HELLP Syndrome

HELLP 综合征
  • 文章类型: Case Reports
    子痫谱系障碍是一组严重的妊娠并发症,通常在妊娠20周后出现。磨牙怀孕之间有联系,一种由受精和配子生成异常引起的妊娠滋养细胞疾病,和子痫谱系障碍,可导致先兆子痫症状的表现早于妊娠20周。我们报告了一例妊娠16周时20多岁时妊娠1para0的病例,出现部分葡萄胎,患有子痫,溶血,肝酶升高和低血小板综合征和后部可逆性脑病综合征。超声检查结果与磨牙妊娠一致,病理证实磨牙部分妊娠具有三倍体69,XYY核型。该病例突出了磨牙妊娠中子痫谱系障碍的早发性潜力,同时建议对此类患者进行高血压疾病筛查。
    Eclampsia spectrum disorders are a set of serious complications of pregnancy that commonly present after 20 weeks of gestation. There is an association between molar pregnancy, a gestational trophoblastic disease resulting from abnormal fertilisation and gametogenesis, and eclampsia spectrum disorders which can result in manifestation of pre-eclamptic symptomatology earlier than 20 weeks of gestation. We report a case of a gravida 1 para 0 in her mid 20s at 16-weeks gestation presenting with partial hydatidiform mole who developed eclampsia, haemolysis, elevated liver enzymes and low platelets syndrome and posterior reversible encephalopathy syndrome. Ultrasound findings were consistent with molar pregnancy and pathology confirmed partial molar pregnancy with triploid 69, XYY karyotype. This case highlights the early onset potential of eclampsia spectrum disorders in molar pregnancies while suggesting screening such patients for hypertensive disorders.
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  • 文章类型: Case Reports
    血栓性微血管病(TMA)是一种罕见但可能危及生命的疾病。诊断是困难的,因为在围产期存在类似于TMA的特征,例如子痫,先兆子痫,溶血,肝酶升高,低血小板(HELLP)综合征,和抗磷脂综合征.一名没有明显既往病史的28岁女性在妊娠41周时紧急剖宫产后大出血后发展为TMA。最终诊断为产后出血(PPH)相关性TMA。患者在血浆置换治疗后完全康复。我们假定累积病例报告的价值,鉴于关于血浆置换在PPH相关TMA中的疗效的文献有限.
    Thrombotic microangiopathy (TMA) is a rare yet potentially life-threatening condition. The diagnosis is difficult as there are other conditions presenting with features akin to TMA during the peripartum period such as eclampsia, preeclampsia, hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome, and antiphospholipid syndrome. A 28-year-old woman with no significant past medical history developed TMA following a massive hemorrhage after an emergency cesarean section at 41 weeks of gestation. This case was finally diagnosed as postpartum hemorrhage (PPH)-associated TMA. The patient fully recovered after plasma exchange therapy. We posit the value of accumulating case reports, given that the documentation on the efficacy of plasma exchange in PPH-associated TMA is limited.
