Hemolysis, Elevated Liver Enzymes, And Low Platelets Syndrome Research Articles

Transfusion-Related acute lung injury in a patient with hellp syndrome: A case report

Transfusion-related acute lung injury (TRALI) is a serious adverse reaction associated with the transfusion of plasma-containing blood components, and presents as hypoxemia and non-cardiogenic pulmonary oedema within 6 hrs of transfusion. Here we are reporting a case of TRALI in 24-year-old pregnant Female presented with HELLP (haemolysis, elevated liver enzymes, and low platelet count) syndrome, an emergency caesarean section delivery was performed, and blood was transfused. In such cases, clinicians should strictly monitor the patient's condition at least during the 6 hrs while the patient receives blood transfusion, and should suspect TRALI. The patient's rapid clinical deterioration with falling O2 saturation and other respiratory symptoms after transfusion supports the diagnosis of TRALI.

Comparison of p38 MAPK, soluble endoglin and endothelin-1 level in severe preeclampsia and HELLP syndrome patients

Objective: To compare the levels of p38 mitogen-activated protein kinase (MAPK), soluble endoglin and endothelin-1 in the serum of patients with severe preeclampsia, hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome and normal pregnancies.Methods: This study was an observational analytic cross-sectional study performed at Wahidin Sudirohusodo Hospital, Makassar, Indonesia, in the period of 5th February 2016 to 20th January 2017. P38 MAPK, soluble endoglin and endothelin-1 levels of patients with normal pregnancies, severe preeclampsia and HELLP syndrome were measured by enzyme-linked immunoabsorbentassay technique, using kits of human soluble endoglin, endothelin-1 and p38 MAPK, Quantikine immunoassay: R&D System Inc.Results: Level of serum p38 MAPK in HELLP syndrome group was higher than in severe preeclampsia and normal pregnancy groups. Soluble endoglin and endothelin-1 levels in pregnancies with severe preeclampsia and HELLP syndrome were higher than normal pregnancy but there was no significant difference between these two groups (P>0.05). Levels of p38 MAPK, soluble endoglin and endothelin-1 also had a positive linear correlation with systolic and diastolic blood pressures (P<0.05).Conclusions: P38 MAPK in serum may be a marker for evidence of the severe hypoxia and its application may be considered for the diagnosis of HELLP syndrome.

Sickle cell crisis in third trimester of pregnancy complicated by hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome

Introduction: Sickle cell disease is one of the most common hemoglobinopathies in the world, affecting approximately 100,000 people annually in the United States alone. This case presents an example of the importance of close monitoring during the pregnancies of patients with sickle cell disease in order to facilitate prompt evaluation and treatment of complications as they arise. Case Report: A 22-year-old, gravida 2 para 1001, was admitted at 306/7 weeks of gestation due to sickle cell crisis. Following admission and observation, she developed hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome on hospital day-4, and decision was made to proceed with delivery. The patient was maintained on magnesium seizure prophylaxis for 24 hours following delivery. The patient was discharged home on post-operative day-5 on labetalol 400 mg q8hrs secondary to persistent hypertension. The patient was doing well at her routine six week postpartum visit and maintained on the anti-hypertensive medication. Conclusion: This case of HELLP syndrome following a sickle cell crisis highlights the importance of close monitoring of sickle cell disease patients during pregnancy. Multi-disciplinary care plays a critical role in the successful management of these patients due to the acute decompensation that can follow a sickle cell pain episode. A facility must be prepared to provide support from obstetrics, hematology, neurology, and intensive care. The obstetrical team must understand sickle cell physiology. The resources to provide prompt blood transfusion and exchange transfusion must also be available. This knowledge is especially important to health care providers who may be taking care of patients in lower resource settings.

