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

POS-001 A NOVEL CFHR5 COPY NUMBER VARIATION ASSOCIATED WITH POST-PARTUM ATYPICAL HUS SUPERIMPOSED TO HELLP SYNDROME

Pregnancy and postpartum are triggers for various forms of thrombotic microangiopathy (TMA), a lesion characterized by endothelial injury and thrombosis in the microcirculation of various organs. These forms include thrombotic thrombocytopenic purpura (TTP), caused by ADAMTS13 deficiency, and atypical hemolytic uremic syndrome (aHUS), most often associated with genetically determined dysregulation of complement system. HUS is the most common form of TMA in the post-partum period. The diagnosis of pregnancy-associated HUS can be difficult because preeclampsia and HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome may present with clinical and laboratory features of TMA that however commonly resolve spontaneously after delivery.Here, we report the biochemical and genetic characterization of a 22 years old primigravida of African origin who presented at 38 weeks of gestation with severe hypertension, nephrotic syndrome (NS) and elevated liver enzymes, consistent with a diagnosis of HELLP.

Maternal – Fetal Outcome in Cases of HELLP Syndrome at the Delta State University Teaching Hospital

Aims: Hypertensive disorders of pregnancy (HDP) are a leading cause of maternal and perinatal morbidity and mortality in resource-poor nations like Africa. This is even more predicated in HELLP (Haemolysis, Elevated Liver enzymes and Low Platelets) syndrome, which is a life-threatening pregnancy complication and is usually considered to be a variant of preeclampsia. The present study therefore investigated maternal – fetal outcome in cases of HELLP syndrome at the Delta State University Teaching Hospital.
 Study Design: The study is a descriptive retrospective investigation, which comprised 210 pregnancies complicated by HELLP syndrome, which were acquired from their separate medical records.
 Place and Duration of Study: The study was carried out at the Delta state University Teaching Hospital (DELSUTH),Oghara, Delta State, Nigeria over a 24-month period.
 Methodology: In newborn medical records, several outcome variables were evaluated. The gestational age was calculated using obstetric characteristics such as menstrual history, early clinic checkup, and ultrasound at 20 weeks of pregnancy.
 Results: The average age of the mothers in the study was 29 years old, with a 95th percentile age of 40 years old. Preeclampsia risk was also enhanced by having more children. Preeclampsia is a condition that affects pregnant women who are more than 30 weeks pregnant. The HELLP syndrome has a significant impact on the outcome of the fetus. New stillbirth accounted for 4.6 percent of fetal outcomes, whereas early neonatal death, which occurred within the first 28 days of life, accounted for 3.4 percent of the 210 participants in the study. In addition, 2.3 percent of stillbirths were macerated, and 10.3 percent of fetuses died in the womb.
 Conclusion: There is a relationship between age and the occurrence of preeclampsia, according to the HELLP syndrome classification. In each age group, a minor percentage of the subjects (6%) had Class I HELLP syndrome, while the majority (60.5%) had Class II HELLP syndrome.

Kidney-placenta crosstalk in health and disease.

ABSTRACTOrgan crosstalk allows the interaction between systems to adapt to a constant changing environment, maintaining homeostasis. The process of placentation and the new hormonal environment during pregnancy trigger physiological changes that modulate kidney function to control extracellular volume, acid–base balance and filtration of metabolic waste products. The bidirectional communication means that acute or chronic dysfunction of one organ can compromise the other. Abnormal placentation in pregnancy-related hypertensive disorders such as pre-eclampsia and HELLP (haemolysis, elevated liver enzymes and low platelet count) syndrome leads to the release of antiangiogenic factors that may cause kidney injury (thrombotic microangiopathy, glomeruloendotheliosis, mesangiolysis and vasoconstriction of peritubular vessels). These hypertensive disorders are a key cause of kidney injury in gestation, which increases maternal morbimortality and adverse foetal outcomes. Conversely, prior kidney injury or causes of kidney injury (diabetes, lupus, glomerulonephritis or other forms of chronic kidney disease) increase the risk of developing hypertensive pregnancy disorders, providing a baseline higher risk. Inherited kidney diseases are a special concern, given the potential for genetic predisposition to kidney disease in the foetus. Understanding the bidirectional potential for compromise from placenta to kidney and vice versa provides a better framework to limit damage to both organs and improve maternal and foetal outcomes.