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  • 文章类型: Journal Article
    背景:先兆子痫,妊娠期高血压疾病,是一种病因不明的多系统疾病,与孕产妇死亡率和发病率的风险增加有关。先兆子痫母亲的婴儿早产发生率明显较高,躯体生长迟缓,血小板减少症,低出生体重,呼吸窘迫综合征,和长期入院新生儿重症监护(NICU)。
    目的:本研究旨在研究重度先兆子痫孕妇的孕产妇死亡率、发病率和胎儿结局。
    方法:这项观察性研究是在妇产科进行的,三级护理中心,从2015年10月到2017年10月。数据是从所有130名在三级保健医院和病房住院的产前诊所就诊的妇女中收集的,以及所有细节,例如人口统计细节,产科检查,并记录了所有临床发现,并从中得出了结果。结果:在应用纳入和排除标准后,本研究中观察到所有130名女性。在130名妇女中,有47名被诊断为先兆子痫。在21-25岁组中,主要是初产妇被诊断为先兆子痫。在47名先兆子痫妇女中,39名女性的BMI为19-25kg/m2。47名妇女中有32名(68.09%)在36-39周左右被诊断为先兆子痫。在所有先兆子痫中,47名妇女中有28名妇女(59.5%)通过阴道分娩,通过剖宫产分娩的47名妇女中有18名(38.3%),47例(2.13%)中的1例接受了阴道早产。在先兆子痫中,女性分娩的婴儿大多(25/47,53.19%)体重≤2.5kg,只有一名婴儿因低出生体重而转入NICU。先兆子痫会增加孕产妇的死亡率和发病率,但在这项研究中,由于我们的医院是三级护理中心,所有ICU(重症监护病房)和NICU都设有死亡率。
    结论:早产和剖宫产是观察到的轻度至重度结局。严重并发症导致ICU和NICU住院,对医疗设施的需求很大。对于妊娠高血压及其并发症的管理,有明确的指南。为了适当的管理,仔细考虑各种因素,和个别案例研究是必需的。
    BACKGROUND: Preeclampsia, a hypertensive disorder in pregnancy, is a multisystem disease of unknown etiology and is associated with an increased risk of maternal mortality and morbidity. Infants from preeclampsia mothers have significantly higher incidence of prematurity, somatic growth retardation, thrombocytopenia, low birth weight, respiratory distress syndrome, and long duration of admission to neonatal intensive care (NICU).
    OBJECTIVE: This study was done to study the maternal mortality and morbidity and foetal outcome in pregnant women with severe preeclampsia.
    METHODS: This observational study was done in the Department of Obstetrics and Gynaecology, of a tertiary care centre, from the period October 2015 to October 2017. Data was collected from all 130 women attending the antenatal clinic of tertiary care hospital and ward admission and all details such as demographic details, obstetrics examination, and all clinical findings were noted and from that made results.  Result: After applying inclusion and exclusion criteria all 130 women were observed in this study. Among 130 women 47 were diagnosed with preeclampsia. Mainly primigravida women were diagnosed with preeclampsia in the 21-25 years group. Among 47 preeclampsia women, 39 women had a BMI of 19-25 kg/m2. Thirty-two of 47 (68.09%) women were diagnosed with preeclampsia around 36-39 weeks. Among all preeclampsia, 28 women out of 47 (59.5%) women delivered babies vaginally, 18 of 47 (38.3%) women delivered through cesarean section, and one of 47 (2.13%) underwent preterm vaginal delivery. In preeclampsia, women\'s babies were delivered mostly (25/47, 53.19%) ≤2.5 kg weight and only one baby was shifted to NICU because of low birth weight. Preeclampsia increases maternal mortality and morbidity but in this study mortality was not done because our hospital is a tertiary care center with all ICU (intensive care unit) and NICU setup.
    CONCLUSIONS: Preterm births and cesarean deliveries were the mild to severe outcomes that were noted. ICU and NICU hospitalizations as a result of severe complications place a heavy demand on medical facilities. There are firm guidelines for the management of pregnancy-induced hypertension and its complications. For appropriate management, there is careful consideration of various factors, and individual case studies are required.
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  • 文章类型: Journal Article
    目的:探讨双胎妊娠早期子宫动脉多普勒与妊娠高血压疾病的关系。
    方法:这是一项在里斯本市中心大学医院中心进行的双胎妊娠的回顾性队列研究,葡萄牙,2010年1月至2022年12月。测定妊娠早期子宫动脉搏动指数(UtA-PI),并比较双胎妊娠(n=454)和单胎妊娠(n=908),与母体和妊娠特征相匹配。母亲特征和平均UtA-PI分析了胎龄,出生体重,妊娠期高血压,早发型和晚发型先兆子痫,HELLP(溶血,肝酶升高,低血小板)综合征,和早产。使用单变量和多变量逻辑回归模型。
    结果:双胎双胎的早孕平均UtA-PI明显低于单胎(P<0.001)。研究双胞胎妊娠高血压疾病,包括390例怀孕:311(79.7%)双胎和79(20.3%)单绒毛膜双胞胎。观察到的早发型和晚发型先兆子痫的发生率,妊娠期高血压,HELLP综合征为1.0%,4.4%,7.4%,和1.5%,分别。我们使用UtA-PI第90百分位数的双胞胎对早发型先兆子痫的检出率为100%。然而,当单例参考文献被考虑时,检出率下降到50%。在第95百分位或以上的UtA-PI与32周前早产的几率增加相关(调整后的优势比4.1,95%置信区间1.0-16.7,P=0.043)。
    结论:除非存在高血压疾病的其他主要危险因素,UtA-PI低的女性可能不会从阿司匹林预防中受益。仍然建议密切监测所有双胎妊娠的高血压疾病。
    OBJECTIVE: To determine the association of first-trimester uterine artery Doppler with hypertensive disorders of pregnancy in twin pregnancies.
