HELLP综合征16例临床分析

目的探讨子痫前期并发HELLP综合征的发病率、诊断、治疗及预后。方法对16例子痫前期并发HELLP综合征患者的临床资料进行回顾性分析。结果完全性HELLP综合征14例，部分性HELLP综合征2例。治疗方法为严密监护母儿情况下积极治疗子痫前期，早期使用糖皮质激素，适时终止妊娠。主要并发症为DIC、肝被膜下血肿、胎盘早剥、肺水肿和急性肾功能衰竭等。16例患者中死亡1例，围产儿死亡3例，死亡率分别为6．25％及18．75％。结论HELLP综合征是子痫前期的一种严重威胁母儿安全的并发症，早期诊断、综合性治疗、适时终止妊娠，可改善HELLP综合征患者的预后。     

Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is more common in women, and commonly occurs during pregnancy and the immediate postpartum period. An important clinical issue is the distinction of TTP-HUS from the more common obstetric complications, preeclampsia and HELLP syndrome (hemolysis, elevated liver function tests, low platelets). Clinical suspicion of TTP-HUS requires urgent intervention with plasma exchange treatment, a procedure with substantial risk, while preeclampsia and HELLP syndrome typically resolve spontaneously following delivery. Since clinical features of these syndromes can be similar, especially if preeclampsia becomes severe or if seizures (defining eclampsia) occur, the differential diagnosis may be arbitrary. This review addresses the evaluation and management of these syndromes and describes a clinical approach for determining when plasma exchange is appropriate.     

Leukocytosis is proportional to HELLP syndrome severity: evidence for an inflammatory form of preeclampsia

BACKGROUND: We investigated the possibility that HELLP syndrome is in part a systemic inflammatory response. METHODS: We evaluated total white blood cell (WBC) counts of all patients with severe preeclampsia with and without HELLP syndrome admitted to our hospital between 1995 and 1997. Patients were grouped by diagnosis and timing of platelet nadir. Analysis of variance and regression analysis were used for data analysis. RESULTS: Of 177 patients, 91 had HELLP syndrome, and 86 had severe preeclampsia alone. The WBC counts were significantly higher in patients with HELLP syndrome (12.5 +/- .442 x 10(9)/L) than in patients with severe preeclampsia (10.3 +/- .288 x 10(9)/L). Regression analysis showed that platelet counts varied inversely with WBC counts. Also, patients with class I HELLP syndrome had significantly higher WBC counts than patients with other classes of HELLP syndrome. CONCLUSION: The finding of an association between increasing leukocytosis and worsening thrombocytopenia early in the course of HELLP syndrome supports the hypothesis that it may represent an inflammatory process.     

Le HELLP syndrome

The HELLP syndrome associates hemolysis, elevated liver enzymes and a low platelet count. It makes part of preeclampsia. Management of HELLP syndrome is still controversial. In order to improve maternal and foetal prognosis, two approaches can be considered: immediate termination of pregnancy with a risk of foetal complications due to prematurity or conservative treatment with maternal risk of complications related to hemorragic disorders. So, treatment choice needs to take into account the maternal and fetal risk/benefit ratio.     

Imaging of subcapsular hepatic and renal hematomas in pregnancy complicated by preeclampsia and the HELLP syndrome

The purpose of this report is to provide an illustrative case of spontaneous hepatic and renal hematomas that occurred during a pregnancy complicated by preeclampsia and the HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. The sonographic and computed tomographic findings included intrahepatic, subcapsular hepatic, and extracapsular perihepatic hematomas in addition to a large subcapsular renal hematoma. Since hepatic and renal hematomas that occur in association with preeclampsia and the HELLP syndrome are rare but potentially life‐threatening complications, prompt laboratory and radiologic evaluations are essential and may reduce the associated morbidity and mortality. © 1999 John Wiley & Sons, Inc. J Clin Ultrasound 27:35–40, 1999.     

