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.

Use of therapeutic apheresis methods during pregnancy.

This review will focus on the current application of TA in pregnancy and possible aspects for future studies. It seems that scientific interest and field for further research in pregnancy is lately focused in specific removal of pathogens implicated in the physiologic mechanism of pre-eclampsia/HELLP syndrome as well as recurrent pregnancy failure.

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.

[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.

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.

Maternal and perinatal outcome of women with early-onset severe pre-eclampsia before 28 weeks: Is expectant management beneficial in a low-resource country? A prospective observational study.

OBJECTIVE: To study the maternal and perinatal outcomes in women with severe pre-eclampsia before 28 weeks of pregnancy. METHODS: A descriptive study from a tertiary care center. All consecutive women with severe pre-eclampsia withonset before 28 weeks of pregnancy were included. The details were collected in a predesigned structured proforma prospectively. RESULTS: The study cohort included 145 women with a mean maternal age of 26.97 ± 5.36 years (range 19-47 years). The mean duration of prolongation of pregnancy was 13.04 ± 10.57 days (range 1-51 days). A total of 29.7% (n = 43) of women had at least one major adverse maternal outcome, and the most common was HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome (n = 24,16.6%), followed by eclampsia (n = 12,8.3%). The stillbirth rate was high (n = 103,68.7%), and most occurred in the antepartum period. Of 47 (31.3%) neonates born alive, only eight (17.02%;8/47) survived up to 28 days of life. Fetal growth restriction with Doppler abnormalities and neonatal sepsis were the most common reasons for perinatal mortality. CONCLUSION: Expectant management should not be considered routinely when the onset of severe pre-eclampsia is before 25+6 weeks of pregnancy. Between 26 and 27+6 weeks it can be offered under close monitoring and the perinatal survival depends on the neonatal services available in their facility.

Differentiating and Managing Rare Thrombotic Microangiopathies During Pregnancy and Postpartum.

The most common thrombotic microangiopathy (TMA) of pregnancy is the well-recognized syndrome of preeclampsia with hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. However, rare TMAs, including thrombotic thrombocytopenic purpura, complement-mediated hemolytic-uremic syndrome, and catastrophic antiphospholipid syndrome, may occur during pregnancy or postpartum and present with features similar to those of preeclampsia with severe features. Early recognition and treatment of these infrequently encountered conditions are key for avoiding serious maternal morbidities with long-term sequelae and possible maternal or fetal death. Differentiating between preeclampsia with severe features and these rare TMAs is diagnostically challenging as there is significant overlap in their clinical and laboratory presentation. Given the rarity of these TMAs, high-quality evidence-based recommendations on diagnosis and management during pregnancy are lacking. Using current objective information and recommendations from working groups, this report provides practical clinical approaches to diagnose and manage these rare TMAs. This report also discusses how to manage individuals with a history of these rare TMAs who are planning to conceive. To optimize favorable outcomes, a multidisciplinary approach including obstetricians, maternal-fetal medicine specialists, hematologists, and nephrologists alongside close clinical and laboratory monitoring is vital.

[Severe lactic acidosis in a pregnant woman with partial HELLP syndrome and COVID-19]. / Acidosis láctica grave en una mujer embarazada con síndrome HELLP parcial y COVID-19.

The present report describes the case of a 23-year old pregnant woman who was in the 36.5th week of gestation of her second pregnancy. She was attended at the emergency room because of dry cough and progressive dyspnea, in association with headache and myalgia. The nasopharyngeal swab for SARS-CoV-2 polymerase chain reaction (PCR) was positive. Oxygen saturation and chest x-ray were normal. Laboratory tests showed elevated values of bilirubin, aminotransferases, alkaline phosphatase and lactic dehydrogenase, and mild thrombocytopenia. Shortly after being admitted she began with labor. Faced with the lack of progression, the termination of the pregnancy by cesarean section was decided. Arterial blood gases showed severe lactic acidosis. She never presented evidence of clinical signs of tissue hypoperfusion or sepsis that could explain it. The patient completed her postoperative period in the intensive care unit, undergoing supportive treatment. All laboratory parameters were normalized after 72 hours, evolving favorably from the clinical point of view. It was interpreted that she had a partial HELLP syndrome. Lactic acidosis is not a component of this syndrome. Nor can it be ascribed to a mild disease by SARS-Cov-2. It probably responded to a summation of causes, including hyperproduction of lactic acid during labor, as well as its reduced clearance because of liver dysfunction related to HELLP syndrome.

