HELLP Syndrome at 20 Gestational Weeks Managed Using the Mississippi Protocol: A Case Report.

Hemolysis, elevated liver enzyme levels, and low platelet count (HELLP) syndrome is one of the most severe complications of hypertensive disorders of pregnancy. HELLP syndrome occurring before 22 gestational weeks (GWs) is extremely rare, and patients prevalently exhibit underlying maternal diseases or fetal abnormalities. Here, we report the case of a pregnant woman who had HELLP syndrome at 20 GWs without any obvious underlying maternal diseases or fetal abnormalities. A 38-year-old pregnant woman was referred to Kobe University Hospital from another hospital at 19 + 5/7 GWs for hypertension, proteinuria, generalized edema, and fetal growth restriction. She was diagnosed with partial HELLP syndrome according to the Mississippi classification at 20 + 2/7 GWs. The patient was managed following the Mississippi protocol, including intravenous dexamethasone, magnesium sulfate, and antihypertensive drugs. She received intensive blood pressure and laboratory data monitoring using an arterial line and additional treatments, including platelet transfusion, intravenous haptoglobin infusion, and human atrial natriuretic peptide. The pregnancy ended in an induced delivery at 20 + 3/7 GWs, and she was discharged without complications 10 days postnatal. We performed laboratory tests for diagnosing underlying diseases but identified no obvious underlying diseases. This report indicates that early and intensive treatment of patients with HELLP syndrome occurring before 22 GWs according to the Mississippi protocol may enable clinicians to complete pregnancy termination without maternal complications and provide useful information to clinical practitioners in perinatal medicine.

Labor and delivery: DIC, HELLP, preeclampsia.

Hematologists are often needed to assist with the management of microangiopathic emergencies in pregnancy. A firm understanding of the diagnosis and management of preeclampsia with severe features, hemolysis elevated liver enzyme and low platelet syndrome, and disseminated intravascular coagulation, which are the most common causes of microangiopathic emergencies, is critical. However, being able to consider when other microangiopathic emergencies (acute fatty liver of pregnancy, congenital and acquired thrombotic thrombocytopenic purpura, complement mediated microangiopathy, antiphospholipid syndrome) should be considered is imperative. The hematologist and obstetric team should work together to optimize the care of common as well as rare hematologic emergencies.

[Nephrotic syndrome during pregnancy. Is it chronic glomerulonephritis or preeclampsia? Case report].

Nephrotic syndrome (NS) during pregnancy is a fairly rare pathology and its descriptions in the literature are few. For a long time, NS was associated only with an exacerbation of chronic glomerulonephritis or de novo nephritis, however, the experience of recent years has shown that NS can be a manifestation of the classical obstetric pathology - preeclampsia (PE). The appearance of massive proteinuria with the development of NS is most typical for early PE, which, of course, makes diagnosis difficult, especially if PE develops at an unusually early time (up to 20 weeks). To describe PE that does not fit into the classical criteria, the term "atypical" PE is now used, the development of which can be promoted by both obstetric and somatic risk factors. The presented clinical observation describes the development of early (within 14 weeks) severe PE with the NS at the onset of the disease in a patient with the first multiple pregnancy and complete hydatidiform mole (HM) of one of the fetuses. The progression of nephropathy with the addition of thrombotic microangiopathy and HELLP syndrome made it possible to assume the diagnosis of PE with a high probability. The rapid relief of all clinical manifestations after delivery confirmed this assumption. The role of HM as the main trigger of unusually early PE is discussed. Apparently, the patient's trophoblast disease in the form of hydatidiform mole caused the formation of a severe angiogenic imbalance already in the early stages of pregnancy, which led to the development of PE, which manifested NS as a consequence of podocytopathy due to VEGF deficiency. Thus, the development of NS in a pregnant patient without a history of kidney disease dictates, first of all, the exclusion of PE, until proven otherwise.

Beckwith-Wiedemann Syndrome in Newborn of Mother with HELLP Syndrome/Preeclampsia: An Analysis of Literature and Case Report with Fetal Growth Restriction and Absence of CDKN1C Typical Pathogenic Genetic Variation.

