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Medicina (Kaunas, Lithuania) Feb 2022HELLP syndrome, also known as the syndrome of hemolysis, elevated liver enzymes, and low platelets, represents a severe pregnancy complication typically associated with... (Review)
Review
HELLP syndrome, also known as the syndrome of hemolysis, elevated liver enzymes, and low platelets, represents a severe pregnancy complication typically associated with hypertension. It is associated with increased risks of adverse complications for both mother and fetus. HELLP occurs in 0.2-0.8% of pregnancies, and, in 70-80% of cases, it coexists with preeclampsia (PE). Both of these conditions show a familial tendency. A woman with a history of HELLP pregnancy is at high risk for developing this entity in subsequent pregnancies. We cannot nominate a single worldwide genetic cause for the increased risk of HELLP. Combinations of multiple gene variants, each with a moderate risk, with concurrent maternal and environmental factors are thought to be the etiological mechanisms. This review highlights the significant role of understanding the underlying pathophysiological mechanism of HELLP syndrome. A better knowledge of the disease's course supports early detection, an accurate diagnosis, and proper management of this life-threatening condition.
Topics: Female; HELLP Syndrome; Humans; Hypertension; Pre-Eclampsia; Pregnancy; Pregnancy Complications
PubMed: 35208649
DOI: 10.3390/medicina58020326 -
Hipertension Y Riesgo Vascular 2020
Topics: Female; HELLP Syndrome; Humans; Pregnancy; Prognosis
PubMed: 32811776
DOI: 10.1016/j.hipert.2020.07.002 -
American Journal of Obstetrics and... Feb 2022Preeclampsia, one of the most enigmatic complications of pregnancy, is considered a pregnancy-specific disorder caused by the placenta and cured only by delivery. This... (Review)
Review
Preeclampsia, one of the most enigmatic complications of pregnancy, is considered a pregnancy-specific disorder caused by the placenta and cured only by delivery. This article traces the condition from its origins-once thought to be a disease of the central nervous system, recognized by the occurrence of seizures (ie, eclampsia)-to the present time when preeclampsia is conceptualized primarily as a vascular disorder. We review the epidemiologic data that led to the recommendation to use diastolic hypertension and proteinuria as diagnostic criteria, as their combined presence was associated with an increased risk of fetal death and the birth of small-for-gestational-age neonates. However, preeclampsia is a multisystemic disorder with protean manifestations, and the condition can be present even in the absence of hypertension and proteinuria. Toxins gaining access to the maternal circulation have been proposed to mediate the clinical manifestations-hence, the term "toxemia of pregnancy," which was used for several decades. The search for putative toxins has challenged investigators for more than a century, and a growing body of evidence suggests that products of an ischemic or a stressed placenta are responsible for the vascular changes that characterize this syndrome. The discovery that the placenta can produce antiangiogenic factors, which regulate endothelial cell function and induce intravascular inflammation, has been a major step forward in the understanding of preeclampsia. We view the release of antiangiogenic factors by the placenta as an adaptive response to improve uterine perfusion by modulating endothelial function and maternal cardiovascular performance. However, this homeostatic response can become maladaptive and lead to damage of target organs during pregnancy or the postpartum period. Early-onset preeclampsia has many features in common with atherosclerosis, whereas late-onset preeclampsia seems to result from a mismatch of fetal demands and maternal supply, that is, a metabolic crisis. Preeclampsia, as it is understood today, is essentially vascular dysfunction unmasked or caused by pregnancy. A subset of patients diagnosed with preeclampsia are at greater risk of the subsequent development of hypertension, ischemic heart disease, heart failure, vascular dementia, and end-stage renal disease. However, these adverse events may be the result of a preexisting vascular pathologic process; it is not known if the occurrence of preeclampsia increases the baseline risk. Therefore, the understanding, prediction, prevention, and treatment of preeclampsia are healthcare priorities.