Se presenta el caso de una mujer embarazada secundigesta, cursando la semana 36.5 de gestación, que concurrió a guardia general por tos seca y disnea progresiva de 3 días de evolución, asociadas a cefalea y mialgias. Se realizó hisopado nasofaríngeo para reacción en cadena de la polimerasa (PCR) para SARS-CoV-2, resultando positivo. La saturación de oxígeno era normal, al igual que la radiografía de tórax. En el laboratorio presentó elevación de bilirrubina, transaminasas, fosfatasa alcalina y LDH, además de plaquetopenia leve. Poco después de su ingreso, comenzó con trabajo de parto. Frente a la falta de progresión del mismo se decidió la finalización del embarazo por cesárea. La gasometría arterial reveló la presencia de acidosis láctica grave. Nunca hubo evidencia clínica de hipoperfusión tisular o sepsis que pudieran explicarla. Cursó su postoperatorio en la unidad de terapia intensiva, realizándose tratamiento de sostén. Todos los parámetros de laboratorio se normalizaron al cabo de 72 horas, evolucionando favorablemente desde el punto de vista clínico. Se interpretó que cursó un síndrome HELLP parcial. La acidosis láctica no es un componente descripto en este síndrome. Tampoco puede adscribirse a la enfermedad leve por SARS-CoV-2. Podría deberse a una sumatoria de causas, incluidas la hiperproducción de ácido láctico en el marco del trabajo de parto, como la disminución de su aclaramiento por la disfunción hepática asociada al síndrome HELLP.

HELLP syndrome manifesting as abnormal fetal umbilical artery blood flow and rapidly worsening laboratory indexes: A case report.

RATIONALE: HELLP syndrome, a rare but serious obstetric complication, is often overdiagnosed due to its nonspecific symptoms and inappropriate clinical testing. PATIENT CONCERNS: A 30-year-old nulliparous pregnant Chinese woman at gestational age of 28+1 weeks was admitted to our hospital because Doppler ultrasonography at a local hospital had detected loss of fetal umbilical artery end-diastolic blood flow lasting 12 hours. On admission to our hospital, the patient showed elevated blood pressure (148/84 mm Hg), but blood pressure and laboratory indicators after admission were normal. However, the patient developed abdominal pain during hospitalization. INTERVENTIONS: Dexamethasone was given after admission to our hospital to promote fetal lung maturation, magnesium sulfate was given to protect fetal brain nerves, and maternal blood pressure was closely monitored. In addition, fetal umbilical artery blood flow was dynamically monitored. After three days in hospital with normal blood pressure, the patient developed abdominal pain accompanied by diarrhea. She was positive for Murphy's sign and laboratory tests showed no obvious abnormalities. Acute cholecystitis was suspected, but symptomatic and supportive treatment did not relieve abdominal pain and her blood pressure increased progressively to 212/130 mm Hg. Magnesium sulfate was given immediately to prevent spasm, and nitroglycerin was administered intravenously against hypertension. Liver enzymes, blood coagulation, and routine urinalysis were abnormal. The patient was diagnosed with HELLP syndrome, and an emergency cesarean section was performed. DIAGNOSIS: HELLP syndrome. OUTCOMES: After the cesarean section, platelet (PLT) count continuously decreased and transaminase and bilirubin levels continously increased. The newborn was transferred to the neonatal intensive care unit after birth and discharged at a corrected gestational age of 34 weeks. By postoperative day 6, laboratory indicators had returned to normal and the patient was discharged. LESSONS SUBSECTIONS: Our case highlights that HELLP syndrome is a serious complication, and it should be diagnosed carefully and not arbitrarily on the basis of some abnormal indicators and stable clinical manifestations. Accurate early identification, active monitoring and management are essential for improving prognosis and avoiding maternal or infant mortality.