Beckwith-Wiedemann Syndrome (BWS) is an imprinting disorder, which manifests by overgrowth and predisposition to embryonal tumors. The evidence on the relationship between maternal complications such as HELLP (hemolysis, elevated liver enzymes, and low platelet count) and preeclampsia and the development of BWS in offspring is scarce. A comprehensive clinical evaluation, with genetic testing focused on screening for mutations in the CDKN1C gene, which is commonly associated with BWS, was conducted in a newborn diagnosed with BWS born to a mother with a history of preeclampsia and HELLP syndrome. The case study revealed typical clinical manifestations of BWS in the newborn, including hemihyperplasia, macroglossia, midfacial hypoplasia, omphalocele, and hypoglycemia. Surprisingly, the infant also exhibited fetal growth restriction, a finding less commonly observed in BWS cases. Genetic analysis, however, showed no mutations in the CDKN1C gene, which contrasts with the majority of BWS cases. This case report highlights the complex nature of BWS and its potential association with maternal complications such as preeclampsia and HELLP syndrome. The atypical presence of fetal growth restriction in the newborn and the absence of CDKN1C gene mutations have not been reported to date in BWS.

The HELLP syndrome: clinical issues and surgical management. A Case Experience.

AIM: This study aims to challenge the current know-how in patients with spontaneous rupture of a liver hematoma, to differentiate amongst patients requiring such specific surgical therapy and avoiding mistakes during surgical operations, in order to terminate pregnancy with beneficial effects on the mother and fetus. MATERIALS AND METHODS: In a emergency scenario we admitted a 37-year-old woman at 35+4 weeks of gestation for emergency cesarean section after the onset of right hypochondrium pain. A diagnosis of hemoperitoneum and severe preeclampsia with liver and splenic bleeding was done and managed with packing of hepatic and splenic hematomas and according to her haemo-dynamic clinical conditions, done in different time. RESULTS: A diagnosis of hemoperitoneum and severe pre-eclampsia with liver and splenic bleeding was done and managed it with 3 xypho-pubic-laparatomy in different time with haemostatic packing. DISCUSSION: In this case report, the patient underwent an emergency caesarean section and was managed with packing of hepatic and splenic hematomas and according to her haemodynamic clinical conditions was operated in different time. The choice of laparotomy and hepatic packing has proved to be a viable option in patients with unstable vital signs and is feasible even in limited resource settings. CONCLUSION: Short interval between diagnosis and management may enhance the feto-maternal survival rate and prevent further morbidity or mortality. The choice of laparotomy and hepatic packing has proved to be a viable option in patients with unstable vital signs and is feasible even in limited resource settings. KEY WORDS: HELLP syndrome, Liver hematoma rupture, Packing.

Can SIRI or other inflammatory indices predict HELLP syndrome in the first trimester?

OBJECTIVE: To investigate the use of systemic immune-response index (SIRI) and other inflammatory indices for the prediction of HELLP syndrome STUDY DESIGN: The presented retrospective case-control study was conducted with twenty-eight pregnant women diagnosed with HELLP syndrome and 100 low-risk pregnant women. The possible predictive indices for HELLP syndrome were determined as NLR (neutrophil/lymphocyte), MLR (monocyte/lymphocyte), HbLR (hemoglobin/lymphocyte), SII (neutrophil×platelet/lymphocyte), and SIRI (neutrophil×monocyte/lymphocyte). The indices were evaluated in the first trimester and at the admission time for delivery for all participants. The statistical analyses were carried out using SPSS 23. Descriptive statistics were presented as the mean and standard deviation (SD), as they conform to a normal distribution. To compare the parameters between the groups, the Student-t test was used. Categorical variables were presented as numbers and percentages. The chi-square test was used to compare categorical variables between groups. The paired sample t-test was used to compare correlated samples. Statistical significance was defined as a two-tailed P value of 0.05. RESULTS: In the first trimester; WBC, neutrophil, and monocyte counts were statistically higher in the HELLP syndrome group. However, no significant difference was observed between the groups for the concerned indices. The hemoglobin, WBC, neutrophil, monocyte counts, NLR, SIRI and MLR were significantly higher in the HELLP group at the delivery time. Platelet count was decreased and ALT/AST counts and adverse outcomes were found to be significantly higher at delivery time admission in the HELLP syndrome group. CONCLUSION: To the best of our knowledge, this was the first study investigating SIRI with the other indices for the prediction of HELLP syndrome in accordance with its inflammatory etiology. The underlying inflammatory process was observed at the delivery time. However, none of the investigated indices was found effective in the first trimester in the prediction. Simple and non-invasive prediction indices might be valuable tools for the prediction and management of HELLP syndrome. Further and larger studies are needed for this purpose.

Value of proteinuria in evaluating the severity of HELLP and its maternal and neonatal outcomes.