Effects of high-dose dexamethasone in postpartum women with class 1 haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome

This study was performed to investigate the effectiveness of dexamethasone in the management of postpartum women with class 1 haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. This retrospective study was conducted on 18 women with class 1 HELLP syndrome at the Shiga University of Medical Science. They were divided into two groups: Group A comprised participants who did not receive dexamethasone, and Group B comprised participants that intravenously received dexamethasone. The main outcomes were the serum laboratory values, mortality and morbidity. The only significant difference between the two groups in baseline characteristics was the aspartate aminotransferase levels. The linear regression analysis showed a significant difference between the two groups in the recovery of platelet counts (p = .046) and aspartate aminotransferase (p = .009). These findings support the use of high-dose dexamethasone to promote recovery of the platelet counts and aspartate aminotransferase levels in postpartum women with class 1 HELLP syndrome.Impact statementWhat is already known on this subject? Haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome is one of the most dangerous complications that can occur during pregnancy and is considered a particularly serious variant of severe preeclampsia. Several clinical trials have been performed since 1994 because it was expected that corticosteroid therapy, primarily with dexamethasone, accelerates recovery after delivery. However, the effect of dexamethasone therapy on class 1 HELLP syndrome is unclear.What do the results of this study add? In this retrospective study, we demonstrated that dexamethasone administration significantly improved the recovery of the platelet count in postpartum women with class 1 HELLP syndrome, and did not increase the rate of maternal postpartum complications.What are the implications of these findings for clinical practice and/or further research? The use of high-dose dexamethasone in postpartum women with class 1 HELLP syndrome might be effective to promote recovery of the platelet count, and contributes a shorter duration of hospitalisation. Because the number of patients with class 1 HELLP syndrome is small, it is important to confirm these findings with well-designed multicentre prospective studies.

863: Pragmatic laboratory studies to differentiate acute fatty liver of pregnancy and HELLP syndrome on admission

Acute fatty liver of pregnancy (AFLP) and hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome are both associated with significant maternal and perinatal morbidity and mortality. Because of the overlap of several clinical and laboratory findings of these syndromes, differentiation can be difficult. Our group has previously suggested application of commonly available laboratory analytes to identify cases of AFLP. The purpose of this analysis was to use these laboratory analytes to distinguish between cases of AFLP and HELLP syndrome in women presenting with elevated transaminases. This was a retrospective observational analysis of women admitted to a tertiary medical center with either AFLP or HELLP syndrome. Women with AFLP were diagnosed using the Swansea criteria and in some cases liver histology, whereas HELLP syndrome were identified using the Tennessee classification system/Mississippi classification system (Class I and II). Clinical and laboratory findings at the time of admission were analyzed in an attempt to differentiate the two syndromes. There were 66 women with AFLP identified from 1975-2018 and these were compared with 67 randomly selected women with HELLP syndrome. Table 1 displays selected demographic and clinical features. There were no significant differences noted among demographic features of age, race, and parity (all p>0.05). Abdominal pain was common in both groups, whereas nausea and vomiting were significantly associated with AFLP (P<0.001). Women with AFLP had markedly abnormal laboratory analytes that included hypofibrinogenemia, hyperbilirubinemia, and hypocholesterolemia, as well as evidence of acute renal insufficiency (Table 2). All of these were significantly different from women with HELLP syndrome (all p<0.001). For these specific analytes, the HELLP values were within the normal range in the third trimester of pregnancy. A pentad of commonly available laboratory analytes can assist with the evaluation of cases of AFLP and HELLP syndrome on admission when elevated transaminase values are identified. We propose the acronym FaCCT —Fibrinogen, and Cholesterol, Creatinine, Total Bilirubin—as a simple moniker to assist when differentiating women with either AFLP or HELLP syndrome.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Comparison of Serum Interleukin-6 (Il-6) Levels Between Patients With HELLP Syndrome Versus Normotensive Pregnant