Clinical Classification, Pregnancy Outcomes and Risk Factors Analysis of Severe Preeclampsia Complicated With HELLP Syndrome.

PurposeTo investigate the clinical classification, pregnancy outcomes and risk factors of pregnant women with severe preeclampsia (SPE) complicated with HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome.MethodsThe clinical data of 50 pregnant women diagnosed with SPE complicated with HELLP syndrome in our hospital from January 2014 to January 2021 were retrospectively analyzed, and they were selected as the observation group. An additional 50 maternities diagnosed with preeclampsia (PE) during the same period were selected as the control group. The clinical classification and pregnancy outcomes of pregnant women in the observation group were recorded. The age and gestational age of onset of pregnancy were recorded and compared between the two groups. Univariate analysis and multivariate logistic regression model were used to analyze the risk factors for its occurrence.ResultsAmong the 50 maternities in the observation group, there were 10 cases of type I, accounting for 20.00%; 35 cases of type II, accounting for 70.00%; 5 cases of type III, accounting for 10.00%. Partial 33 cases, the composition ratio of 66.00%; complete 17 cases, the composition ratio of 34.00%. Among the fetuses of 50 maternities in the observation group, 35 were premature, accounting for 70.00%; 13 had fetal growth restriction, accounting for 26.00%; and 2 died during perinatal period, accounting for 4.00%. Among the 50 maternities in the observation group, 48 cases were cesarean section, the composition ratio was 96.00%; 2 cases were induced labor, the composition ratio was 4.00%; there was no natural birth, the composition ratio was 0.00%. Univariate analysis showed that age, gestational age at onset, gestational age at termination of pregnancy, HGB, LDH, ALT, AST, TBIL, PLT, PT, and FIB were all associated with the occurrence of SPE complicated with HELLP syndrome (P < 0.05). Multivariate logistic analysis showed that gestational age at onset, gestational age at termination of pregnancy, HGB, LDH, ALT, AST, TBIL, PLT, and FIB were independent risk factors for SPE complicated with HELLP syndrome (P < 0.05).ConclusionSPE complicated with HELLP syndrome has significantly increased adverse pregnancy outcomes. Understanding its clinical classification is of great significance for the preventive application of platelet transfusion therapy and the selection of transfusion timing. Gestational age at onset and gestational age at termination of pregnancy are independent risk factors for its occurrence. Fully understanding the high-risk factors of HELLP syndrome, taking preventive measures in time, and carrying out targeted nursing can effectively improve the prognosis of pregnant women and reduce the risk of HELLP syndrome.

S1189 Efficacy of Corticosteroid Administration in Patients With Hemolysis, Elevated Liver Enzymes, and Low Platelet Count (HELLP) Syndrome: A Meta-Analysis

Introduction: Hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome is considered an obstetrical emergency, posing risk for high maternal morbidity and mortality if left untreated. While corticosteroids (CS) have been postulated to modify some of the pro-inflammatory features of HELLP syndrome, results from meta-analyses have thus far lacked the evidence to support CS’ overall benefit in attenuating disease process. In the past several years, there has been additional data published on the use of CS in HELLP syndrome, prompting an updated meta-analysis on the matter. Our study aims to evaluate the prognostic benefits of CS in HELLP syndrome. Methods: We performed a systematic literature review using multiple electronic databases. Continuous data under the generic inverse variance model was pooled and expressed as a standardized mean difference while the dichotomous data was analyzed using the Mantel-Haenszel model and expressed as odds ratios. Results: Nineteen studies were included. Administration of CS in HELLP syndrome was associated with a significantly increased platelet count (MD: 38.71 p < 0.05), decreased LDH (MD: -0.17 p < 0.05), decreased requirement for packed red blood cell transfusion (OR: 0.46 p < 0.05), decreased requirement for plasma transfusion (OR: 0.29 p < 0.05), and an increased risk of infection (OR: 1.65 p < 0.05). There was no significant difference in the following outcomes: hemorrhage, cardiopulmonary edema, renal failure, transaminitis, mode of delivery, eclampsia, and maternal death. Conclusion: Our results reaffirm those of prior meta-analyses conducted on the effects of CS in HELLP syndrome. Particularly, they highlight CS known effect in optimizing platelet count for the obstetrical patient. Interestingly, our meta-analysis demonstrates a significant decrease in LDH amongs patients with HELLP who received CS. As LDH is a known byproduct of hepatic dysfunction, our findings suggest a possible hepato-protective role of CS on the cellular level. While the effect of CS on hepatic morbidity was not statistically significant, the small sample size of women diagnosed with serious liver pathology made it challenging to assess the clinical impact thereof. At this time, there is not enough evidence to recommend utilization of CS for a hepatic benefit, given their deleterious effect on rates of maternal infection. Future studies with larger sample size data of liver injury in HELLP are warranted to evaluate the impact of CS on hepatic morbidity.Figure 1.: This forrest plot reflects that platelet count increases with corticosteroid administration in patients with HELLP syndrome.

