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本文引用的文献

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Risk of diagnostic delay in congenital thrombotic thrombocytopenic purpura.先天性血栓性血小板减少性紫癜的诊断延迟风险。
J Thromb Haemost. 2019 Apr;17(4):666-669. doi: 10.1111/jth.14409. Epub 2019 Mar 6.
2
Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome.妊娠期间诊断为 Upshaw-Schulman 综合征并伴有可逆性脑血管收缩综合征。
Transfus Apher Sci. 2018 Dec;57(6):790-792. doi: 10.1016/j.transci.2018.10.023. Epub 2018 Nov 16.
3
Upshaw-Schulman Syndrome With c.2728C>T Mutation in ADAMTS13 Gene.伴有ADAMTS13基因c.2728C>T突变的厄普肖-舒尔曼综合征
J Pediatr Hematol Oncol. 2019 Jan;41(1):e60-e62. doi: 10.1097/MPH.0000000000001226.
4
PROFOUND VISUAL RECOVERY AT 16 MONTHS AFTER RESOLUTION OF SEROUS RETINAL DETACHMENTS SECONDARY TO THROMBOTIC THROMBOCYTOPENIC PURPURA: CASE REPORT AND LITERATURE REVIEW.血栓性血小板减少性紫癜继发浆液性视网膜脱离 16 个月后视力完全恢复：病例报告及文献复习。
Retin Cases Brief Rep. 2021 Jan 1;15(1):18-21. doi: 10.1097/ICB.0000000000000727.
5
Inherited Thrombotic Thrombocytopenic Purpura (Upshaw Schulman Syndrome) as Differential Diagnosis to Neonatal Septicaemia with Disseminated Intravascular Coagulation - a Case Series.遗传性血栓性血小板减少性紫癜（舒-乌综合征）作为新生儿败血症合并弥散性血管内凝血的鉴别诊断——病例系列
Z Geburtshilfe Neonatol. 2017 Feb;221(1):39-42. doi: 10.1055/s-0042-109404. Epub 2016 Jul 6.
6
Upshaw-Schulman Syndrome.厄普肖-舒尔曼综合征
Med J Armed Forces India. 2010 Apr;66(2):188-9. doi: 10.1016/S0377-1237(10)80149-2. Epub 2011 Jul 21.
7
A case of congenital TTP presenting with microganiopathy in adulthood.一例成年期出现微血管病的先天性血栓性血小板减少性紫癜病例。
BMC Hematol. 2014 Sep 12;14(1):16. doi: 10.1186/2052-1839-14-16. eCollection 2014.
8
Inherited ADAMTS13 deficiency (Upshaw-Schulman syndrome): a short review.遗传性ADAMTS13缺乏症（乌-舒二氏综合征）：简要综述。
Thromb Res. 2014 Dec;134(6):1171-5. doi: 10.1016/j.thromres.2014.09.004. Epub 2014 Sep 10.
9
Paradigm shift of childhood thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency.伴有严重ADAMTS13缺乏的儿童血栓性血小板减少性紫癜的范式转变。
Presse Med. 2012 Mar;41(3 Pt 2):e137-55. doi: 10.1016/j.lpm.2011.10.027. Epub 2012 Jan 20.
10
Ten candidate ADAMTS13 mutations in six French families with congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome).六个患有先天性血栓性血小板减少性紫癜（乌-舒综合征）的法国家族中的十种ADAMTS13候选突变。
J Thromb Haemost. 2004 Mar;2(3):424-9. doi: 10.1111/j.1538-7933.2004.00623.x.

与厄普肖-舒尔曼综合征相关的双侧缺血性视网膜病变：一例报告

Bilateral Ischemic Retinopathy Associated With Upshaw-Schulman Syndrome: A Case Report.

作者信息

Cernichiaro-Espinosa Mariam, Meizner-Grezemkovsky Daniela, López-Santiago Norma, Borbolla-Pertierra Ana M, Morales-Cantón Virgilio, Cernichiaro-Espinosa Linda A

机构信息

Pediatric Retina Unit, Retina and Vitreous Department, Asociación para Evitar la Ceguera en México, I.A.P. Ciudad de México, Mexico.

Pediatric Onco-Hemathology Department, Instituto Nacional de Pediatría, Ciudad de México, Mexico.

出版信息

J Vitreoretin Dis. 2021 Oct 21;6(2):163-166. doi: 10.1177/24741264211049694. eCollection 2022 Mar-Apr.

DOI:10.1177/24741264211049694
PMID:37008656
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9976007/
Abstract

PURPOSE

This is the first report to our knowledge of ischemic retinopathy in a pediatric patient with Upshaw-Schulman syndrome (USS).

METHODS

A 6-year-old girl previously diagnosed with USS was referred to our clinic with exodeviation of the left eye and a 2-month-long decrease in vision of both eyes. A dilated fundus examination showed a total vitreous hemorrhage in both eyes. The first course of action was conservative treatment, with the patient experiencing visual-acuity improvement in her right eye.

RESULTS

An ischemic retina and optic nerve atrophy was found once the left eye was cleared of the hemorrhage.

CONCLUSIONS

We present a case of a vitreous hemorrhage, possibly secondary to an episode of severe thrombocytopenia. Following USS diagnosis, providers should perform dilated ophthalmologic examinations as part of initial and follow-up general evaluations. This case exemplifies that, in understudied and underdescribed pediatric retinal diseases, extreme therapeutic decisions-such as surgery-should not be rushed.

摘要

目的

据我们所知，这是关于患有厄普肖-舒尔曼综合征（USS）的儿科患者发生缺血性视网膜病变的首例报告。

方法

一名先前被诊断为USS的6岁女孩因左眼外斜视和双眼视力下降2个月前来我院就诊。散瞳眼底检查显示双眼全玻璃体出血。首要治疗措施为保守治疗，患者右眼视力有所改善。

结果

左眼出血清除后，发现存在缺血性视网膜和视神经萎缩。

结论

我们报告了一例可能继发于严重血小板减少症发作的玻璃体出血病例。在诊断USS后，医疗人员应进行散瞳眼科检查，作为初始和后续综合评估的一部分。该病例表明，在研究不足和描述较少的儿科视网膜疾病中，不应仓促做出诸如手术等极端治疗决策。