Background: Interleukin-6 (IL-6) has been known associated with oxidative stress and endothelial dysfunction, and also has important roles in pathogenesis of preeclampsia. Several studies have demonstrated that there was a significant increase of serum IL-6 levels in preeclamptic compared to normotensive pregnant women. However, study to evaluate serum IL-6 level in pregnant women with HELLP (hemolysis, elevated liver enzyme, and low platelet) syndrome is not well documented yet. This study aims to evaluate serum IL-6 levels between women with HELLP syndrome compared to normotensive pregnant.Methods: The research design was observational cross-sectional study. Samples were recruited consecutively using inclusion and exclusion criteria from emergency department and inpatient wards at Dr. Sardjito General Hospital, Yogyakarta, Indonesia. Serum IL-6 were collected from venous blood sample and measured by enzyme-linked immunoassay (ELISA) method. Independent-samples t-test or Mann-Whitney test was used to compare serum IL-6 values between women with HELLP syndrome and normotensive pregrnancy. A statistical measurement conducted using SPSS IBM Statistics 19® and considered significance when p&lt;0,05.Results: There were 46 subjects, consisted of 23 women with HELLP syndrome and 23 women with normotensive pregnancy. Serum IL-6 level was a mean of 0,11±0,08 pg/mL and 0,15±0,20 pg/mL in women with HELLP syndrome compared to normotensive pregnancy respectively. There was no significant statistical difference between both groups (p=0,17).Conclusion:.The result of this study has shown that no significant difference of serum IL-6 level in HELLP syndrome women compared to normotensive pregnant women.

Interventionist versus expectant care for severe pre-eclampsia between 24 and 34 weeks' gestation.

Severe pre-eclampsia can cause significant mortality and morbidity for both mother and child, particularly when it occurs remote from term, between 24 and 34 weeks' gestation. The only known cure for this disease is delivery. Some obstetricians advocate early delivery to ensure that the development of serious maternal complications, such as eclampsia (fits) and kidney failure are prevented. Others prefer a more expectant approach, delaying delivery in an attempt to reduce the mortality and morbidity for the child that is associated with being born too early. To evaluate the comparative benefits and risks of a policy of early delivery by induction of labour or by caesarean section, after sufficient time has elapsed to administer corticosteroids, and allow them to take effect; with a policy of delaying delivery (expectant care) for women with severe pre-eclampsia between 24 and 34 weeks' gestation. For this update, we searched Cochrane Pregnancy and Childbirth's Trials Register, ClinicalTrials.gov, the WHO International Clinical Trials Registry Platform (ICTRP) on 27 November 2017, and reference lists of retrieved studies. Randomised trials comparing the two intervention strategies for women with early onset, severe pre-eclampsia. Trials reported in an abstract were eligible for inclusion, as were cluster-trial designs. We excluded quasi-randomised trials. Three review authors independently assessed trials for inclusion and risk of bias, extracted data, and checked them for accuracy. We assessed the quality of the evidence for specified outcomes using the GRADE approach. We included six trials, with a total of 748 women in this review. All trials included women in whom there was no overriding indication for immediate delivery in the fetal or maternal interest. Half of the trials were at low risk of bias for methods of randomisation and allocation concealment; and four trials were at low risk for selective reporting. For most other domains, risk of bias was unclear. There were insufficient data for reliable conclusions about the comparative effects on most outcomes for the mother. Two studies reported on maternal deaths; neither study reported any deaths (two studies; 320 women; low-quality evidence). It was uncertain whether interventionist care reduced eclampsia (risk ratio (RR) 0.98, 95% confidence interval (CI) 0.06 to 15.58; two studies; 359 women) or pulmonary oedema (RR 0.45, 95% CI 0.07 to 3.00; two studies; 415 women), because the quality of the evidence for these outcomes was very low. Evidence from two studies suggested little or no clear difference between the interventionist and expectant care groups for HELLP (haemolysis, elevated liver enzymes, and low platelets) syndrome (RR 1.09, 95% CI 0.62 to 1.91; two studies; 359 women; low-quality evidence). No study reported on stroke. With the addition of data from two studies for this update, there was now evidence to suggest that interventionist care probably made little or no difference to the incidence of caesarean section (average RR 1.01, 95% CI 0.91 to 1.12; six studies; 745 women; Heterogeneity: Tau² = 0.01; I² = 63%).For the baby, there was insufficient evidence to draw reliable conclusions about the effects on perinatal deaths (RR 1.11, 95% CI 0.62 to 1.99; three studies; 343 women; low-quality evidence). Babies whose mothers had been allocated to the interventionist group had more intraventricular haemorrhage (RR 1.94, 95% CI 1.15 to 3.29; two studies; 537 women; moderate-quality evidence), more respiratory distress caused by hyaline membrane disease (RR 2.30, 95% CI 1.39 to 3.81; two studies; 133 women), required more ventilation (RR 1.50, 95% CI 1.11 to 2.02; two studies; 300 women), and were more likely to have a lower gestation at birth (mean difference (MD) -9.91 days, 95% CI -16.37 to -3.45 days; four studies; 425 women; Heterogeneity: Tau² = 31.74; I² = 76%). However, babies whose mothers had been allocated to the interventionist group were no more likely to be admitted to neonatal intensive care (average RR 1.19, 95% CI 0.89 to 1.60; three studies; 400 infants; Heterogeneity: Tau² = 0.05; I² = 84%). Babies born to mothers in the interventionist groups were more likely to have a longer stay in the neonatal intensive care unit (MD 7.38 days, 95% CI -0.45 to 15.20 days; three studies; 400 women; Heterogeneity: Tau² = 40.93, I² = 85%) and were less likely to be small-for-gestational age (RR 0.38, 95% CI 0.24 to 0.61; three studies; 400 women). There were no clear differences between the two strategies for any other outcomes. This review suggested that an expectant approach to the management of women with severe early onset pre-eclampsia may be associated with decreased morbidity for the baby. However, this evidence was based on data from only six trials. Further large, high-quality trials are needed to confirm or refute these findings, and establish if this approach is safe for the mother.