HELLP Syndrome.

Preeclampsia (PE) is the most common complication of pregnancy. PE is a multisystem disorder that can result in maternal morbidity and mortality. A severe complication of PE is the HELLP syndrome. HELLP syndrome is a pregnancy-associated liver disease characterized by the presence of hemolysis (H), elevated liver enzymes, and low platelet count. Management of HELLP syndrome includes monitoring of obstetric complications, controlling hypertension, seizure prevention, and planning for delivery. Also, postpartum care of the mother and the newborn should focus on physiologic and emotional wellbeing.

Ruptura hepática espontánea en paciente con síndrome HELLP.

Hemorrhagic liver rupture is a rare and deadly complication. The pathogenesis is unknown. This situation forces the multidisciplinary team, the immediate termination of pregnancy, the treatment and management of the patient in an intensive care unit (ICU). We report the results of two patients with spontaneous rupture of the liver during pregnancy and HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome, asymptomatic, during cesarean section, with management in ICU, poor evolution without adequate response; one died and the other leaves hospital. Liver rupture requires high suspicion and timely, aggressive multidisciplinary management in all cases and surgical intervention in those who develop liver ruptura, to improve survival.

La hemorragia por ruptura hepática es una rara y letal complicación, de etiología desconocida. Obliga al equipo multidisciplinario a la interrupción del embarazo, al tratamiento agresivo y al manejo de la paciente en una unidad de cuidado intensivo (UCI). Se presentan dos pacientes con embarazo de término con ruptura de hematoma hepático subcapsular asociado a síndrome HELLP (hemolysis, elevated liver enzymes, low platelet count), asintomáticas, durante operación cesárea, con manejo en UCI, ambas con evolución tórpida; una fallece y la otra se egresa. La ruptura hepática requiere una alta sospecha y un manejo multidisciplinario oportuno, agresivo en todos los casos y de intervención quirúrgica en quienes desarrollen ruptura hepática, para mejorar la supervivencia.

Pregnancy-Associated Liver Diseases.

The liver disorders unique to pregnancy include hyperemesis gravidarum, intrahepatic cholestasis of pregnancy, acute fatty liver of pregnancy, and preeclampsia-associated hepatic impairment, specifically hemolysis, elevated liver enzymes, and low platelet count syndrome (HELLP). Their importance lies in the significant maternal and fetal/neonatal morbidity and mortality. Expeditious diagnosis and clinical evaluation is critical to ensure timely, appropriate care and minimize risks to the pregnant woman and her fetus/baby. A multidisciplinary approach is essential, including midwives, maternal-fetal-medicine specialists, anesthetists, neonatologists, and hepatologists.

Conservative management of early-onset severe preeclampsia: comparison between randomized and observational studies a systematic review.