    METHODS: This was a retrospective cohort study of twin pregnancies followed at the University Hospital Center of Central Lisbon, Portugal, between January 2010 and December 2022. First-trimester uterine artery pulsatility index (UtA-PI) was determined and compared between twin pregnancies (n = 454) and singleton pregnancies (n = 908), matched to maternal and pregnancy characteristics. Maternal characteristics and mean UtA-PI were analyzed for gestational age, birth weight, gestational hypertension, early- and late-onset pre-eclampsia, HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome, and preterm birth. Univariable and multivariable logistic regression models were used.
    RESULTS: The mean first-trimester UtA-PI was significantly lower in dichorionic twins than in singletons (P < 0.001). To study hypertensive disorders of pregnancy in twins, 390 pregnancies were included: 311 (79.7%) dichorionic and 79 (20.3%) monochorionic twins. The observed rates of early- and late-onset pre-eclampsia, gestational hypertension, and HELLP syndrome were 1.0%, 4.4%, 7.4%, and 1.5%, respectively. We achieved a 100% detection rate for early-onset pre-eclampsia using the UtA-PI 90th centile for twins. However, when singleton references were considered, the detection rate decreased to 50%. UtA-PI at or above the 95th centile was associated with increased odds for preterm birth before 32 weeks (adjusted odds ratio 4.1, 95% confidence interval 1.0-16.7, P = 0.043).
    CONCLUSIONS: Unless other major risk factors for hypertensive disorders are present, women with low UtA-PI will probably not benefit from aspirin prophylaxis. Close monitoring of all twin pregnancies for hypertensive disorders is still recommended.
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  • 文章类型: Journal Article
    目的:评估明显的蛋白尿与具有严重特征的先兆子痫风险之间的可能关联,由美国妇产科学院定义。
    方法:这项回顾性研究包括2017年至2022年在一所大学附属医院记录的数据。包括妊娠24周或超过24周的蛋白尿(24小时尿液中蛋白质水平>300mg)和最初48小时内血压正常的妇女。比较轻度蛋白尿(300-1000mg/24h)和明显蛋白尿(≥1000mg/24h)的妇女的产科和新生儿结局。
    结果:在有明显蛋白尿的妇女(n=48)与有轻度蛋白尿的妇女(n=108)相比,先兆子痫的发病率较高(50.0%vs.22.2%,p=0.001)和具有严重特征的先兆子痫(18.8%vs.2.8%,p<0.001)。在调整了产妇年龄的多变量分析中,原语奇偶校验,多胎妊娠,尿酸水平>6mg/dL和阿司匹林治疗,显著的蛋白尿是具有重度特征的子痫前期(校正比值比[aOR]=10.2,置信区间[CI]95%1.9-54.0,p=0.007)和小于胎龄儿(aOR=2.4,95%CI1.02-5.6,p=0.001)的危险因素.在与轻度蛋白尿相比明显的女性中,引产率也较高(58.3%vs.25.9%,p<0.001),指示早产(41.7%与25.0%,p=0.04)和新生儿重症监护病房(44.1%vs.25.8%,p=0.017)。
    结论:与轻度孤立性蛋白尿相比,具有明显特征的女性表现出更高的先兆子痫和分娩小于胎龄新生儿的风险。
    OBJECTIVE: To assess a possible association between marked proteinuria and the risk of preeclampsia with severe features, as defined by the American College of Obstetricians and Gynecologists.