FAS A-670G and Fas ligand IVS2nt A 124G polymorphisms are significantly increased in women with pre-eclampsia and may contribute to HELLP syndrome: a case-controlled study.

We evaluated the association between the Fas-670A/G and the Fas ligand FasL IVS2nt 124 A/G polymorphisms and the risk of pre-eclampsia and its complications. A case-controlled study. University Hospitals in most areas of Tunisia. We recruited 300 pregnant women who developed pre-eclampsia and 300 age-matched healthy pregnant women from the same hospital. Genotyping of Fas-670A/G and the FasL IVS2nt 124A/G gene polymorphisms were conducted using polymerase chain reaction-restriction fragment length polymorphism among our cohort. Fisher's exact test was used to compare the statistical differences between groups for categorical variables and Student t tests were used for continuous variables. The frequency of the Fas-670G gene variant was significantly increased in women with pre-eclampsia (42%) compared with control women (30%; P < 0.001). Also, a statistically significant difference was obtained in the distribution of the FasL IVS2nt 124G gene variant when comparing women with pre-eclampsia (43%) with controls (30%; P < 0.001). Interestingly, we found that the carriage of Fas-670G was associated with increased liver enzymes, suggesting an increased prevalence of the haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome, a pre-eclampsia complication. The Fas-670G and FasL IVS2nt 124G polymorphisms are associated with a higher risk of pre-eclampsia and its complications. Polymorphisms in the Fas and FasL genes are associated with increased risk of pre-eclampsia and HELLP syndrome.

Eculizumab in pregnancy: a narrative overview.

Pregnancy can be a dangerous trigger for patients with paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), or hemolysis, elevated liver enzymes and low platelet (HELLP) syndrome. Due to the possibility of several serious complications, pregnancy is somewhat discouraged in the presence of the above diseases. Eculizumab is a humanized antibody that may dramatically change the clinical course of PNH, aHUS and HELLP syndrome. However, data on the safety of eculizumab in pregnancy are scarce. In this narrative overview, we summarize current evidence on the use of eculizumab during pregnancy in women with PNH, aHUS and HELLP syndrome. Eculizumab is not present in breast milk, and the levels observed in umbilical cord blood samples are not sufficient to affect the concentrations of complement in newborns. Therefore, eculizumab may be regarded as safe in pregnancy. Nonetheless, given that data on eculizumab in pregnancy are limited, it is not possible to completely exclude risks for both mother and fetus in treating PNH, aHUS and HELLP syndrome.

A Retrospective Evaluation of Obstetric Patients Admitted to the Intensive Care Unit: Has Anything Changed in the New Era of Technology?