Topics: Albuminuria; Eclampsia; Edema; Female; Fetal Mortality; Gene-Environment Interaction; HELLP Syndrome; History, 19th Century; History, Ancient; Humans; Placenta; Placenta Growth Factor; Pre-Eclampsia; Pregnancy; Proteinuria; Puerperal Disorders; Seizures; Severity of Illness Index; Terminology as Topic; Vascular Endothelial Growth Factor Receptor-1
PubMed: 35177220
DOI: 10.1016/j.ajog.2021.12.001 -
BMC Pregnancy and Childbirth Feb 2009The HELLP syndrome is a serious complication in pregnancy characterized by haemolysis, elevated liver enzymes and low platelet count occurring in 0.5 to 0.9% of all... (Review)
Review
BACKGROUND
The HELLP syndrome is a serious complication in pregnancy characterized by haemolysis, elevated liver enzymes and low platelet count occurring in 0.5 to 0.9% of all pregnancies and in 10-20% of cases with severe preeclampsia. The present review highlights occurrence, diagnosis, complications, surveillance, corticosteroid treatment, mode of delivery and risk of recurrence.
METHODS
Clinical reports and reviews published between 2000 and 2008 were screened using Pub Med and Cochrane databases.
RESULTS AND CONCLUSION
About 70% of the cases develop before delivery, the majority between the 27th and 37th gestational weeks; the remainder within 48 hours after delivery. The HELLP syndrome may be complete or incomplete. In the Tennessee Classification System diagnostic criteria for HELLP are haemolysis with increased LDH (> 600 U/L), AST (>or= 70 U/L), and platelets < 100 x 10(9)/L. The Mississippi Triple-class HELLP System further classifies the disorder by the nadir platelet counts. The syndrome is a progressive condition and serious complications are frequent. Conservative treatment (>or= 48 hours) is controversial but may be considered in selected cases < 34 weeks' gestation. Delivery is indicated if the HELLP syndrome occurs after the 34th gestational week or the foetal and/or maternal conditions deteriorate. Vaginal delivery is preferable. If the cervix is unfavourable, it is reasonable to induce cervical ripening and then labour. In gestational ages between 24 and 34 weeks most authors prefer a single course of corticosteroid therapy for foetal lung maturation, either 2 doses of 12 mg betamethasone 24 hours apart or 6 mg or dexamethasone 12 hours apart before delivery. Standard corticosteroid treatment is, however, of uncertain clinical value in the maternal HELLP syndrome. High-dose treatment and repeated doses should be avoided for fear of long-term adverse effects on the foetal brain. Before 34 weeks' gestation, delivery should be performed if the maternal condition worsens or signs of intrauterine foetal distress occur. Blood pressure should be kept below 155/105 mmHg. Close surveillance of the mother should be continued for at least 48 hours after delivery.
Topics: Delivery, Obstetric; Dexamethasone; Diagnosis, Differential; Female; Fetal Organ Maturity; Glucocorticoids; HELLP Syndrome; Humans; Lung; Pregnancy
PubMed: 19245695
DOI: 10.1186/1471-2393-9-8 -
International Journal of Molecular... Mar 2022Preeclampsia (PE) constitutes one of the principal reasons for maternal and perinatal morbidity and mortality worldwide. The circumstance typically implicates formerly... (Review)
Review
Preeclampsia (PE) constitutes one of the principal reasons for maternal and perinatal morbidity and mortality worldwide. The circumstance typically implicates formerly healthful normotensive women, after 20 weeks of gestation, typically withinside the third trimester, without regarded threat elements or past deliveries. PE can be further complicated with hemolysis and thrombocytopenia, leading to the emergence of HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low platelets). Both conditions are classified as hypertensive diseases of pregnancy (HDP), and their pathogenesis has been linked to an excessive maternal inflammatory response, accompanied by enhanced endothelial activation. Several studies have found that in pregnancies affected by PE/HELLP, von Willebrand factor (vWF) antigen levels (vWF:Ag) are significantly elevated, while its cleaving protease (ADAMTS-13, A Disintegrin-like and Metalloprotease with Thrombospondin type 1 motif, member 13) activity is normal to decreased. Furthermore, the higher urine excretion of the terminal complement complex C5b-9, as well as its greater deposition in the placental surface in preeclamptic women, imply that the utero-placental unit's distinctive deficits are intimately tied to disproportionate complement activation. The goal of this updated evaluation is to provide the most up-to-date molecular advances in the pathophysiology of PE/HELLP syndromes. Recent medical data on vWF:Ag levels in patients with PE, ADAMTS-13, and dysregulation of the complement system, are highlighted and evaluated. Furthermore, we discuss the relationship between those entities and the progression of the disease, as well as their significance in the diagnostic process. Finally, considering the difficulties in analyzing and controlling those symptoms in pregnant women, we can provide a current diagnostic and therapeutic algorithm.