COVID-19 Mimicking Hemolysis, Elevated Liver Enzymes and Low Platelets (HELLP) Syndrome: A Case Report

Background & aim: Hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP) was mimicked by several infectious conditions. It is critically important to distinguish these two, since their management and course differs, substantially.Case report: The case was a 27-year-old gravid patient with gestational age of 30 weeks who initially presented with headache and lower limb pain as well as leukopenia (and lymphopenia), normochromic normocytic anemia, thrombocytopenia, abnormal liver enzymes, increased lactate dehydrogenase enzyme and C-reactive protein. The patient was initially managed for HELLP syndrome, but due to the atypical presentation (low blood pressure and an episode of delirium when admitted), the novel coronavirus disease 2019 (COVID-19), real-time reverse transcription polymerase chain reaction (rRT-PCR) was requested for the patient that was positive. The spiral lung high-resolution computed tomography scan revealed changes compatible with COVID-19 diagnosis. Finally, the patient underwent uncomplicated normal vaginal delivery at 39th gestational week.Conclusion: It is important to consider the COVID-19 in differential diagnosis of patients suspected to HELLP syndrome, as the isolation and treatment of the patient is different and time-sensitive.

Quantitative Alterations in Complement Alternative Pathway and Related Genetic Analysis in Severe Phenotype Preeclampsia.

Preeclampsia and hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome share many clinical and biologic features with thrombotic microangiopathy syndromes caused by complement abnormalities. Our hypothesis was that similar functional and genetic alterations in the complement alternative pathway (CAP) are present in these disorders of pregnancy. We conducted quantitative analysis of proteins involved in CAP using ELISA and nephelometry on prospectively collected blood samples from patients with severe phenotype preeclampsia (defined as delivery ≤34 weeks due to preeclampsia), HELLP syndrome, or eclampsia, and matched normotensive controls (n=25 in each arm) between 2011 and 2016. Sequencing was performed to interrogate 14 genes encoding CAP components. Both groups were similar in age, gravidity, parity, marital status, and race. The study group had a higher BMI (mean±SD, 32±8 versus 25±4 kg/m2; P=0.002) and earlier gestational age at delivery (32.5±3.6 versus 40.3±1 weeks; P<0.001). Serologic studies demonstrated elevated Bb subunit (median [range], 1.2 [0.5-4.3] versus 0.6 [0.5-1] μg/ml; P<0.001), complement C5 concentration (28 [18-33] versus 24 [15-34] mg/dl; P=0.03), and sMAC (371 [167-761] versus 184 [112-249] ng/ml; P<0.001) concentrations in patients with preeclampsia. Two thirds of patients with preeclampsia had at least one nonsynonymous sequence variant in CAP genes. Patients with severe phenotype preeclampsia manifest functional alterations in CAP activation. Genetic variants in the CAP genes were detected in several patients, but a larger population study is necessary to fully evaluate genetic risk. Genetic screening and complement-targeted treatment may be useful in risk stratification and novel therapeutic approaches.

Risk factors for occurrence of placenta accrete spectrum following primary cesarean delivery