BACKGROUND: HELLP syndrome refers to a group of clinical syndromes characterized by hemolysis, elevated liver enzymes and low platelet, and the evidence on the association between proteinuria and the severity of HELLP and its maternal and neonatal outcomes is rare. METHODS: 106 pregnant women were assigned to the proteinuric group (24-hUPro ≥ 0.3 g, 79 cases) and the non-proteinuric group (24-hUPro < 0.3 g, 27 cases). The proteinuric group was further divided into three subgroups: mild group (24-hUPro:0.3-2.0 g, 33 cases), moderate group (24-hUPro:2.0-5.0 g, 21 cases) and severe group (24-hUPro: ≥5.0 g, 25 cases). The general clinical data, laboratory indexes, complications and pregnancy outcome and adverse neonatal outcomes of HELLP with or without proteinuric were analyzed. RESULTS: Compared with proteinuric group, the non-albuminuric group or in the three proteinuric subgroups of HELLP pregnant women's, increased proteinuria was associated with earlier onset gestations, higher incidence of abdominal pain, skin jaundice, headache, blurred vision (p < 0.05 respectively), and also the higher levels of ALT, AST, LDH, Fib, APTT, ATII, proportions of tubular urine and lower levels of ALB, PLT (p < 0.05 respectively). In the three subgroups of the proteinuric group, the ratio of fetal growth restriction, cesarean section and postpartum hemorrhage were compared, and the difference was statistically significant (p < 0.05 respectively). Compared with the proteinuric group, the non-proteinuric group had higher birth weight, birth length, and lower SGA, admission rate in NICU (p < 0.05 respectively). In the three subgroups of the proteinuric group, significant differences were identified in the adverse outcomes of newborns (p < 0.05 respectively), and the incidence of adverse outcomes in neonates tended to be higher. Significant differences were identified in birth weight, birth length, and lower SGA and NICU occupancy rate among the three subgroups (p < 0.05 respectively). CONCLUSIONS: HELLP syndrome is a severe complication of pregnancy, involving multiple systems of the whole body. It has posed a great challenge to obstetricians for its acute onset, dangerous condition, rapid progress, and great harm. Thus, insights into HELLP syndrome should be gained, and early diagnosis, early treatment and timely termination of pregnancy should be conducted to reduce the incidence of maternal and fetal adverse outcomes and improve maternal and fetal prognosis.

[Acute Fatty Liver of Pregnancy - Case Series]. / Akute Schwangerschaftsfettleber ­ Fallserie einer seltenen Schwangerschaftskomplikation.

With a prevalence of 0,01-0,03%, acute fatty liver in pregnancy (AFLP) is a rare and dangerous complication of pregnancy and is difficult to distinguish from other, sometimes more common, pregnancy diseases such as HELLP syndrome, aHUS and TTP because of its mostly non-specific symptoms. Due to its rarity, AFLP is often not obvious to the obstetrician as a possible differential diagnosis. Yet early diagnosis and the fastest possible delivery is the only causal therapy and is important for the mortality rate. In the present manuscript, the pathophysiology, diagnosis and therapy of acute fatty liver in pregnancy are highlighted for the clinical routine based on case descriptions from three university hospitals, and reference is made to possible findings that are helpful in establishing the diagnosis. The angiogenic preeclampsia marker sFlt-1 plays a role and provides new opportunities to consider pathophysiological approaches.

Severe hepatic rupture in HELLP syndrome.

OBJECTIVE: To describe the multidisciplinary approach that led to the successful management of severe hepatic rupture in HELLP syndrome at 35 weeks of gestation. CASE REPORT: The clinical course and management procedure of a 34-year-old female patient with ruptured liver due to HELLP syndrome, who was admitted with symptoms lasting about 4 hours (pain in the right hypochondrium, nausea, vomiting, flashes before the eyes) are described in the form of a case report. An acute caesarean section was performed, during which a rupture of the subcapsular hematoma of the liver was dia-gnosed. Subsequently, the patient developed hemorrhagic shock and coagulopathy with the need for repeated surgical revisions of bleeding from the rupture of the liver. CONCLUSION: Subcapsular hematoma rupture is a rare but serious complication of HELLP syndrome. This case shows the importance of early dia-gnosis and prompt termination of pregnancy in the shortest possible time in pregnancy after 34 weeks. The most fundamental factor that influenced the patient's outcome and morbidity was the management of multidisciplinary cooperation and the correct timing of individual steps.

Segmental hair metabolomics analysis in pregnant women with pregnancy complications.