Topics: ADAMTS13 Protein; Female; HELLP Syndrome; Hemolysis; Humans; Placenta; Pre-Eclampsia; Pregnancy; von Willebrand Factor
PubMed: 35409211
DOI: 10.3390/ijms23073851 -
Blood Reviews Jul 2017The complement system is an essential part of the innate immune system that requires careful regulation to ensure responses are appropriately directed against harmful... (Review)
Review
The complement system is an essential part of the innate immune system that requires careful regulation to ensure responses are appropriately directed against harmful pathogens, while preventing collateral damage to normal host cells and tissues. While deficiency in some components of the complement pathway is associated with increased susceptibility to certain infections, it has also become clear that inappropriate activation of complement is an important contributor to human disease. A number of hematologic disorders are driven by complement, and these disorders may be termed "complementopathies". This includes paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome (aHUS), cold agglutinin disease (CAD) and other related disorders, which will be the focus of this review. A better understanding of the central role of the complement system in the pathophysiology of these disorders may allow for application of therapies directed at blocking the complement cascade.
Topics: Adaptive Immunity; Anemia, Hemolytic, Autoimmune; Animals; Atypical Hemolytic Uremic Syndrome; Complement Activation; Complement System Proteins; Female; HELLP Syndrome; Hemoglobinuria, Paroxysmal; Humans; Immunity, Innate; Pregnancy
PubMed: 28215731
DOI: 10.1016/j.blre.2017.02.003 -
European Journal of Case Reports in... 2022Haemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome is a leading cause of maternal mortality. The emergence of coronavirus disease 2019 (COVID-19)...
INTRODUCTION
Haemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome is a leading cause of maternal mortality. The emergence of coronavirus disease 2019 (COVID-19) has led to challenges in diagnosing HELLP syndrome due to overlapping clinical and laboratory presentations. We report a case of HELLP syndrome complicated by COVID-19 infection.
CASE DESCRIPTION
An otherwise healthy pregnant 31-year-old woman presented with fever, myalgia and headache. She was found to be COVID-positive with laboratory signs of HELLP syndrome. Symptoms and laboratory findings trended toward normal after delivery confirming the diagnosis of HELLP syndrome.
DISCUSSION
A prompt diagnosis of HELLP syndrome is essential to avoid maternal and fetal complications. Clinicians should be aware of the similarities in presentation between HELLP syndrome and COVID-19 for timely diagnosis and treatment.
LEARNING POINTS
SARS-CoV-2 preferentially binds to ACE2 which is expressed in extrapulmonary tissue including placental tissue.COVID-19, HELLP syndrome and preeclampsia may have similar characteristics including elevated blood pressures, liver dysfunction, cardiopulmonary complaints and hypercoagulability.The temporal relationship of symptomatic improvement with delivery and after delivery may better differentiate HELLP syndrome from COVID-19.