Background: Many maternal and fetal complications werereported during delivery for patients diagnosed to have placenta accrete spectrum (PAS). Cesarean delivery (CD) isconsidered to be the most common risk factor for developing PAS disorders during pregnancy specially with increasing rate of CD in developing countries.Methods: A cross- sectional study over 12 months in a tertiary care obstetric unit between January 2020 and January2021.Results: 47 pregnant females having history of previousone CD were divided into 2 groups after ultrasonic and intraoperative evaluation of placenta site and invasion to theuterine wall.14 cases were in the low risk group ad 33 caseswere in the high-risk group. The mean ages of patients were(27.6 ± 4.6 & 27.6 ± 4.6, p value =0.961) respectively. Themedian gravidity was (3 & 2) in both groups. We found that36.4 % of case in the high-risk group had unreliable indications of the primary CD. Emergency caesarean deliverieswere done in about 18 % of cases in the high-risk group either due to failure to progress in labour or foetal distress.We reported successful conservative management in bothgroups using either cervico-isthmic compression sutureor step wise approach. There was statistically significantin the mean amount of intraoperative blood loss (1000 ml(850-1200) &1600 ml (850-2500), p < 0.001) in the low andhigh-risk groups respectively. We reported 3 cases of intraoperative pulmonary embolism, urinary bladder injury and HELLP (hemolysis, elevated liver enzymesand low platelet count) syndrome among patients in the high-risk group. There was statistically significant longer hospital stay durationamong patients in the high-risk group rangingfrom 2 days up to 21 days. On the other hand,there were no serious complications reportedin the low risk group.Conclusion: Decreasing rate of primary CDand optimizing obstetric care are mandatoryto prevent maternal of fetal complication thatcould happen due to having future PAS. Fertility sparing surgery is feasible during surgical management of PAS disorders.

Functional Analysis of p21Cip1/CDKN1A and Its Family Members in Trophoblastic Cells of the Placenta and Its Roles in Preeclampsia

Preeclampsia (PE), a gestational hypertensive disease originating from the placenta, is characterized by an imbalance of various cellular processes. The cell cycle regulator p21Cip1/CDKN1A (p21) and its family members p27 and p57 regulate signaling pathways fundamental to placental development. The aim of the present study was to enlighten the individual roles of these cell cycle regulators in placental development and their molecular involvement in the pathogenesis of PE. The expression and localization of p21, phospho-p21 (Thr-145), p27, and p57 was immunohistochemically analyzed in placental tissues from patients with early-onset PE, early-onset PE complicated by the HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome as well as late-onset PE compared to their corresponding control tissues from well-matched women undergoing caesarean sections. The gene level was evaluated using real-time quantitative PCR. We demonstrate that the delivery mode strongly influenced placental gene expression, especially for CDKN1A (p21) and CDKN1B (p27), which were significantly upregulated in response to labor. Cell cycle regulators were highly expressed in first trimester placentas and impacted by hypoxic conditions. In support of these observations, p21 protein was abundant in trophoblast organoids and hypoxia reduced its gene expression. Microarray analysis of the trophoblastic BeWo cell line depleted of p21 revealed various interesting candidate genes and signaling pathways for the fusion process. The level of p21 was reduced in fusing cytotrophoblasts in early-onset PE placentas and depletion of p21 led to reduced expression of fusion-related genes such as syncytin-2 and human chorionic gonadotropin (β-hCG), which adversely affected the fusion capability of trophoblastic cells. These data highlight that cell cycle regulators are important for the development of the placenta. Interfering with p21 influences multiple pathways related to the pathogenesis of PE.

Maternal myocardial dysfunction after hemolysis, elevated liver enzymes, and low platelets syndrome: a speckle-tracking study.

Pregnancy complicated by pre-eclampsia (PE) and hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome is associated with an increased risk of cardiovascular (CV) diseases later in life. Subclinical cardiac alterations precede eminent CV diseases. Speckle-tracking echocardiography (STE) is an effective method to assess subclinical myocardial dysfunction. We performed a myocardial speckle tracking study to investigate the prevalence of subclinical myocardial dysfunction in former PE patients (with and without HELLP syndrome) compared to normotensive women affected by HELLP syndrome. In this cross-sectional retrospective study, women with a history of normotensive HELLP (n = 32), PE without HELLP (n = 59), and PE with HELLP (n = 101) underwent conventional and STE as part of the clinical CV work-up after their complicated pregnancies from 6 months to 4 years postpartum. We excluded women with comorbidities, including chronic hypertension, hypercholesterolemia, and obesity. Women with a history of PE with HELLP syndrome were characterized by a higher prevalence of altered left ventricular circumferential and global longitudinal two-dimensional (2D) strain (74 and 20%, respectively), altered right ventricular longitudinal 2D strain (37%), and left atrial (LA) 2D strain (57%). Moreover, a higher proportion of alterations of biventricular and LA strains was also present in former PE without HELLP as well as in the normotensive HELLP group. In the first years after a pregnancy complicated by HELLP syndrome, irrespective of whether there was concomitant PE, a higher rate of abnormal STE myocardial function is observed. Therefore, these women may benefit from CV risk management.