INTRODUCTION: Pregnancy complications, as preeclampsia (PE) and HELLP syndrome, occurring with similar pathophysiological mechanisms, have adverse effects on the health of both mother and fetus during pregnancy and thereafter, they are leading causes of maternal and fetal morbidity and mortality. The hair metabolome has been recognized as a valuable source of information in pregnancy research, as it provides stable metabolite information to be able to assist with studying biomarkers or metabolic mechanisms of pregnancy and its complications. OBJECTIVE: The aim of this study was to investigate the hair metabolome profile of pregnant women with PE, HELLP syndrome and healthy women. METHOD: Hair samples of new-borns' mothers (patients and controls) were investigated segmentally relevant to each trimester using a proper sample preparation and gas chromatography-mass spectrometry (GC-MS) to identify robust biomarkers that can be useful for screening, early detection, follow-up and treatment of PE and HELLP syndrome, the etiology of which are still unknown. RESULTS: The results showed a significant change in the metabolome profiles of the patient and control groups regarding the trimesters. A striking decrease was observed in all 100 metabolites investigated in the patient group (p < 0.000). The metabolic pathways associated with significant metabolites have also been investigated, and the most affected pathways were observed to be the urea cycle, glycine, serine, aspartate, methionine and purine metabolism, ammonia cycle, and phosphatidylethanolamine biosynthesis. CONCLUSION: The found metabolites provide us with extensive data on the ability to establish biomarkers for predicting, early detection and monitoring of PE.

[Loss of sight in the postpartum period]. / Verminderd zicht in het kraambed.

BACKGROUND: Vision problems in case of pre-eclampsia or Hemolysis, Elevated Liver, Low Platelets syndrome (HELLP) occur in 25-40% of the cases. Ablatio retinae as a complication occurs in only 0,1-2%. CASE DESCRIPTION: This article describes the case of a healthy 31-year-old woman who gave birth to her first child. A few hours after delivery she experienced vision loss. HELLP was diagnosed. Because of persistent vision loss combined with headache, the ophthalmologist and neurologist were consulted. A bilateral ablatio retinae as a complication of HELLP was diagnosed. Headache was most likely due to the side effect of nifedipine tablets, tension headache or a symptom of HELLP. Vision loss recovered spontaneously within a few weeks. CONCLUSION: Ablatio retinae due to preeclampsia or HELLP is very rare. For all concerned health care providers it is essential to pay attention to vision loss being the first symptom of possible acute underlying diagnosis postpartum.

First-Trimester Screening for HELLP Syndrome-Prediction Model Based on MicroRNA Biomarkers and Maternal Clinical Characteristics.

We evaluated the potential of cardiovascular-disease-associated microRNAs for early prediction of HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Gene expression profiling of 29 microRNAs was performed on whole peripheral venous blood samples collected between 10 and 13 weeks of gestation using real-time RT-PCR. The retrospective study involved singleton pregnancies of Caucasian descent only diagnosed with HELLP syndrome (n = 14) and 80 normal-term pregnancies. Upregulation of six microRNAs (miR-1-3p, miR-17-5p, miR-143-3p, miR-146a-5p, miR-181a-5p, and miR-499a-5p) was observed in pregnancies destined to develop HELLP syndrome. The combination of all six microRNAs showed a relatively high accuracy for the early identification of pregnancies destined to develop HELLP syndrome (AUC 0.903, p < 0.001, 78.57% sensitivity, 93.75% specificity, cut-off > 0.1622). It revealed 78.57% of HELLP pregnancies at a 10.0% false-positive rate (FPR). The predictive model for HELLP syndrome based on whole peripheral venous blood microRNA biomarkers was further extended to maternal clinical characteristics, most of which were identified as risk factors for the development of HELLP syndrome (maternal age and BMI values at early stages of gestation, the presence of any kind of autoimmune disease, the necessity to undergo an infertility treatment by assisted reproductive technology, a history of HELLP syndrome and/or pre-eclampsia in a previous gestation, and the presence of trombophilic gene mutations). Then, 85.71% of cases were identified at a 10.0% FPR. When another clinical variable (the positivity of the first-trimester screening for pre-eclampsia and/or fetal growth restriction by the Fetal Medicine Foundation algorithm) was implemented in the HELLP prediction model, the predictive power was increased further to 92.86% at a 10.0% FPR. The model based on the combination of selected cardiovascular-disease-associated microRNAs and maternal clinical characteristics has a very high predictive potential for HELLP syndrome and may be implemented in routine first-trimester screening programs.

HELLP syndrome, intracerebral hemorrhage, and hemophagocytic syndrome after cesarean section in a pregnant patient with severe preeclampsia: a case report.