    METHODS: This retrospective study included data recorded at a tertiary university-affiliated hospital between 2017 and 2022. Women at or beyond 24 weeks of gestation with proteinuria (protein levels > 300 mg in a 24 h urine collection) and normal blood pressure during the initial 48 h of admission were included. Obstetrical and neonatal outcomes were compared between women with mild proteinuria (300-1000 mg/24 h) and marked proteinuria (≥ 1000 mg/24 h).
    RESULTS: Among the women with marked proteinuria (n = 48) compared to those with mild proteinuria (n = 108), the incidences were higher of preeclampsia (50.0% vs. 22.2%, p = 0.001) and of preeclampsia with severe features (18.8% vs. 2.8%, p < 0.001). In multivariate analysis that adjusted for maternal age, primiparity, multiple pregnancy, uric acid level > 6 mg/dL and aspirin treatment, marked proteinuria was a risk factor for preeclampsia with severe features (adjusted odds ratio [aOR] = 10.2, confidence interval [CI] 95% 1.9-54.0, p = 0.007) and for small-for-gestational-age infants (aOR = 2.4, 95% CI 1.02-5.6, p = 0.001). Among women with marked compared to mild proteinuria, rates were also higher of labor induction (58.3% vs. 25.9%, p < 0.001), indicated preterm delivery (41.7% vs. 25.0%, p = 0.04) and admission to the neonatal intensive care unit (44.1% vs. 25.8%, p = 0.017).
    CONCLUSIONS: Women with marked compared to mild isolated proteinuria showed higher risk of developing preeclampsia with severe features and of delivering small-for-gestational-age neonates.
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  • 文章类型: Journal Article
    在少数具有多种原因的妊娠中注意到肝功能异常。其中一小部分发展为严重的肝损伤并发展为急性肝衰竭(ALF)。妊娠ALF的病因有一组离散的,全面的了解将有助于紧急评估。某些疾病,如妊娠急性脂肪肝,溶血,肝酶升高,低血小板(HELLP)综合征和先兆子痫继发于妊娠状态,可表现为ALF。快速和有针对性的评估与病因学特定管理的紧急机构,尤其是妊娠相关肝病患者的紧急分娩,是避免孕产妇死亡的关键.怀孕,和胎儿的生命一样,在评估中赋予了另一层并发症,这些ALF患者的预后和管理。最佳管理通常需要在设备齐全的中心采用多学科方法。在这次审查中,我们讨论评估,妊娠ALF患者的评估和管理,关注妊娠相关肝病的治疗方法。
    Liver function abnormalities are noted in a minority of pregnancies with multiple causes for the same. A small proportion of these develop severe liver injury and progress to acute liver failure (ALF). There is a discrete set of etiology for ALF in pregnancy and comprehensive understanding will help in urgent evaluation. Certain diseases such as acute fatty liver of pregnancy, hemolysis, elevated liver enzyme, low platelet (HELLP) syndrome and pre-eclampsia are secondary to pregnant state and can present as ALF. Quick and targeted evaluation with urgent institution of etiology-specific management, especially urgent delivery in patients with pregnancy-associated liver diseases, is the key to avoiding maternal deaths. Pregnancy, as also the fetal life, imparts a further layer of complication in assessment, prognosis and management of these sick patients with ALF. Optimal management often requires a multidisciplinary approach in a well-equipped centre. In this review, we discuss evaluation, assessment and management of pregnant patients with ALF, focussing on approach to pregnancy-associated liver diseases.
