溶血性尿毒症综合征的妊娠和分娩:一例代孕病例报告。

IF 2.6 3区 医学 Q2 OBSTETRICS & GYNECOLOGY
Norayr Nver Ghukasyan, Edita Eduard Gharibyan, Andranik Poghos Poghosyan, Milena Manvel Voskanyan, Georgy Grigory Okoev, Harutyun Norayr Mangoyan, Lusine Simon Sahakyan, Heghine Seyran Khachatryan
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引用次数: 0

摘要

妊娠合并溶血性尿毒症综合征(溶血性尿毒症综合征)及其变异对产科护理提出了重大挑战。血栓性微血管病(TMA)是溶血性尿毒综合征的一个关键特征,涉及可影响任何器官的微血管血栓形成,导致血小板减少、库姆阴性溶血性贫血和器官功能障碍。妊娠患者中最常见的血栓性微血管病变形式包括溶血、肝酶升高、血小板计数低(HELLP)综合征、血栓性血小板减少性紫癜、溶血性尿毒综合征和妊娠急性脂肪肝。tma分为遗传性或获得性原发性、继发性或感染相关tma。目前的分类将原发性tma定义为遗传性(ADAMTS13、MMACHC [cb1c缺乏症]或编码补体蛋白的基因突变)或获得性(ADAMTS13自身抗体或补体因子H (FH)自身抗体,与CFHR3/1纯合子缺失相关)。TMA与多种感染有关,包括产志贺毒素大肠杆菌引起的溶血性尿毒综合征(STEC-HUS)和肺炎球菌引起的溶血性尿毒综合征,以及其他细菌和病毒感染。继发性tma发生在各种情况下,在许多情况下,致病机制是多因素的或未知的。这里提出的分类并不明确:在一些继发性TMA中,例如妊娠相关TMA或移植后新发TMA,很大一部分个体将具有原发性TMA的遗传易感性。非典型溶血性尿毒综合征在怀孕期间尤其令人担忧,因为它是由参与免疫系统替代途径的补体调节蛋白的遗传和获得性突变引起的。我们报告一个亚美尼亚代孕母亲谁发展大肠杆菌介导的溶血性尿毒综合征并发败血症,急性肾损伤和TMA的临床特征,包括神经系统的改变。尽管有这些严重的并发症,患者在剖宫产后接受血浆交换治疗后才开始出现改善。本病例强调了在妊娠期出现TMA特征时高度怀疑溶血性尿毒综合征的必要性。及时诊断对于确保及时有效治疗至关重要,因为延误可能导致严重的孕产妇和胎儿发病率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pregnancy and delivery in the context of hemolytic uremic syndrome: A surrogacy case report.

Pregnancy complicated by hemolytic uremic syndrome (HUS) and its variants presents significant challenges in obstetric care. Thrombotic microangiopathy (TMA), a key feature of HUS, involves microvascular thrombosis that can affect any organ, leading to thrombocytopenia, Coombs-negative hemolytic anemia, and organ dysfunction. The most common forms of thrombotic microangiopathies encountered in pregnant patients include hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome, thrombotic thrombocytopenic purpura, HUS and acute fatty liver of pregnancy. TMAs are classified into inherited or acquired primary, secondary, or infection-associated TMAs. Current classifications define primary TMAs as hereditary (mutations in ADAMTS13, MMACHC [cb1c deficiency], or genes encoding complement proteins) or acquired (autoantibodies to ADAMTS13, or autoantibodies to complement Factor H (FH), which is associated with homozygous CFHR3/1 deletion). TMA is associated with various infections, including Shiga toxin-producing Escherichia coli-induced HUS (STEC-HUS) and pneumococcal HUS, as well as other bacterial and viral infections. Secondary TMAs occur in a spectrum of conditions, and in many cases the pathogenic mechanisms are multifactorial or unknown. The classification presented here is not unequivocal: in some secondary TMAs, for example pregnancy-associated TMA or de novo TMA after transplantation, a significant proportion of individuals will have a genetic predisposition to a primary TMA. Atypical HUS is particularly concerning during pregnancy as it results from genetic and acquired mutations in complement regulatory proteins, those involved in the alternative pathway of the immune system. We report the case of an Armenian surrogate mother who developed Escherichia coli-mediated HUS complicated by septicemia, acute kidney injury and clinical features of TMA, including neurological alterations. Despite these severe complications, the patient only began to show improvement after undergoing plasma exchange therapy following a cesarean delivery. This case underscores the critical need for heightened suspicion of HUS during pregnancy when TMA features are present. Prompt diagnosis is essential to ensure timely and effective treatment, as delays can lead to significant maternal and fetal morbidity.

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来源期刊
CiteScore
5.80
自引率
2.60%
发文量
493
审稿时长
3-6 weeks
期刊介绍: The International Journal of Gynecology & Obstetrics publishes articles on all aspects of basic and clinical research in the fields of obstetrics and gynecology and related subjects, with emphasis on matters of worldwide interest.
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