Increasing maternal weight: a risk factor for preeclampsia/eclampsia but apparently not for HELLP syndrome

BACKGROUND: Maternal obesity is a risk factor for severe preeclampsia. We sought to ascertain whether a similar relationship exists between maternal weight and HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) as an atypical form of severe preeclampsia. METHODS: In this retrospective investigation, 434 patients with HELLP syndrome were assigned to one of four study groups according to maternal weight and were analyzed in relation to selected maternal and perinatal data reflective of disease severity. RESULTS: We found no significant associations between maternal weight and parameters of HELLP syndrome severity, race, delivery mode, gestational age, or perinatal outcome. Significantly associated with increasing maternal weight were maternal age, parity, admission mean arterial pressure, peak peripartum systolic blood pressures, concurrent essential hypertension, and the interval between admission and delivery. Inversely associated were eclampsia and the interval between delivery and discharge. CONCLUSIONS: Severity and complications attendant with HELLP syndrome appear unrelated to maternal weight. Paradoxically, eclampsia occurs most commonly in the lighter gravida with HELLP syndrome.     

Thrombotic thrombocytopenic purpura and its look-alikes

The thrombotic thrombocytopenic purpura syndrome (TTP) can be mistaken for a number of other conditions, and it is important to diagnose correctly and treat appropriately. We describe the features of TTP that can help make a positive diagnosis and other conditions in the differential diagnosis with symptoms that can overlap and mimic those of TTR. We discuss TTP and its variants, hemolytic uremic syndrome, disseminated intravascular coagulation, heparin-induced thrombocytopenia, antiphospholipid syndrome, Evans syndrome, preeclampsia/eclampsia, HELLP syndrome, acute fatty liver of pregnancy, and multiorgan failure.     

Association of HELLP syndrome with autoimmune antibodies and glucose intolerance

OBJECTIVE: HELLP syndrome is a severe form of preeclampsia, characterized by hemolysis (H), elevated liver enzymes (EL), and low platelets (LP), whose pathogenesis is unclear. Autoimmunity is thought to play an important role. After the observation of development of type 1 diabetes in a patient with HELLP syndrome, we assumed a possible disease association based on autoimmune reactions. RESEARCH DESIGN AND METHODS: We examined 70 women with HELLP syndrome for the presence of autoimmune markers and glucose intolerance. Free thyroxine, triiodothyronine, thyroid-stimulating hormone, anti-thyroglobulin antibodies, thyroperoxidase antibodies, thyrotropin receptor antibodies, antinuclear antibodies (ANAs) and anti-DNA, islet cell antibodies, GADA, an oral glucose tolerance test, and HbA1c were determined postpartum. Patients with positive autoimmune markers or glucose intolerance were prospectively followed and repeated testing was performed. There were 60 women with a normal course of pregnancy matched for age, BMI, and number of pregnancies, which served as a control group. RESULTS: From the HELLP patients, 22 (31%) compared with only 6 (10%) control subjects had autoimmune antibodies (P < 0.01). There were 16 HELLP patients (23%) who exhibited only 1 kind of autoantibody (5 ANA, 9 thyroid antibodies, and 2 GADA), whereas in 6 HELLP patients (8.5%) 2 different antibodies were found. In all but 4 patients of the study group, these antibodies disappeared during 3 +/- 1.5 years of follow-up. Glucose intolerance was detected in 22 (31%) of the HELLP patients, 17 of them had impaired glucose tolerance (IGT), and 5 had diabetes, whereas only 4 subjects (6.5%) with IGT at postpartum were found in the control group (P < 0.01). During the follow-up, 2 HELLP patients were still diabetic and another 2 HELLP patients (1 GADA positive) had IGT versus 1 control subject. CONCLUSIONS: Our data give evidence that HELLP syndrome is associated with various autoimmune antibodies and glucose intolerance. Because glucose intolerance and/or autoimmune markers persisted during long-term follow-up in 6 patients with HELLP syndrome versus 1 in the control group, it may become advisable to reexamine patients with HELLP syndrome for detection of diabetes and autoimmune disorders.     

Critical care of the obstetric patient

The obstetric patient poses exceptional challenges in the intensive care unit. Knowledge of the physiologic changes of pregnancy and specific pregnancy-related disorders is necessary for optimal management. Intensive care unit diagnoses may include preeclampsia, including the HELLP syndrome, pulmonary embolic disease, amniotic fluid embolism, status asthmaticus, respiratory infection, the acute respiratory distress syndrome, and sepsis. The management of mechanical ventilation is based on principles of avoiding lung injury, and hypercapnia may be tolerated even during the pregnancy. When the clinician is faced with the extraordinary instance of cardiopulmonary arrest, perimortem cesarean delivery must be considered to improve the potential for maternal and fetal survival.     