Maternal Risk Factors and Adverse Birth Outcomes Associated With HELLP Syndrome: A Population-based Study

(BJOG. 2020;127:1189–1198) HELLP (hemolysis, elevated liver enzymes and low platelets) syndrome has varying definitions and therefore a wide range of reported incidence. Only smaller studies, mostly in tertiary-care hospitals, have examined the risk factors and serious adverse outcomes associated with this syndrome. This study aimed to assess risk factors and adverse fetal and maternal outcomes associated with HELLP syndrome using a large population-based cohort.

A retrospective review of clinical and non-clinical factors associated with unscheduled cesarean deliveries at one urban medical center.

To investigate factors associated with unscheduled cesarean delivery at one urban tertiary medical center. A retrospective chart review was performed on 11162 deliveries between 2009 and 2019. The dependent variable was unscheduled cesarean delivery. Covariates examined included time of delivery, as well as several maternal and pregnancy-related factors. There were a total of 7037 (63.1%) vaginal, 1133 (10.1%) elective cesarean, and 2992 (26.8%) unscheduled cesarean deliveries. Independent factors associated with increased odds for unscheduled cesarean delivery included daytime delivery (odds ratio [OR] 1.29, 95% confidence interval [CI] 1.18-1.42, P<0.001); advanced maternal age (OR 1.40, 95% CI 1.26-1.56, P<0.001); obesity (OR 1.04, 95% CI 1.03-1.05, P<0.001); history of previous cesarean delivery (OR 2.77, 95% CI 1.91-4.01, P<0.001); hypertension (OR 1.72, 95% CI 1.27-2.32, P<0.001); multiparity (OR 3.99, 95% CI 2.82-5.64, P<0.001); pre-eclampsia (OR 1.96, 95% CI 1.33-2.89, P=0.001); and HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome (OR 5.45, 95% CI 1.13-26.28, P=0.035). Factors associated with unscheduled cesarean delivery in this study cohort included daytime delivery, advanced maternal age, obesity, hypertension, previous cesarean delivery, multiparity, preterm labor, pre-eclampsia, and HELLP syndrome.

May human epididymis 4 protein play a role in the etiopathogenesis of hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome?

Hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome is an extremely advanced form of preeclampsia. Currently, there is no parameter or marker to predict this syndrome; however, it is emphasized that vascular endothelial damage and abnormal immune responses can be the possible etiologies of HELLP syndrome. It is known that human epididymis protein 4 (HE4) is a protease inhibitor and previous studies have shown that HE4 protein levels are increased in many malignancies and inflammatory conditions. Considering that metalloproteinases may also play a role in endothelial damage, which is thought to be involved in the etiopathogenesis of HELLP syndrome, we thought that HE4 protein, which is a protease inhibitor, may be associated with vascular damage. We aimed to investigate the relationship between HELLP syndrome and HE4 protein and to identify a biomarker that can be utilized in the diagnosis of HELLP syndrome. In this study, 40 patients with HELLP syndrome and 40 healthy pregnant women with similar characteristics without HELLP syndrome were compared. When the results were evaluated, no statistically significant difference was found between serum HE4 protein levels in patients with HELLP syndrome and patients without HELLP syndrome in this study (p: 0.29). HE4 protein has no field of use in obstetrics yet. In our study, we aimed to find a new biomarker to identify patients with HELLP syndrome. However, we could not find any statistically significant difference in terms of HE4 protein levels between patients with and without HELLP syndrome. Our study is an important study as it is the first study aiming to use HE4 protein in obstetrics.

Early delivery or expectant management for late preterm preeclampsia: A meta-analysis of randomized controlled trials.