Objective: Despite the fact that obstetric cases constitute only a small portion of intensive care hospitalizations, maternal mortality still remains an important health problem in the world. In this study, we aimed to evaluate the frequency and reasons of admission of obstetric patients to the intensive care unit (ICU), as well as the ‘advanced’ treatments applied and the clinical outcomes. Materials and Methods: The medical records of 8800 obstetric patients who were admitted to the hospital between 2012-2017 were retrospectively reviewed. The demographic data, reasons for admission to the ICU, concomitant diseases, intensive care interventions (advanced monitoring, extracorporeal therapies, etc.) and clinical outcomes were evaluated. Results: Of the 8800 obstetric patients, 40 (0.45%) patients required intensive care. The mean age was 32.2±5 years. The mean duration of stay in the ICU was 4.3 days. The most common reasons for admission to the ICU were HELLP-Preeclampsia-Eclampsia in 16 (40%) patients and bleeding (due to disseminated intravascular coagulation, uterine perforation, uterine atonia) in 11 (27%) patients. Seventeen patients received invasive mechanical ventilation and 3 patients received non-invasive mechanical ventilation. Two patients received extracorporeal membrane oxygenation (ECMO), 4 patients received plasmapheresis and 5 patients received hemodiafiltration. Three patients died. One patient died of multiorgan failure secondary to HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Two patients died due to HELLP and intracerebral hemorrhage. Conclusion: Advanced intensive care settings where new-era technological equipment (ECMO, hemodiafiltration, advanced monitoring, etc.) is in-reach and employing a multidisciplinary approach in the ICU may decrease the maternal mortality and morbidity.

Incidence of and risk factors for severe maternal complications associated with hypertensive disorders after 36 weeks' gestation in uncomplicated twin pregnancies: A prospective cohort study.

To elucidate the incidence of and risk factors for severe hypertensive disorders (HD) and related maternal complications in uncomplicated twin pregnancies that reached 36 weeks' gestation. We conducted a prospective cohort study of twin pregnancies delivered after 36 weeks' gestation. Cases of twin-twin transfusion syndrome, twin anemia-polycythemia sequence, malformed fetuses, monoamniotic twins, selective reduction, fetal therapy and HD or fetal death before 35 weeks' gestation were excluded. The study's primary outcome was the incidence of severe maternal complications, including severe HD, eclampsia, placental abruption, HELLP (hemolysis, elevated liver enzyme and low platelet) syndrome, pulmonary edema and cerebrovascular disease. Perinatal factors associated with the primary outcome were identified using a multivariate logistic regression model. In 330 enrolled women, the number of cases with the primary outcome was 28 (8.5%; 95% confidence interval 5.9-12.0), including 25 cases of severe HD and each one case of placental abruption, HELLP syndrome and eclampsia. The rate of severe maternal complications significantly increased with gestational age, demonstrating 1.2% at 36 weeks, 3.9% at 37 weeks and 6.4% at 38 weeks. Only gestational proteinuria was identified as the independent risk factor for severe maternal complications (adjusted odds ratio 17.1 [95% confidence interval 6.71-45.4]). Severe maternal HD and related complications increased from late preterm to early term; particularly, patients with gestational proteinuria were at high risk.

Germline mutations in the alternative pathway of complement predispose to HELLP syndrome.

HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome is a severe variant of hypertensive disorders of pregnancy affecting approximately 1% of all pregnancies, and has significant maternal and fetal morbidity. Previously, we showed that upregulation of the alternative pathway of complement (APC) plays a role in HELLP syndrome. We hypothesize that HELLP syndrome follows a 2-hit disease model similar to atypical hemolytic uremic syndrome (aHUS), requiring both genetic susceptibility and an environmental risk factor. Our objective was to perform a comparative analysis of the frequency of APC activation and germline mutations in affected women and to create a predictive model for identifying HELLP syndrome. Pregnant women with HELLP syndrome, and healthy controls after 23 weeks of gestation were recruited, along with aHUS and thrombotic thrombocytopenic purpura participants. We performed a functional assay, the mHam, and targeted genetic sequencing in all groups. Significantly more participants with rare germline mutations in APC genes were present in the HELLP cohort compared with controls (46% versus 8%, P = 0.01). In addition, significantly more HELLP participants were positive for the mHam when compared with controls (62% versus 16%, P = 0.009). Testing positive for both a germline mutation and the mHam was highly predictive for the diagnosis of HELLP syndrome. HELLP syndrome is characterized by both activation of the APC and frequent germline mutations in APC genes. Similar to aHUS, treatment via complement inhibition to mitigate maternal and fetal morbidity and mortality may be possible. National Heart Lung and Blood Institute grants T32HL007525 and R01HL133113.