Hepatic disorders severely affected by pregnancy: medical and obstetric management

Hepatic disorders severely affected by pregnancy include choledochal cysts that can be compressed by the gravid uterus and potentially rupture; hepatic adenomas that exhibit accelerated growth because of hyperestrogenemia during pregnancy; acute intermittent porphyria that is exacerbated by increased female sex hormones during pregnancy; splenic artery aneurysms that can rupture during pregnancy because of compression by the gravid uterus; Budd-Chiari syndrome that is promoted by hyperestrogenemia; and hepatitis E and herpes simplex hepatitis that are particularly severe during pregnancy. Hepatic disorders unique to pregnancy include intrahepatic cholestasis of pregnancy; acute fatty liver of pregnancy; preeclampsia and eclampsia; and hemolysis, elevated liver function tests, and low platelet count (HELLP) syndrome. Most disorders uniquely related to pregnancy are treated by prompt fetal delivery as soon as the fetus is sufficiently mature.     

影响子痫前期发病的孕检因素分析

目的探讨孕检因素对子痫前期发病的影响。方法选取于该院产科住院治疗的妊娠高血压孕妇160例,其中规律孕检妇女80例(规律组),非规律孕检妇女80例(非规律组),分析两组患者临床症状及与子痫前期发生相关的孕检因素。结果非规律孕检组的孕妇发生子痫前期、子痫、胎盘早剥、视神经炎、HELLP综合征和产后出血的概率高于规律孕检组(P0.05)。非规律组中,轻度子痫前期者与重度子痫前期者比较,首次孕检孕周更早、孕检次数更多、间隔时间更短,差异均有统计学意义(P0.05)。将上述孕检因素进行多元Logistic回归分析,结果显示,首次孕周、孕检次数和间隔时间是重度子痫前期发病的独立影响因素。结论非规律产检会增加患者不良妊娠结局和产后并发症,而且非规律产检者重度子痫前期的发生与首次产检孕周、孕检次数和间隔时间有关,所以坚持规律产检对顺利分娩具有重要意义。     

Hypertensive disorders of pregnancy

The National High Blood Pressure Education Program Working Group on High Blood Pressure in Pregnancy has defined four categories of hypertension in pregnancy: chronic hypertension, gestational hypertension, preeclampsia, and preeclampsia superimposed on chronic hypertension. A maternal blood pressure measurement of 140/90 mm Hg or greater on two occasions before 20 weeks of gestation indicates chronic hypertension. Pharmacologic treatment is needed to prevent maternal end-organ damage from severely elevated blood pressure (150 to 180/100 to 110 mm Hg); treatment of mild to moderate chronic hypertension does not improve neonatal outcomes or prevent superimposed preeclampsia. Gestational hypertension is a provisional diagnosis for women with new-onset, nonproteinuric hypertension after 20 weeks of gestation; many of these women are eventually diagnosed with preeclampsia or chronic hypertension. Preeclampsia is the development of new-onset hypertension with proteinuria after 20 weeks of gestation. Adverse pregnancy outcomes related to severe preeclampsia are caused primarily by the need for preterm delivery. HELLP (i.e., hemolysis, elevated liver enzymes, and low platelet count) syndrome is a form of severe preeclampsia with high rates of neonatal and maternal morbidity. Magnesium sulfate is the drug of choice to prevent and treat eclampsia. The use of magnesium sulfate for seizure prophylaxis in women with mild preeclampsia is controversial because of the low incidence of seizures in this population.     