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  • 文章类型: Journal Article
    子痫前期是一种以蛋白尿为特征的进行性多系统疾病,严重的器官损伤,和新发高血压.HELLP综合征(溶血,肝酶升高,低血小板),导致严重的肝或肾损伤,播散性凝血,和严重的癫痫发作。这项研究旨在检查ADAMTS13,冯维勒布兰德,和补体系统在子痫前期/HELLP综合征发病机制中的作用。我们研究了30名高加索先兆子痫孕妇和15名健康妊娠的对照组。ADAMTS13和补体调节基因的基因测序(MiniSeq系统,Illumina)进行。改良的火腿测试用于检查补体激活,ADAMTS13活性,vonWillebrand抗原(vWFAG)水平,测定可溶性C5b-9水平。先兆子痫患者的ADAMTS13活性降低,C5b-9水平升高。vWFAG与ADAMTS13活性显著相关(r=0.497,p=0.003)。在ADAMTS13,C3,血栓调节蛋白,CFB,CFH,MBL2,和,最后,MASP2.部分先兆子痫孕妇显示ADAMTS13活性下降,与vWFAG水平相关。这些患者还表现出升高的补体激活和调节基因中的高风险遗传变异。需要进一步的研究来确定这些因素是否可以作为可靠的生物标志物。
    Preeclampsia is a progressive multi-systemic disorder characterized by proteinuria, critical organ damage, and new-onset hypertension. It can be further complicated by HELLP syndrome (hemolysis, elevated liver enzymes, low platelets), resulting in critical liver or renal damage, disseminated coagulation, and grand mal seizures. This study aimed to examine the involvement of ADAMTS13, von Willebrand, and the complement system in the pathogenesis of preeclampsia/HELLP syndrome. We studied 30 Caucasian preeclamptic pregnant women and a control group of 15 healthy pregnancies. Genetic sequencing of ADAMTS13 and complement regulatory genes (MiniSeq System, Illumina) was performed. The modified Ham test was used to check for complement activation, ADAMTS13 activity, von Willebrand antigen (vWFAg) levels, and soluble C5b-9 levels were measured. Patients with preeclampsia had a decreased ADAMTS13 activity and increased C5b-9 levels. The vWFAg was significantly correlated with ADAMTS13 activity (r = 0.497, p = 0.003). Risk-factor variants were found in the genes of ADAMTS13, C3, thrombomodulin, CFB, CFH, MBL2, and, finally, MASP2. A portion of pregnant women with preeclampsia showed a decline in ADAMTS13 activity, correlated with vWFAg levels. These patients also exhibited an elevated complement activation and high-risk genetic variants in regulatory genes. Further research is needed to determine if these factors can serve as reliable biomarkers.
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  • 文章类型: Journal Article
    硫酸镁在治疗子痫中的作用是公认的。该药物被证明优于其他抗惊厥药,可以减少子痫妇女反复惊厥的发生率。此外,硫酸镁已被用于具有不同严重特征的先兆子痫妇女。然而,尽管有这些建议,许多临床医生对硫酸镁的使用仍然没有信心,即使在高发先兆子痫和不可接受的孕产妇死亡率的环境中。这篇综述带来了基础科学和临床信息,以支持建议,鼓励临床医生对所有重度子痫前期患者使用硫酸镁。不仅仅是有神经症状的女性。此外,还介绍了硫酸镁在麻醉和胎儿神经保护方面的其他益处。最后,一项全面的算法提出了治疗34~36+6周重度子痫前期患者的建议.
    The role of magnesium sulfate for treatment of eclampsia is well established. The medication proved to be superior to other anticonvulsants to reduce the incidence of recurrent convulsions among women with eclampsia. Additionally, magnesium sulfate has been indicated for women with preeclampsia with different severe features. However, despite these recommendations, many clinicians are still not confident with the use of magnesium sulfate, even in settings with high incidence of preeclampsia and unacceptable rates of maternal mortality. This review brings basic science and clinical information to endorse recommendations to encourage clinicians to use magnesium sulfate for patients with all severe features of preeclampsia, not only for women with neurological symptoms. Additionally, other benefits of magnesium sulfate in anesthesia and fetal neuroprotection are also presented. Finally, a comprehensive algorithm presents recommendations to manage patients with preeclampsia with severe features between 34 and 36+6 weeks.