Use of Antenatal Corticosteroids in Special Circumstances: A Comprehensive Review

(Acta Obstet Gynecol Scand. 2017;96(4):395–409) A literature review was undertaken to formulate recommendations on the usage of antenatal corticosteroids for pregnancies with specific complications—preterm premature rupture of membranes (PPROM), the severe form of preeclampsia known as HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome, very early deliveries (23 to 26 wk), near term deliveries (34 to 38 wk), intrauterine growth restriction, and cases of multiple births. PubMed, Cochrane Database of Systematic Reviews (1990 to 2015), Clinicaltrials.gov, and Web of Science were searched and 49 studies were found acceptable to be used as the basis of this review.

Severe preeclampsia with HELLP syndrome with Wilson's disease.

Severe Preeclampsia is characterized by systolic blood pressure ≥ 160 mmHg or diastolic pressure >110 mmHg, protein excretion >5 gm/24 hour or 3+ or more on random samples, oliguria <500 ml/24 hr, platelet count <1,00,000/mm3, hemolysis, elevated alanine aminotransferase and aspartate aminotransferase, cerebral and visual disturbance, persistent severe epigastric pain, retinal hemorrhages, exudates and papilledema, intrauterine growth restriction of the fetus and pulmonary edema. HELLP (Hemolysis, elevated liver enzymes and low platelets) syndrome is the variant of preeclampsia. Definitive treatment of HELLP syndrome is delivery of the baby. Wilson's disease is autosomal recessive disorder of copper metabolism. It is characterized by the toxic accumulation of copper mainly in liver and brain but also in cornea and kidney due to defective biliary excretion of copper. It is one of the major causes of cirrhosis of liver. Excess copper levels are associated with the disorder of the menstrual cycle and infertility. Increased copper levels are also associated with the preeclampsia, intrauterine growth restriction and neurologic damages in the fetus. However Pregnancy is not contraindicated in well treated Wilson's disease. A case of decompensated cirrhosis due to Wilson's disease, presented in third trimester of pregnancy, with Preeclampsia and HELLP syndrome is discussed.

Liver Injury in Hemolysis, Elevated Liver Enzymes, and Low Platelets Syndrome Measured by Diffusion-Weighted Magnetic Resonance Imaging.

The objective of this study was to evaluate acute liver injury (ALI) detected by diffusion-weighted magnetic resonance imaging (MRI) and the associated laboratory findings in women with hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome. This was a retrospective, observational study of women with HELLP syndrome defined by serum aspartate aminotransferase (AST) levels ≥100 U/L and thrombocytopenia with platelets ≤100,000/µL. All women underwent MRI postpartum including diffusion-weighted imaging to estimate the volume of ALI with reconstructed apparent diffusion coefficient (ADC) maps. The ADC map and the volume of ALI were compared with laboratory abnormalities by Spearman's correlation analysis. From March 2013 through August 2015, 16 women with HELLP syndrome underwent MRI, and of these, 14 (88%) women had areas of increased signal intensity suggestive of ALI. Their median (range) maximum AST level was 262 (140-1,958) IU/L, and at the time of MRI, AST was 103 (36-1,426) IU/L. Both of these AST levels significantly correlated with ADC map as well as the volume of ALI (both p-values <0.001). Women with HELLP syndrome frequently exhibited areas of abnormal diffusion in the liver on diffusion-weighted MRI, suggestive of ALI. The extent of liver injury was significantly correlated with serum AST.