PubMed: 36299845
DOI: 10.12890/2022_003540 -
BMJ (Clinical Research Ed.) Sep 2018To determine the efficacy of high dose folic acid supplementation for prevention of pre-eclampsia in women with at least one risk factor: pre-existing hypertension,... (Randomized Controlled Trial)
Randomized Controlled Trial
OBJECTIVE
To determine the efficacy of high dose folic acid supplementation for prevention of pre-eclampsia in women with at least one risk factor: pre-existing hypertension, prepregnancy diabetes (type 1 or 2), twin pregnancy, pre-eclampsia in a previous pregnancy, or body mass index ≥35.
DESIGN
Randomised, phase III, double blinded international, multicentre clinical trial.
SETTING
70 obstetrical centres in five countries (Argentina, Australia, Canada, Jamaica, and UK).
PARTICIPANTS
2464 pregnant women with at least one high risk factor for pre-eclampsia were randomised between 2011 and 2015 (1144 to the folic acid group and 1157 to the placebo group); 2301 were included in the intention to treat analyses.
INTERVENTION
Eligible women were randomised to receive either daily high dose folic acid (four 1.0 mg oral tablets) or placebo from eight weeks of gestation to the end of week 16 of gestation until delivery. Clinicians, participants, adjudicators, and study staff were masked to study treatment allocation.
MAIN OUTCOME MEASURE
The primary outcome was pre-eclampsia, defined as hypertension presenting after 20 weeks' gestation with major proteinuria or HELLP syndrome (haemolysis, elevated liver enzymes, low platelets).
RESULTS
Pre-eclampsia occurred in 169/1144 (14.8%) women in the folic acid group and 156/1157 (13.5%) in the placebo group (relative risk 1.10, 95% confidence interval 0.90 to 1.34; P=0.37). There was no evidence of differences between the groups for any other adverse maternal or neonatal outcomes.
CONCLUSION
Supplementation with 4.0 mg/day folic acid beyond the first trimester does not prevent pre-eclampsia in women at high risk for this condition.
TRIAL REGISTRATION
Current Controlled Trials ISRCTN23781770 and ClinicalTrials.gov NCT01355159.
Topics: Adult; Argentina; Australia; Canada; Diabetes, Gestational; Dietary Supplements; Double-Blind Method; Female; Folic Acid; HELLP Syndrome; Humans; Hypertension; Jamaica; Pre-Eclampsia; Pregnancy; Proteinuria; Risk Factors; United Kingdom; Vitamin B Complex; Young Adult
PubMed: 30209050
DOI: 10.1136/bmj.k3478 -
Frontiers in Immunology 2020Innate and adaptive immune involvement in hemolysis, elevated liver enzymes and low platelet (HELLP) syndrome is an understudied field, although it is of high clinical... (Review)
Review
Innate and adaptive immune involvement in hemolysis, elevated liver enzymes and low platelet (HELLP) syndrome is an understudied field, although it is of high clinical importance. This syndrome implies a risk of serious morbidity and mortality to both the mother and the fetus during pregnancy. It was proposed that HELLP syndrome occurs in a circulatory inflammatory milieu, that might in turn participate in a complex interplay between the secreted inflammatory immunomodulators and immune cell surface receptors. Meanwhile, reported immune cell attenuation during HELLP may consequently lead to a prolonged immunoactivation and tissue damage. In this regard, learning more about the immune components of this syndrome should widen the understanding of the HELLP pathophysiology and eventually enable development of novel immune-based therapeutics. This review aims to summarize and discuss the recent and previous findings of the innate and adaptive immune responses during HELLP in order to update the current knowledge of the immune involvement in HELLP pathogenesis.
Topics: Adaptive Immunity; Animals; Complement System Proteins; Female; HELLP Syndrome; Humans; Immunity, Innate; Immunotherapy; Pregnancy; Pregnancy Complications
PubMed: 32351511
DOI: 10.3389/fimmu.2020.00667