Currently, there is no consensus regarding the timing of delivery in women with non-severe preeclampsia at the late preterm period. The aim of the present meta-analysis is to compare expectant management with immediate delivery in pregnant women with preeclampsia between 34+0 and 36+6 gestational weeks, in terms of maternal and neonatal outcomes. A search was conducted until October 1, 2020 and eligible studies were identified in MEDLINE, Scopus, Cochrane Central Register of Controlled Trials (Central), the US Registry of clinical trials (www.clinicaltrials.gov), and sources of gray literature without limitations concerning the publication dates and languages. Randomized controlled trials, comparing planned delivery vs expectant management in women with preeclampsia at 34-37weeks were included. The primary outcomes were neonatal intensive care unit (NICU) admission and progression to eclampsia. Secondary outcomes were HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome, severe preeclampsia, composite adverse maternal outcome (defined by the presence of any of the potential preeclampsia-related complications, ranging from severe hypertension to maternal death), neonatal respiratory disease, and respiratory distress syndrome. Relative risks (RRs) and mean differences with 95% CI were used for the estimation of the effect sizes; the quality of the evidence was assessed per GRADE guidelines. The electronic search yielded 10721 potential studies, of which six were fully reviewed and three (1773 participants) were included in the meta-analysis. Immediate delivery increased the risk for NICU admission, (RR 1.23, 95% CI 1.05-1.45) and decreased the risk for the composite adverse maternal outcome (RR 0.86, 95% CI 0.78-0.93). There were no differences for eclampsia (RR 0.55, 95% CI 0.16-1.85), HELLP syndrome (RR 0.58, 95% CI 0.25-1.33), severe preeclampsia (RR 0.27, 95% CI 0.02-3.52), respiratory disease of neonate (RR 1.04, 95% CI 0.75-1.44), and respiratory distress syndrome (RR 2.3, 95% CI 0.73-7.25). Immediate delivery of women with non-severe preeclampsia at the period of late prematurity decreases the risk of a composite adverse maternal outcome by 14%, at the cost of an increase in NICU admissions by 23%. The overall quality of the evidence for these outcomes is high, indicating a high degree of certainty for the results.

Comparison of maternal and neonatal outcomes between acute fatty liver of pregnancy and hemolysis, elevated liver enzymes and low platelets syndrome: a retrospective cohort study

BackgroundAcute fatty liver of pregnancy (AFLP) and hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome are two uncommon disorders that mimic each other clinically, but are distinct pathophysiologically. This study aimed to compare maternal and neonatal outcomes between AFLP and HELLP syndrome.MethodsThis retrospective cohort study was performed at a tertiary referral center in Taiwan between June 2004 and April 2020. We used the Swansea Criteria to diagnose AFLP, and the Tennessee Classification System to diagnose HELLP syndrome. Maternal characteristics, laboratory data, complications, and neonatal outcomes were compared. We analyzed the categorical variables with Chi-square test or Fisher’s exact test and continuous variables with Student’s t test or Mann-Whitney U test. Subsequent logistic regression analyses adjusting by potential confounding factors with significant difference were analyzed.ResultsDuring the study period, 21 women had AFLP and 80 women had HELLP syndrome. There was a higher rate of preeclampsia (95.0 % versus 23.8 %) in the HELLP syndrome group compared to the AFLP group. However, the AFLP group had more other maternal complications including jaundice (85.7 % versus 13.8 %), acute kidney injury (61.9 % versus 15.0 %), disseminated intravascular coagulopathy (66.7 % versus 8.8 %), and sepsis (47.6 % versus 10.0 %) compared to the HELLP syndrome group. Nevertheless, higher rates of small for gestational age neonates (57.1 % versus 33.3 %), neonatal respiratory distress syndrome (39.2 % versus 8.3 %) and neonatal sepsis (34.2 % versus 12.5 %) were noted in the HELLP syndrome group.ConclusionsAFLP is associated with a higher rate of multiple organ dysfunction in mothers, whereas HELLP syndrome is associated with a higher rate of neonatal morbidity.

Magnetic Resonance Imaging in Peripartum Encephalopathy: A Pictorial Review.

Acute neurological problems are significant disorders of pre- and postpartum period in women. We analyzed the magnetic resonance imaging (MRI) profile of patients presenting with peripartum encephalopathy over 2 years. Of 51 patients, MRI was abnormal in 40 patients and normal in 11 patients. Posterior reversible encephalopathy (13/40), cerebral venous thrombosis (6/40), and postpartum angiopathy (3/40) are the three most common causes of peripartum encephalopathy as identified in MRI. The other less common but important etiology include HELLP (Hemolysis, Elevated Liver enzymes and Low Platelet) syndrome (2), osmotic demyelination (2), antiphospholipid syndrome (2), tubercular meningitis/cerebritis (3), pituitary hyperplasia with hemorrhage (2), postictal edema (2), cerebellitis (1), transient splenial lesion (1), and changes of old trauma and stroke (one each).