Dexamethasone in pregnant women with hemolysis, elevated liver enzymes, and low platelet count (hellp) syndrome: A case report

Introduction: HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome is a hypertensive disorder in pregnancy with severe pre-eclampsia, resulting in increased maternal and neonatal morbidity and mortality. The management of HELLP syndrome is controversial because the pathophysiology of the disease is not well understood. The current standard of care is supportive therapy and immediate delivery of the fetus. However, the use of corticotherapy may have potential benefit on improving maternal recovery, but various studies reported inadequate and inconsistent findings. Case presentation: A 25-year-old pregnant patient presented to the emergency room with vaginal bleeding and hypertension. She was diagnosed with severe pre-eclampsia and underwent delivery via a cesarean section and was then transferred to the intensive care unit (ICU) due to exacerbation of HELLP syndrome. Prompt initiation of supportive care was provided and dexamethasone 10 mg IV Q 12 hours was administered. The patient recovered and was discharged from the hospital four days after corticotherapy was initiated, on day eight. Conclusion: Various studies evaluated the use of corticosteroids, specifically dexamethasone, in the improvement of HELLP syndrome and maternal outcomes. Evidence on the management of the syndrome remains controversial due to limits in the strength of the conclusions of various clinical trials. As a result, the evidence is insufficient to justify the use of corticotherapy in the management of HELLP syndrome to improve maternal outcomes.

The Lack of Neonatal Consequences in Pre-Eclampsia, Eclampsia and HELLP Syndrome: A Surprising Finding

Background: Knowledge of the correlation between maternal conditions, especially during pregnancy and fetal outcomes is paramount to optimal care in pediatrics. One which has not been frequently studied in the literature is pre-eclampsia, eclampsia and hemolysis, elevated liver enzymes and low platelets (HELLP).Methods: A retrospective review of all documented cases of pre-eclampsia, eclampsia and HELLP was done at a community Regional Neonatal Center. Over a five year period, 291 mothers were diagnosed with one of the three diseases and gave birth to 318 children. The children were stratified for gestational age and birth weight. Selected conditions were screened for in the medical record over the first year of life.Results: Of the 318 neonates, two were still births and one mother died from intracranial hemorrhage in the face of HELLP. Two hundred and twelve (66.67%) were born before 37 weeks, which is higher than the expected rate for these conditions. A total of 114 neonates were low birth weight (36%), while 75 were very low birth weight (23%). None of the conditions appeared significantly different for their suspected incidence when stratified for gestational age and birth weight.Conclusion: Eclampsia and HELLP are serious conditions in the pregnant patient, which significantly increase rates of preterm delivery, as well as correlates with low birth weights. As with all maternal conditions, thought must be given to the effect of these derangements on the developing fetus. Conditions such as necrotizing enterocolitis (NEC) caused by low flow sates and intestinal atresias, thought to occur with vascular incidents, were not seen at a higher rate in these patients once stratified for gestational age and birth weight.

P193 Incidence of AT1R- antibody in women with hellp syndrome

Aim Antibodies against the angiotensin II type 1 receptor (AT1R-Abs) have been implicated in a number of disease processes, including development of fibrosis in cystic fibrosis and systemic sclerosis, antibody mediated rejection in solid organ transplantation and more recently frailty in an aging population. AT1R-Abs were originally isolated in women with preeclampsia characterized by development of hypertension during pregnancy. Hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome has been recognized as a complication of preeclampsia in pregnant women. Diagnostic tools are needed to distinguish women who develop HELLP syndrome from preeclampsia. In this study we determined the prevalence of AT1R-Ab in patients with HELLP syndrome. Methods Sera from 45 women (11 controls defined as having normal pregnancies, 19 HELLP or partial HELLP, 15 preeclampsia) were tested for presence of AT1R-Ab. The average gestational age was 31.9 weeks. The group included 21 Caucasian, 19 African American and 5 non-Caucasian, non-African American women. AT1R-Ab testing was performed using quantitative ELISA (One Lambda, ThermoFisher). A concentration >17 Units/ml was considered positive for AT1R-Ab. Results Three of the 19 HELLP patients (16%) and 1 of the 15 preeclampsia patients (6%) were positive for AT1R-Ab. None of the patients in the control group had AT1R-Ab >17 Units/ml. Overall, there was no significant difference in the mean AT1R-Ab concentration between the control group and the diseased group (preeclampsia and HELLP; 11.39 Units/ml versus 9.48 Units/ml respectively; p = 0.2). Conclusions In this small study, we did not find a significant difference in the concentration of AT1R-Ab in the disease population compared to the control group. Interestingly, while none of the patients in the control group had concentrations >17 Units/ml, 4 patients in the diseased group had strong antibody. A larger patient population may be needed to determine significance. Evaluation of the AT1R-Ab ELISA for detection of AT1R-Ab in populations other transplant patients can be beneficial.