重度子痫前期合并HELLP综合征产妇行剖宫产的术后护理

目的:总结重度子痫前期合并HELLP综合征产妇剖宫产术后的护理方法。方法:选取我院在2010年6月~2013年6月收治的20例行剖宫产的重度子痫前期合并HELLP综合征产妇作为研究对象,对本组产妇术后的护理方法进行回顾性分析。结果:20例产妇中,18例成功娩出活胎,胎死宫内2例,产妇中无死亡病例,均未发生产后子痫。10例因新生儿低体重、早产转至新生儿监护室,母婴分离;8例母婴同室。剖宫产后,并发产后出血2例,并发急性肾功能衰竭2例,经对症处理后,症状均有所好转。结论:在剖宫产术后,严密观察产妇病情变化,做好血压控制、出血预防措施,对于预防重度子痫前期合并HELLP综合征产妇术后并发症的发生具有重大意义。     

Plasma exchange for preeclampsia: III. Immediate peripartal utilization for selected patients with hellp syndrome

OBJECTIVE: To explore the potential efficacy of plasma exchange as an ancillary interventive therapeutic tool immediately before or after delivery in the patient with severe preeclampsia/eclampsia and hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. STUDY DESIGN: Two gravidas with complicated severe preeclampsia/eclampsia/HELLP syndrome were treated emergently in the immediate peripartal period with single-volume plasma exchange and fresh frozen plasma fluid replacement using the IBM 2997 Cell Separator. RESULTS: Despite multiple platelet unit infusions, one primigravida in active labor at 5 cm cervical dilatation and 39 weeks' gestation remained at a platelet count of 14,000/μL and began to ooze from her gums. A second primigravida remained obtunded, oliguric, and thrombocytopenic with epistaxis and hematuria following cesarean delivery and platelet transfusions. A single expedited 3-liter plasma exchange procedure reversed the rapidly deteriorating clinical situation for each patient and accelerated recovery from HELLP syndrome. Both patients and progeny suffered no permanent sequelae. CONCLUSION: Based on our experience, we believe that the therapeutic modality of plasma exchange with fresh frozen plasma can be employed effectively for the pregnant patient with severe atypical HELLP syndrome that progressively worsens during labor or the early puerperium despite the use of conventional transfusion therapy.     

Vascular endothelial growth factor (VEGF) polymorphisms in HELLP syndrome patients determined by quantitative real-time PCR and melting curve analyses

BACKGROUND: The vascular endothelial growth factor (VEGF) has a critical role in vasculogenesis and vascular permeability in several diseases including preeclampsia. There are at least 30 single nucleotide polymorphic (SNP) places on this gene. VEGF G+405C, C-2578A and C-460T SNPs are known to be related to VEGF production. VEGF polymorphisms were studied in preeclampsia, but not in HELLP syndrome. Therefore, we decided to determine the allele and genotype frequencies of VEGF G+405C, C-460T and C-2578A SNPs in healthy pregnant women and HELLP syndrome patients. METHODS: The authors introduced a quantitative real-time PCR method for the determination of the three VEGF SNPs. Blood samples were collected from 71 HELLP syndrome patients and 93 healthy controls. DNA was isolated by using silica adsorption method. The SNPs were determined by quantitative real-time PCR and melting curve analysis using LightCycler. RESULTS: There were significant differences in the allele and genotype frequencies of VEGF C-460T SNP between the two study groups. The T allele was present in 71.1% in the HELLP group, while in 53.8% in the controls (p=0.0014). The TT genotype occurred significantly more frequently in the HELLP group than in the control group (45.1% vs. 21.5%; p (for genotype frequencies)=0.0011). The TT genotype carriers had an increased risk of HELLP syndrome, which was independent of maternal age and primiparity (adjusted odds ratio (OR)=3.03, 95% confidence interval (CI)=1.51-6.08; p=0.002). Although the VEGF G+405C allele and genotype distributions did not differ significantly between the two groups, the CC genotype carriers were also found to have an increased risk for HELLP syndrome after adjustment for maternal age and primiparity (adjusted OR=3.67, 95% CI=1.05-12.75; p=0.041). The VEGF C-2578A SNP was not associated with HELLP syndrome. CONCLUSIONS: The quantitative real-time PCR combined with melting curve analyses is a fast and reliable method for the determination of VEGF SNPs. We found that the VEGF -460TT and +405CC genotype carriers have an increased risk of HELLP syndrome. As these two SNPs were previously observed to be related to production of the VEGF protein, we suppose that these VEGF polymorphisms -- interacting with other genetic and environmental factors - could play a role in the development of HELLP syndrome.     