Liver transaminase levels during pregnancy: a Japanese multicenter study

Introduction There are conflicting reports on the effect of pregnancy on liver transaminase (aspartate aminotransferase [AST] and alanine aminotransferase [ALT]) levels. In this study, we sought to investigate the trajectories of AST and ALT levels during normal pregnancy and to compare them with AST and ALT levels of matched nonpregnant controls. Materials and Methods Our multicenter retrospective study included 34,396 women who delivered at term at 12 primary maternity care units between January 2011 and December 2018 and 57,152 nonpregnant women younger than 45 years who received a medical checkup between 2016 and 2019. After matching at a ratio of 1:1 for adjustment of several factors (age, weight, and height), a total of 30,460 normal pregnant women and 30,460 nonpregnant women were selected for this study. We measured serum AST and ALT levels during each trimester and the postpartum period to compare with those of the nonpregnant women. Results The ALT level began to decrease in the first half of the third trimester and was lowest in the second half of third trimester and at postpartum day 1 (median [interquartile range]: 8 [6–11] U/L, 8 [6–10] U/L, respectively). The decline reversed and returned to the level of a nonpregnant state by postpartum days 2–7. The AST level remained unchanged regardless of pregnancy. The prevalence of abnormal liver transaminases (AST >40 U/L and ALT >40 U/L) was <1% at third trimester; however, it increased to 3–5% on postpartum days 2–7. Conclusions The ALT level was lower during pregnancy compared with nonpregnant women matched for several factors, whereas the AST level remained unchanged during pregnancy. Understanding the trajectories of AST and ALT levels during pregnancy may facilitate early recognition and diagnosis of impaired liver function, including liver disease and pregnancy complications that affect liver transaminases, such as pre-eclampsia and HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome.

New indicators in evaluation of hemolysis, elevated liver enzymes, and low platelet syndrome: A case-control study.

BACKGROUNDIndices such as the neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), mean platelet volume (MPV), platelet distribution width (PDW), and red cell distribution width (RDW) are considered new markers of the systemic inflammatory response (SIR), and have been widely implemented for the diagnosis of patients with inflammatory diseases. These new indicators have also been widely investigated in preeclampsia (PE) but less analyzed in hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome.AIMTo compare SIR markers among HELLP patients, PE only patients, and healthy gravidae.METHODSThis retrospective case-control study enrolled 630 cases, including 210 patients with HELLP syndrome (HELLP group), 210 patients with only PE (PE group) and 210 healthy gravidae (control group). The three groups were matched by age, parity, status of assisted reproduction, and multiple pregnancies. Birthweight, gestational age at complete blood count collection, gestational age at delivery, mode of delivery, etc. were recorded. The main indices as NLR, PLR, MPV, PDW, and RDW among the groups were compared, as well as some secondary outcomes including neutrophil, platelets, and hemoglobin.RESULTSThe NLR (6.4 vs 4.3 vs 3.5), MPV (11.9 vs 11.2 vs 10.7), PDW (16.4 vs 13.3 vs 14.2), leukocyte (12.4 × 109/L vs 9.7 × 109/L vs 8.7 × 109/L) and neutrophil count (9.9 × 109/L vs 7.3 × 109/L vs 6.1 × 109/L) were highest in the HELLP group, lower in the PE group, and lowest in the control group. Both the overall comparisons between the three groups (all bP < 0.01) and pairwise comparisons between every two groups elicited statistically significant differences (all dP < 0.01, except control vs PE: cP < 0.05 in PDW). The average lymphocyte counts were 1.4 (1.1, 2.0) × 109/L in the HELLP group, 1.6 (1.3, 2.0) × 109/L in the PE group and 1.7 (1.4, 2.0) × 109/L in the control group. The overall comparison of lymphocyte count within the three groups had statistically significant differences (P = 0.000). The pairwise comparisons between every two groups demonstrated that the HELLP group had a lower lymphocyte count than both the PE (P = 0.019) and control groups (P = 0.000), but the difference between the PE and control groups was not statistically significant (P = 0.432). The overall comparisons on platelet counts and the PLR among these three groups also showed statistically significant differences (both P = 0.000), from low to high being those in the HELLP group (43.4 × 109/L, 64.0), control group (180.5 × 109/L, 103.6) and PE group (181.5 × 109/L, 112.8). Pairwise comparisons of neither index displayed statistically significant differences between the PE and control groups (both P > 0.05), while the differences in the two indices between the HELLP group and the two other groups were still statistically significant (all P = 0.000). RDW values were highest in the HELLP group (14.5% [13.6, 15.3]), lower in the control group (14.1% [13.5, 14.8]) and lowest in the PE group (13.9% [13.4, 14.9]). The difference between the PE and control group did not show statistical significance (P = 1.000), while RDW values in the HELLP group were higher than those in the other two groups (cP < 0.05 vs control, dP < 0.01 vs PE).CONCLUSIONSIR markers such as NLR, RDW, MPV, and PDW were increased and PLR was decreased in HELLP. These SIR markers may become new indicators in the evaluation of HELLP syndrome.