Pregnancy with primary hyperparathyroidism: analysis of five cases

Objective To study the clinical presentations, diagnosis and managements of primary hyperparathyroidism (pHPT) in pregnancy. Methods A total of five cases of pHPT in pregnancy were enrolled from January 2005 to December 2014 in Peking Union Medical College Hospital. Their clinical presentations, managements, maternal-fetal complications and pregnancy outcomes were retrospectively analyzed. Results The median age was 32 (29, 41) years. Of the five cases, three were diagnosed in the second trimester, one was before pregnancy and one was after delivery. Most of the clinical symptoms were nonspecific to pHPT, such as nausea, vomiting and loss of appetite. Frequent urination and nocturia occurred in one; unconsciousness and manifestations of acute pancreatitis and eclampsia relevant symptoms were complained of by one. The common maternal complications were nephrolithiasis and hydronephrosis (3/5), osteoporosis (2/5), anemia (2/5) and kaliopenia (2/5), while the severe complications were hypercalcemic crisis (2/5), acute pancreatitis (1/5), eclampsia (1/5), HELLP (hemolysis, elevated liver enzymes and low platelets) syndrome (1/5), disseminated intravascular coagulation (DIC) (1/5), cerebral infarction (1/5) and intrauterine fetal death of one twin (1/5). The median level of calcium in serum samples was 3.70 (2.78, 4.50) mmol/L; the median level of parathyroid hormone (PTH) in serum samples was 294 (151, 634) pg/ml. All of the five cases were positive for parathyroid ultrasonography. Four cases received parathyroid radionuclide imaging and had positive results. One asymptomatic patient received no specific treatment, whereas the neonate presented with hypocalcemia after birth. Two cases received surgical resections in the second trimester; one of them had a live birth without fetal complication, while the other had induced abortion. Two cases received postpartum surgery; one asymptomatic patient had a live birth without fetal complication, while the other with twin pregnancy suffered stillbirths (one intrauterine fetal death and one neonatal death). Pathologic diagnosis were solitary parathyroid adenomas in four cases who received surgery. Their operations were effective except that one case, which was improved after treatment, was complicated with secondary hypoparathyroidism. Conclusions The symptoms of pHPT in pregnancy are often nonspecific, but it can cause maternal and fetal morbidity and mortality. Early diagnosis of pHPT, followed by appropriate managements, has been shown to significantly reduce the complications. Surgical management should be a safe and effective choice. Key words: Hyperparathyroidism, primary; Pregnancy complications

Dysregulation of the Fas/FasL system in an experimental animal model of HELLP syndrome.

Placental FasL is up-regulated in women with HELLP (hemolysis elevated liver enzyme and low platelet) syndrome and has been proposed to contribute to the liver damage seen in these patients. This study aimed to determine if an experimental rodent model of HELLP also had dysregulation of Fas/FasL compared to normal pregnant (NP) rats. We also set out to determine if blockade of the endothelin system regulated Fas/FasL expression in HELLP rats. On gestational day (GD) 12, sEng (7ug/kg) and sFlt-1 (4.7ug/kg) infusion began via mini-osmotic pump into NP rats. On GD19 plasma and tissue were collected and FasL and Fas were measured via enzyme linked immunosorbent assay and gene expression via real-time PCR. HELLP rats had significantly more circulating and placental FasL compared to NP rats, whereas hepatic FasL was decreased and placental Fas was increased compared to NP rats. Administration of an endothelin A receptor antagonist (ETA) beginning on GD12 significantly decreased placental expression of Fas in HELLP rats. Liver mRNA transcript of Fas was significantly increased in HELLP rats compared to NP rats. These data suggest that rats in this experimental model of HELLP syndrome have abnormal expression of the Fas/FasL system. Future studies will examine the sources of Fas/FasL dysregulation in this model and if blockade could reduce some of the inflammation and hypertension associated with HELLP syndrome.