OBJECTIVE: To compare maternal and perinatal outcomes between randomized trials and observational studies in which conservative management was performed for more than 48 h in patients with early-onset severe preeclampsia. METHODOLOGY: We searched PubMed, LILACS, Cochrane and Google Scholar. The studies were divided in two groups: randomized and observational studies, from 1990 to 2018 that included patients with severe preeclampsia before 34 weeks of gestation with pregnancy prolongation ≥48 h but that did not include fetal growth restriction or HELLP syndrome at the beginning. The main variables recorded were maternal and perinatal complications. MAIN RESULTS: Forty-four studies met the inclusion criteria, and 5 of these were randomized. The average pregnancy prolongation was 9 days, with no difference between groups. Maternal complications were significantly more common in observational studies, RR = 0.71, 95% CI (0.54-0.93), p = .009. Perinatal complications were also significantly more common in observational studies (RR = 0.89, 95% CI (0.80-0.98), p = .01) at the expense of stillbirth and neonatal deaths. The percentages of cesarean sections were significantly higher in randomized studies, RR = 1.54, 95% CI (1.46-1.64). There were 2 maternal deaths, both in observational studies. CONCLUSION: Observational studies in which conservative management of early-onset preeclampsia is performed and do not include patients with fetal growth restriction or patients with HELLP syndrome and where at least 2 days of pregnancy prolongation is achieved are associated with significantly more maternal and perinatal complications.

Conservative management of a pregnancy complicated by preeclampsia and postpartum spontaneous hepatic rupture: A case report and review of the literature.

INTRODUCTION AND OBJECTIVES: Most spontaneous hepatic rupture cases are associated with a pregnancy-induced hypertensive disorder like preeclampsia and HELLP syndrome. Although it is a rare complication, it is still associated with high maternal and fetal morbidity and mortality rates. With this study, we aim to present a case report and review the available literature on hepatic rupture associated with hypertensive disorders of the pregnancy. METHODS: We present a case report and a review of the literature of the last 20 years on hepatic rupture associated with pregnancy-induced hypertensive disorders. The selected cases were reviewed to collect information on maternal characteristics, clinical presentation, diagnostic studies, therapeutic modalities and maternal and fetal outcomes. RESULTS: Our review has found 57 publications describing a total of 93 cases of hepatic hemorrhage with capsule rupture associated with pregnancy-induced hypertensive disorders. Most of the patients were less than 35 years old and primiparous and the first symptoms of hepatic rupture included epigastric and right upper abdominal pain. Most of the diagnoses were made during surgery without previous diagnosis and, in the majority of cases, a surgical approach was necessary to achieve hemostasis. Perihepatic packing was the most used surgical method. DISCUSSION/CONCLUSION: Our clinical case and literature review reinforces the importance of closely monitoring all pregnancies complicated with hypertensive disorders, including in the postpartum period. Although hepatic rupture accounts for high maternal and fetal morbidity and mortality rates, it is possible to keep a conservative approach with good maternal and fetal outcomes, with a high index of suspicious, an early diagnosis and a multidisciplinary approach.

Transcatheter Arterial Embolization for Spontaneous Hepatic Rupture Associated with HELLP Syndrome: A Case Report.

Background: Spontaneous hepatic rupture associated with the syndrome characterized by hemolysis, elevated liver enzymes, and a low platelet count (HELLP syndrome) is a rare and life-threatening condition, and only a few cases regarding the management of this condition through transcatheter arterial embolization (TAE) have been previously reported. Case summary: Herein, we report a case involving a 35-year-old pregnant woman who presented at 28 weeks of gestation with right upper quadrant pain, hypotension, and elevated levels of liver enzymes. Transabdominal ultrasound revealed fetal death. She required an emergency cesarean section, and hepatic rupture was identified after the fetus had been delivered. Hepatic packing and TAE were performed. The postprocedural course was uneventful, and the patient was discharged 14 days after she had been admitted to our hospital. Conclusions: Spontaneous hepatic rupture associated with HELLP syndrome is a very serious condition that requires prompt and decisive management. The high maternal and fetal mortality rates associated with this condition can be reduced through early accurate diagnosis and adequate management. The findings in the reported case indicate that TAE may be an attractive alternative to surgery for the management of spontaneous hepatic rupture associated with HELLP syndrome.

Idiopathic spontaneous liver hemorrhage A report of two cases and a review of the literature.

