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溶血尿毒综合征背景下的妊娠与分娩:一例代孕病例报告。

Pregnancy and delivery in the context of hemolytic uremic syndrome: A surrogacy case report.

作者信息

Ghukasyan Norayr Nver, Gharibyan Edita Eduard, Poghosyan Andranik Poghos, Voskanyan Milena Manvel, Okoev Georgy Grigory, Mangoyan Harutyun Norayr, Sahakyan Lusine Simon, Khachatryan Heghine Seyran

机构信息

Erebouni Medical Center, Yerevan, Armenia.

YSMU aft.M.Heratsi, Yerevan, Armenia.

出版信息

Int J Gynaecol Obstet. 2025 Apr 4. doi: 10.1002/ijgo.70144.

Abstract

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.

摘要

妊娠合并溶血尿毒综合征(HUS)及其变异型在产科护理中带来了重大挑战。血栓性微血管病(TMA)是HUS的关键特征,涉及微血管血栓形成,可影响任何器官,导致血小板减少、抗人球蛋白试验阴性的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形式包括溶血、肝酶升高和血小板计数降低(HELLP)综合征、血栓性血小板减少性紫癜、HUS和妊娠急性脂肪肝。TMA分为遗传性或获得性原发性、继发性或感染相关性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(针对ADAMTS13的自身抗体,或针对补体因子H(FH)的自身抗体,这与纯合CFHR3/1缺失有关)。TMA与各种感染有关,包括产志贺毒素大肠杆菌引起的HUS(STEC-HUS)和肺炎球菌HUS,以及其他细菌和病毒感染。继发性TMA发生在一系列疾病中,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,例如妊娠相关性TMA或移植后新发TMA,相当一部分个体将具有原发性TMA的遗传易感性。非典型HUS在妊娠期间尤其令人担忧,因为它是由补体调节蛋白的遗传和获得性突变引起的,这些蛋白参与免疫系统的替代途径。我们报告了一例亚美尼亚代孕母亲的病例,她发生了大肠杆菌介导的HUS,并伴有败血症、急性肾损伤和TMA的临床特征,包括神经改变。尽管出现了这些严重并发症,但患者在剖宫产术后接受血浆置换治疗后才开始好转。该病例强调了在妊娠期间出现TMA特征时,高度怀疑HUS的迫切需要。及时诊断对于确保及时有效的治疗至关重要,因为延误可能导致严重的母婴发病。

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