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未接触过钆的患者发生肾源性系统性纤维化:口服西罗莫司治疗成功

Nephrogenic systemic fibrosis in a gadolinium-naïve patient: successful treatment with oral sirolimus.

作者信息

Ross Christopher, De Rosa Nicholas, Marshman Gillian, Astill David

机构信息

Department of Dermatology, Flinders Medical Centre, Adelaide, South Australia, Australia.

Department of Surgical Pathology, Flinders Medical Centre, Adelaide, South Australia, Australia.

出版信息

Australas J Dermatol. 2015 Aug;56(3):e59-62. doi: 10.1111/ajd.12176. Epub 2014 Oct 21.

DOI:10.1111/ajd.12176
PMID:25330850
Abstract

A 52-year old woman with chronic renal failure presented with tender buttock nodules, bilateral non-tender periocular papules and yellow scleral plaques. The patient then developed sclerodermoid changes of the hands, as well as woody induration, oedema and hyperpigmentation of lower limbs. There was no previous exposure to gadolinium contrast. Her histology and clinical features were consistent with nephrogenic systemic fibrosis. Treatment with oral sirolimus resulted in a marked reduction of induration and oedema, and an improvement in distal upper limb and lower limb joint mobility.

摘要

一名52岁的慢性肾衰竭女性出现臀部压痛性结节、双侧眼周无压痛性丘疹和巩膜黄斑。随后,患者手部出现硬皮病样改变,下肢出现木样硬结、水肿和色素沉着。患者既往未接触过钆造影剂。其组织学和临床特征符合肾源性系统性纤维化。口服西罗莫司治疗后,硬结和水肿明显减轻,上肢远端和下肢关节活动度改善。

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引用本文的文献

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The Clinical Manifestations and Efficacy of Different Treatments Used for Nephrogenic Systemic Fibrosis: A Systematic Review.肾源性系统性纤维化不同治疗方法的临床表现及疗效:一项系统评价
Int J Nephrol Renovasc Dis. 2023 Jan 12;16:17-30. doi: 10.2147/IJNRD.S392231. eCollection 2023.
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A unique case of nephrogenic systemic fibrosis from gadolinium exposure in a patient with normal eGFR.一名估算肾小球滤过率(eGFR)正常的患者因接触钆而发生肾源性系统性纤维化的罕见病例。
BMJ Case Rep. 2017 Oct 11;2017:bcr-2017-221016. doi: 10.1136/bcr-2017-221016.
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Edema, Hyperpigmentation, Induration: 3 Skin Signs Heralding Danger in Patients on Maintenance Hemodialysis.
水肿、色素沉着、硬结:血液透析患者出现的三种预示危险的皮肤体征
Medicine (Baltimore). 2016 Mar;95(12):e3121. doi: 10.1097/MD.0000000000003121.