HELLP syndrome and cholecystitis: Case report and review of the literature

A case of the HELLP syndrome is reported that was initially diagnosed as cholecystitis. Much overlap exists between the two diagnoses, and the emergency physician must be aware of the important differences between them. Because the HELLP syndrome and preeclampsia may occur in both the second and third trimesters, they represent serious diagnoses that must be considered when evaluating a pregnant patient with right upper quadrant abdominal pain.     

Plasma exchange for preeclampsia: II. Unsuccessful antepartum utilization for severe preeclampsia with or without hellp syndrome

OBJECTIVE: To explore the efficacy of plasmapheresis/plasma exchange as the primary therapy to arrest and reverse the progression of severe preeclampsia with or without HELLP syndrome in order to postpone delivery and improve perinatal outcome in very preterm pregnancies. STUDY DESIGN: In this case series of patients managed over a 4-year period from 1984 to 1987, seven gravidas with severe preterm preeclampsia underwent 1-2 plasmaphereses/plasma exchange procedures using the IBM 2997 Cell Separator with continuous electronic fetal heart rate monitoring (n = 7 patients) and central cardiovascular monitoring (n = 3 patients). RESULTS: The seven patients (one with HELLP syndrome, six without HELLP) presented between 24 and 30 weeks gestation and, despite plasmapheresis/plasma exchange, the severity of each study subject's preeclampsia persisted without clinically significant improvement. Maternal-fetal deterioration required cesarean delivery in all cases within 48 (in four patients within <36) hours of therapy. No clinically significant adverse effect of plasma exchange therapy was recorded during cardiovascular and laboratory monitoring; two fetuses developed repetitive late decelerations during exchange despite adequate maternal fluid preload. The only patient with HELLP syndrome developed eclampsia as her third plasma exchange within 25 hours was being initiated. Significant problems with fluid retention and displacement (variable amounts of pulmonary edema, pleural effusions, large volume ascites) were encountered in all patients. Four neonates died (24-27 weeks/438-820 g) and three survived intact (740, 950, and 1,280 g). One mother (case 5) developed end-stage renal disease 21 months postpartum. CONCLUSIONS: The application of plasmapheresis/plasma exchange therapy as described in order to prolong very preterm pregnancies in the undelivered patient with severe preeclampsia/eclampsia with or without HELLP syndrome did not produced encouraging results. Patients in general were exposed to additional medical and surgical risk without a corresponding improvement in perinatal outcome.     

重度妊高征病人合并HELLP综合征行剖宫产的麻醉处理

目的探讨重度妊娠高血压综合征(妊高征)合并HELLP综合征行剖宫产的麻醉处理。方法对2例HELLP综合征行剖宫产麻醉处理进行回顾性总结、分析。结果2例患者术中麻醉基本平稳,术后均痊愈出院。结论掌握好手术指征,及时行剖宫产终止妊娠,术中选择全身麻醉,对重度妊高征合并HELLP综合征患者的痊愈起到了重要作用。     

妊娠合并血小板减少的病因及母婴结局分析

目的 探讨妊娠合并血小板减少（PT）的病因及母婴结局。方法 收集228例PT患者,其中血小板（51 ~ 100）×10⁹/L为轻度组,血小板（30 ~ 50）×10⁹/L为中度组,血小板< 30×10⁹/L为重度组,比较3组患者的病因和母婴结局的差异。结果 228例PT孕妇中,轻度血小板减少159例（69.8%）,中度血小板减少33例（14.5%）,重度血小板减少36例（15.8%）。主要病因有妊娠相关性血小板减少症（63.6%）、特发性血小板减少性紫癜（11.8%）、HELLP综合征（3.9%）、SLE（3.9%）和子痫前期-子痫（2.2%）。轻度组、中度组和重度组的妊娠丢失、早产、产后出血、新生儿血小板减少的发生率及分娩孕周比较差异均有统计学意义（P均< 0.05）,其中重度组的妊娠丢失率、早产率、产后出血率均高于轻度组,分娩孕周短于轻度组（P均< 0.017）。结论 PT的病因复杂多样,病因多见妊娠相关性血小板减少症、特发性血小板减少性紫癜、HELLP综合征、SLE和子痫前期-子痫。血小板< 30×10⁹/L的PT患者发生妊娠丢失、早产、产后出血的概率明显增加。