Spontaneous liver hemorrhage (SLH) is a serious, extremely rare, and life-threatening occurrence requiring a multidisciplinary approach. Since diagnosis might be difficult, a high mortality rate is reported. Survival depends on a prompt diagnosis followed by an appropriate management. If left untreated, SLH progresses, in fact, to a hemorrhagic shock and death. SLH is rarely idiopathic, whereas more commonly is secondary to severe preeclampsia and HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome, hepatocellular carcinoma (HCC), adenoma, focal nodular hyperplasia or hemangioma, and connective tissue diseases. We report two patients presenting with an idiopathic SLH successfully treated with angioembolization, and the results of an extensive literature review. KEY WORDS: Intrahepatic hematoma, Spontaneous liver hemorrhage, Spontaneous liver rupture.

Liver diseases in pregnancy.

Liver disease in pregnancy can be related to a pre-existing condition (such as autoimmune liver disease) or arise as a consequence of pregnancy. In women with pre-existing disease, pre-pregnancy counselling is important to discuss the potential complications that may occur during pregnancy and how best to manage these. Acute fatty liver of pregnancy and HELLP (haemolysis, elevated liver enzymes and low platelets) syndrome are pregnancy-related liver diseases and are considered obstetric emergencies. Women with liver dysfunction need appropriate investigations, including blood tests and imaging. They should be managed as part of a multidisciplinary team with obstetricians, obstetric anaesthetists, specialist midwives, gastroenterologists and obstetric physicians.

The effect of therapeutic plasma exchange on management of HELLP Syndrome: The report of 47 patients.

BACKGROUND AND OBJECTIVES: HELLP syndrome is a life-threatening condition that may potentially cause complications during pregnancy. If not diagnosed and treated quickly, HELLP syndrome may lead to serious complications both for the mother and the baby. The aim of this study was to determin the effectiveness of therapeutic plasma exchange (TPE) for treatment of Class-I HELLP syndrome. MATERIALS AND METHODS: Laboratory results from 47 patients with Class-I HELLP syndrome patients who underwent TPE between 2011 and 2020 were recorded before and after the procedure. A central venous catheter was inserted, and TPE was performed in patients who had not responded to delivery, steroid, and supportive therapy (blood products, anti-hypertensive therapy, intravenous fluid administration, and antibiotics) within 24 hours after the diagnosis of Class I HELLP syndrome according to the Mississippi Criteria. RESULTS: The average age of patients was 33 ± 4.7 years (range; 21-39 years). A mean of 5 (range; 4 to 6) TPE sessions were performed. There was a statistically significant decrease in total bilirubin, lactic dehydrogenase, aspartate aminotransferase, and alanine aminotransferase levels in all patients, whereas a significant increase in platelet count was observed (p < 0.05). Furthermore, clinical and laboratory improvement was achieved. CONCLUSION: In all patients with HELLP syndrome, a dramatically clinical and laboratory improvement occurred after TPE. Our study suggests that postpartum use of TPE within 24 hours is an efficient treatment option for Class-I HELLP syndrome.

Thrombotic microangiopathy during pregnancy.

Pregnancy is a high-risk time for the development of different kinds of thrombotic microangiopathy (TMA). Three major syndromes including TTP (thrombotic thrombocytopenic purpura), PE/HELLP (preeclampsia/hemolysis, elevated liver function tests, low platelets), and aHUS (atypical hemolytic- uremic syndrome) should be sought in pregnancy-TMA. These severe disorders share multiple clinical features and overlaps and even the coexistence of more than one pathologic mechanism. Each of these disorders finally ends in endothelial damage and fibrin thrombi formation within the microcirculation that fragments RBCs (schystocytes), aggregates platelets, and creates ischemic injury in the targeted organs i.e.; kidney and brain. Although the mechanisms of these severe disorders have been revealed, pregnancy-related TMA still interfaces with diagnostic and therapeutic challenges. Here, we highlight the current knowledge of diagnosis and management of these complications during pregnancy.