Disorders of placental villous maturation are present in one-third of cases with spontaneous preterm labor.

Spontaneous preterm labor is an obstetrical syndrome accounting for approximately 65-70% of preterm births, the latter being the most frequent cause of neonatal death and the second most frequent cause of death in children less than five years of age worldwide. The purpose of this study was to determine and compare to uncomplicated pregnancies (1) the frequency of placental disorders of villous maturation in spontaneous preterm labor; (2) the frequency of other placental morphologic characteristics associated with the preterm labor syndrome; and (3) the distribution of these lesions according to gestational age at delivery and their severity. A case-control study of singleton pregnant women was conducted that included (1) uncomplicated pregnancies (controls, n=944) and (2) pregnancies with spontaneous preterm labor (cases, n=438). All placentas underwent histopathologic examination. Patients with chronic maternal diseases (e.g., chronic hypertension, diabetes mellitus, renal disease, thyroid disease, asthma, autoimmune disease, and coagulopathies), fetal malformations, chromosomal abnormalities, multifetal gestation, preeclampsia, eclampsia, preterm prelabor rupture of the fetal membranes, gestational hypertension, gestational diabetes mellitus, and HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome were excluded from the study. Compared to the controls, the most prevalent placental lesions among the cases were the disorders of villous maturation (31.8% [106/333] including delayed villous maturation 18.6% [62/333] vs. 1.4% [6/442], q<0.0001, prevalence ratio 13.7; and accelerated villous maturation 13.2% [44/333] vs. 0% [0/442], q<0.001). Other lesions in decreasing order of prevalence included hypercapillarized villi (15.6% [68/435] vs. 3.5% [33/938], q<0.001, prevalence ratio 4.4); nucleated red blood cells (1.1% [5/437] vs. 0% [0/938], q<0.01); chronic inflammatory lesions (47.9% [210/438] vs. 29.9% [282/944], q<0.0001, prevalence ratio 1.6); fetal inflammatory response (30.1% [132/438] vs. 23.2% [219/944], q<0.05, prevalence ratio 1.3); maternal inflammatory response (45.5% [195/438] vs. 36.1% [341/944], q<0.01, prevalence ratio 1.2); and maternal vascular malperfusion (44.5% [195/438] vs. 35.7% [337/944], q<0.01, prevalence ratio 1.2). Accelerated villous maturation did not show gestational age-dependent association with any other placental lesion while delayed villous maturation showed a gestational age-dependent association with acute placental inflammation (q-value=0.005). Disorders of villous maturation are present in nearly one-third of the cases of spontaneous preterm labor.

HELLP Syndrome with Unusual Features in a Rare Setting

HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelet) syndrome is a rare disorder exclusively associated with pregnancy. It occurs mostly during the antepartum period in about 70% of cases but could also occur during the post-partum period in about 30% of reported cases especially during the first 48 hours following delivery. The syndrome is mostly reported among multiparous Caucasians in association with severe preeclampsia, hypertension, and proteinuria. It is rarely reported within Nigeria and the post-partum variant has never been documented among women of Nigerian origin in the literature to date. Herein, we report a very unusual case of HELLP syndrome in a very rare setting that developed eleven days post-partum without the usual features of hypertension and proteinuria in a 26-year-old G1P1. Upon presentation, she was promptly diagnosed and aggressively managed using standard protocols by a multidisciplinary medical team of specialists. She developed acute kidney injury (KDIGO stage 1) in the course of management of which she required conservative management. Remarkably, with supportive care, all the clinical and laboratory derangements, including hepatic and renal functions, normalized after seven days on admission. Her condition remained stable during weekly follow-up visits for four consecutive weeks. This case and its management protocols reinforce the need for a high index of clinical suspicion for this dreaded disorder and its prompt diagnosis/aggressive management even in very rare circumstances and settings.