BACKGROUND: Pregnancy-related intracranial hemorrhage (ICH) is a rare but potentially life-threatening event with complex and varied cause, such as HELLP syndrome and hemophagocytic syndrome. CASE PRESENTATION: A 33-year-old patient underwent a cesarean section with a preliminary diagnosis of "severe preeclampsia and class3 HELLP syndrome ". The patient had poor response to language before surgery, and the catheter drainage fluid was hematuria. Later, the surgeon reported severe bleeding in the operation. Following thromboelastography (TEG) result and postoperative laboratory tests confirmed class1 HELLP syndrome and ICH occurred on the second day after the surgery, and hemophagocytic syndrome was diagnosed during subsequent treatments. CONCLUSION: For patients with HELLP syndrome, we should pay attention to their coagulation condition. The coagulation tests and platelet counts should be repeated if their clinical presentation changed. Those with neurological alarm signs should receive CT or MRI scan. If a pregnant woman had prolonged hemocytopenia and thrombocytopenia, not only the HELLP but also the hemophagocytic syndrome should be considered.

Evaluation of long noncoding RNA (LncRNA) in pathogenesis of HELLP syndrome: diagnostic and future approach.

HELLP syndrome is a disorder during pregnancy which is defined by elevation of liver enzymes, haemolysis, and low platelet count. This syndrome is a multifactorial one and both genetic and environmental components can have a crucial role in this syndrome's pathogenesis. Long noncoding RNAs (lncRNAs), are defined as long non-protein coding molecules (more than 200 nucleotides), which are functional units in most cellular processes such as cell cycle, differentiation, metabolism and some diseases progression. As these markers discovered, there has been some evidence that they have an important role in the function of some organs, such as placenta; therefore, alteration and dysregulation of these RNAs can develop or alleviate HELLP disorder. Although the role of lncRNAs has been shown in HELLP syndrome, the process is still unclear. In this review, our purpose is to evaluate the association between molecular mechanisms of lncRNAs and HELLP syndrome pathogenicity to elicit some novel approaches for HELLP diagnosis and treatment.

Conservative Treatment of Huge Hepatic Subcapsular Hematoma Complicated with Hepatic Infarction after Cesarean Section Caused by HELLP Syndrome - a Case Report and Literature Review.

Hepatic subcapsular hematoma and hepatic infarction in labor are mostly secondary to HELLP syndrome and preeclampsia. There are few reported cases with a complicated diagnosis and treatment and high mortality. Here, we present a case of a huge hepatic subcapsular hematoma complicated with hepatic infarction after cesarean section that was secondary to HELLP syndrome and the patient was treated conservatively. Further, we have discussed the diagnosis and treatment of hepatic subcapsular hematoma and hepatic infarction caused by HELLP syndrome.

HELLP syndrome and COVID-19: A minor revision of a possible new "COVID-19-linked HELLP-like syndrome".

To report the characteristics described in the literature on a possible new "COVID-19-linked HELLP-like syndrome" in pregnant women with COVID-19: its association with the severity; prevalence; clinical; laboratory; pathophysiological and therapeutic management differences from the classic HELLP syndrome and their impact on outcomes. Observational, cohort, case-control, case-series and case-report studies were included. Data were extracted independently by the authors of the study, to ensure accuracy, consistency and performed the quality assessment. The database search resulted in 77 references, of which two satisfied the eligibility criteria. In these 2 studies we found a possible "COVID-19-linked HELLP-like syndrome", associated with severe COVID-19. There is a high possibility of the existence of "COVID-19-linked HELLP-like syndrome" and its association with severe COVID-19 in pregnant women, with a prevalence of 28,6%. Some characteristics of "COVID-19-linked HELLP-like syndrome" and the classic HELLP syndrome are similar. Differential diagnosis indicated two different types of therapeutic management: conservative for "COVID-19-linked HELLP-like syndrome" and delivery for the HELLP syndrome. HELLP clinical management is mandatory for both.

Spontaneous subcapsular hepatic hematoma in pregnant patients.

Spontaneous subcapsular hepatic hematoma (SSHH) with or without previous history of preeclampsia and/or HELLP syndrome represents a very rare pathological condition in pregnancy and postpartum, (1/45,000-1/225,000 pregnancies). Its importance for the anesthesiologist lays in its association with high morbidity and mortality for the mother (60-86%, 39%) and newborn (42%). After a high clinical suspicion, the certainty clinical diagnosis is settled by different imaging techniques. However, in most cases the diagnosis of SSHH is a casual intraoperative finding associated to a maternal or foetal compromise. Nowadays the obstetric and anaesthetic management of a SSHH is not standardized and depends on its integrity, hemodynamic stability and the gestational period when diagnosed. The possibility of an acute critic haemorrhage with necessity of massive transfusion, makes advisable to provide updated protocols for the treatment of obstetric hemorrhage, adapting them to the clinical peculiarities of these patients. After the acute phase, close attention should be kept on thromboembolic complications.