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  • 文章类型: Case Reports
    肝破裂是重度子痫前期的罕见并发症。在孕妇中存在伴有血液动力学代偿失调的腹痛的情况下,需要高度怀疑。肝破裂构成需要立即干预的医疗紧急情况,通常在其他医学学科的支持下,在高度专业化的医院环境中。未破裂的肝血肿可以保守治疗。立即分娩和肝脏的手术修复对于母体生存是必要的。妊娠期自发性肝破裂通常无法识别,高度致命,并且尚未完全理解,文献中报道的病例很少。因此,我们目前有2例HELLP(溶血,肝酶升高,和低血小板)综合征伴肝破裂,强调他们的临床表现和治疗方法。
    Hepatic rupture is a rare complication of severe preeclampsia. A high index of suspicion is required in the presence of abdominal pain accompanied by hemodynamic decompensation in a pregnant woman. Hepatic rupture constitutes a medical emergency that demands immediate intervention, often with the support of other medical disciplines, in a highly specialized hospital setting. Unruptured hepatic hematomas can be managed conservatively. Immediate delivery and surgical repair of the liver are necessary for maternal survival. Spontaneous liver rupture in pregnancy is often unrecognized, highly lethal, and not completely understood with few cases having been reported in the literature. Therefore, we present two cases of HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome with hepatic rupture, emphasizing their clinical presentation and therapeutic approaches.
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  • 文章类型: Case Reports
    溶血,肝酶水平升高,低血小板计数(HELLP)综合征是妊娠期高血压疾病最严重的并发症之一。在22孕周(GWs)之前发生的HELLP综合征极为罕见,患者普遍表现出潜在的母体疾病或胎儿异常。这里,我们报道一例孕妇,在20个GWs时出现HELLP综合征,但没有任何明显的潜在母体疾病或胎儿异常.一名38岁的孕妇因高血压于19+5/7GWs从另一家医院转诊到神户大学医院,蛋白尿,全身性水肿,胎儿生长受限.根据密西西比州分类,她在202/7GWs时被诊断为部分HELLP综合征。病人按照密西西比州的方案进行管理,包括静脉注射地塞米松,硫酸镁,和抗高血压药物。她接受了强化血压和实验室数据监测,使用动脉管线和额外的治疗,包括血小板输注,静脉内输注触珠蛋白,和人心房利钠肽.妊娠以20+3/7GWs的诱导分娩结束,产后10天出院,无并发症。我们进行了诊断潜在疾病的实验室测试,但未发现明显的潜在疾病。该报告表明,根据密西西比州协议,在22GWs之前对HELLP综合征患者进行早期和强化治疗可能使临床医生能够在没有母体并发症的情况下完成终止妊娠,并为围产期医学的临床医师提供有用的信息。
    Hemolysis, elevated liver enzyme levels, and low platelet count (HELLP) syndrome is one of the most severe complications of hypertensive disorders of pregnancy. HELLP syndrome occurring before 22 gestational weeks (GWs) is extremely rare, and patients prevalently exhibit underlying maternal diseases or fetal abnormalities. Here, we report the case of a pregnant woman who had HELLP syndrome at 20 GWs without any obvious underlying maternal diseases or fetal abnormalities. A 38-year-old pregnant woman was referred to Kobe University Hospital from another hospital at 19 + 5/7 GWs for hypertension, proteinuria, generalized edema, and fetal growth restriction. She was diagnosed with partial HELLP syndrome according to the Mississippi classification at 20 + 2/7 GWs. The patient was managed following the Mississippi protocol, including intravenous dexamethasone, magnesium sulfate, and antihypertensive drugs. She received intensive blood pressure and laboratory data monitoring using an arterial line and additional treatments, including platelet transfusion, intravenous haptoglobin infusion, and human atrial natriuretic peptide. The pregnancy ended in an induced delivery at 20 + 3/7 GWs, and she was discharged without complications 10 days postnatal. We performed laboratory tests for diagnosing underlying diseases but identified no obvious underlying diseases. This report indicates that early and intensive treatment of patients with HELLP syndrome occurring before 22 GWs according to the Mississippi protocol may enable clinicians to complete pregnancy termination without maternal complications and provide useful information to clinical practitioners in perinatal medicine.
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