fever.依那西普对一名高IgD综合征伴周期性发热儿童无效。
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Cytokine activation during attacks of the hyperimmunoglobulinemia D and periodic fever syndrome.高免疫球蛋白D血症和周期性发热综合征发作期间的细胞因子激活
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患者患有高免疫球蛋白 D 综合征,出现钱币状角膜病变。

Nummular keratopathy in a patient with Hyper-IgD Syndrome.

机构信息

Washington University School of Medicine, 660 South Euclid Ave, Saint Louis, Missouri 63110, USA.

出版信息

Pediatr Rheumatol Online J. 2009 Aug 5;7:14. doi: 10.1186/1546-0096-7-14.

DOI:10.1186/1546-0096-7-14
PMID:19656398
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2731058/
Abstract

PURPOSE

To report a case of recurrent nummular keratitis in a pediatric patient with Hyperimmunoglobulinemia D syndrome.

METHODS

A retrospective chart review.

RESULTS

A 14-year-old boy with Hyperimmunoglobulinemia D syndrome (HIDS) presented with photophobia and ocular irritation concomitant with disease exacerbation. He was found on exam to have significant nummular keratitis, which responded to a short course of topical steroids. Despite acute response to local immunosuppression, the patient had several recurrent attacks and eventually developed a large corneal scar and decreased vision. After initiation of infliximab therapy his ocular sequelae improved dramatically and his vision returned to 20/20.

CONCLUSION

One possible form of end-organ damage associated with HIDS is vision threatening nummular keratopathy.

摘要

目的

报告一例儿童高免疫球蛋白 D 综合征(HIDS)患者反复发作的钱币状角膜炎病例。

方法

回顾性病历分析。

结果

一名 14 岁男孩患有 HIDS(高免疫球蛋白 D 综合征),在疾病加重时出现畏光和眼部刺激。检查发现他有明显的钱币状角膜炎,局部应用皮质类固醇治疗后迅速缓解。尽管局部免疫抑制有急性反应,但患者反复发作数次,最终发展为大的角膜瘢痕和视力下降。开始使用英夫利昔单抗治疗后,他的眼部后遗症明显改善,视力恢复至 20/20。

结论

HIDS 相关的一种可能的靶器官损害形式是威胁视力的钱币状角膜病变。