Acute liver failure in pregnancy.

Liver function abnormalities are noted in a minority of pregnancies with multiple causes for the same. A small proportion of these develop severe liver injury and progress to acute liver failure (ALF). There is a discrete set of etiology for ALF in pregnancy and comprehensive understanding will help in urgent evaluation. Certain diseases such as acute fatty liver of pregnancy, hemolysis, elevated liver enzyme, low platelet (HELLP) syndrome and pre-eclampsia are secondary to pregnant state and can present as ALF. Quick and targeted evaluation with urgent institution of etiology-specific management, especially urgent delivery in patients with pregnancy-associated liver diseases, is the key to avoiding maternal deaths. Pregnancy, as also the fetal life, imparts a further layer of complication in assessment, prognosis and management of these sick patients with ALF. Optimal management often requires a multidisciplinary approach in a well-equipped centre. In this review, we discuss evaluation, assessment and management of pregnant patients with ALF, focussing on approach to pregnancy-associated liver diseases.

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.

Hepatic infarction in pregnancy: a systematic review.

OBJECTIVE: Hepatic infarction is a rare complication of pregnancy most often associated with hemolysis, elevated liver enzymes, and low platelets syndrome. The objective of this review is to identify risk factors, present signs and symptoms, identify methods of diagnosis, and identify best management practices on the basis of published case reviews. DATA SOURCES: PubMed and MEDLINE (Ovid) databases were searched for citations regarding hepatic infarction in pregnancy or the postpartum period from database inception until the study date of December 18, 2023. Key words included "liver infarction" or "hepatic infarction" and "pregnancy" or "obstetrics." STUDY ELIGIBILITY CRITERIA: Case reviews or case series published in the English language were included. Our study was registered with the Prospective Register of Systematic Reviews (registration number CRD42023488176) and was conducted in accordance with the published Prospective Register of Systematic Reviews and Meta-analyses Of Observational Studies in Epidemiology guidelines. METHODS: Included papers were evaluated for bias using a previously published tool. RESULTS: A total of 38 citations documenting 50 pregnancies published between 1979 and 2023 were included. Of these, 34% had a history of hypertensive disease, 26% had antiphospholipid syndrome, and 22% had a history of thrombus. Of those without a preexisting diagnosis of antiphospholipid syndrome, 24% tested positive during hospitalization. Most patients presented with epigastric or right upper quadrant pain (78%), and 32% and 16% had severe blood pressure or mild blood pressure, respectively. Sixty-four percent of patients presented with transaminitis. Forty-six percent of patients delivered preterm, and 32% of pregnancies ended in intrauterine fetal demise, abortion, or early termination of pregnancy for maternal benefit. Computed tomography scans were used to confirm diagnosis of hepatic infarction in 58% of cases, magnetic resonance imaging in 14%, and ultrasound in 6%. In cases that described management, treatment was always multimodal, including antihypertensives (18%), therapeutic anticoagulation (45%), blood product transfusion (36%), plasma exchange or intravenous immunoglobulin (20%), and steroids (39%). Transfer to the intensive care unit was required in 20% of cases. CONCLUSION: Hepatic infarction should be considered in all cases of hemolysis, elevated liver enzymes, and low platelets syndrome, but specifically in patients with a history of antiphospholipid syndrome who present with epigastric or right upper quadrant pain. The diagnosis can usually be confirmed with a computed tomography scan alone, and management should be prompt with supportive care, therapeutic anticoagulation, and steroids.

Síndrome HELLP: controversias y pronóstico / HELLP syndrome: controversies and prognosis

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Niveles plaquetarios y marcadores de disfunción endotelial multiorgánica en síndrome HELLP / Platelet levels and multiorganic endothelial dysfunction markers in the Hellp Syndrome (Hemolysis, elevated liver enzimes, low platelets)

HELLP syndrome (hemolysis, elevated liver enzimes, low platelets count) is one of the major complications of the progression of thrombotic microangiopathies characterized by thrombocytopenia, hemolistic anemia and organic dysfunction. It can be found in between 0,5 - 0,9 % of all pregnancies, and between 4- 14 % in all those women who develop preeclampsia. The general objectives of this report were to describe the relationship existing between the platelet levls and the organic endothelial dysfunction because of the HELLP syndrome, and to determine the perinatal results, during the 10 yr. period of the study, in patients that were assited between 2007 and 2016.

Consideraciones anestésicas en el síndrome de HELLP / Hellp syndrome and anesthesia

The HELLP syndrome is one of the most relevant obstetrical pathologies but for the anestesiologist, which, can even condition the anesthetic technique before a caesarean section. The early colocation of a epidural catheter in obstetrical pathologies that can finish in a cesarean section with the aim of diminishing the mortality associated to the pathology is still under discussion. We present a case of a 25 years old patient programmed for a caesarean section, with probable diagnosis of HELLP syndrome, In which a epidural catheter was placed 12 hours before the surgery presenting a trombocitopenia of 86,000/mm3. Our objetive is to discuss the issues of the anesthetic management in patients with HELLP syndrome and the advantages of the early placement of a epidural catheter.

El síndrome de HELLP es una de las patologías obstétricas más relevantes para el anestesiólogo, la cual, puede incluso condicionar la técnica anestésica ante una eventual cesárea. Actualmente, está en discusión la colocación de un catéter epidural temprano en patologías obstétricas que puedan terminar en una cesárea, con el objetivo de disminuir la morbimortalidad asociada a ella. Presentamos el caso de una paciente de 25 años programada para una operación cesárea, con probable diagnóstico de síndrome HELLP a la que se le colocó un catéter epidural 12 horas antes de la cirugía con una trombocitopenia de 86 x 103/uL. Se presenta con el objetivo de discutir las directivas del manejo anestésico de las pacientes con síndrome de HELLP y las ventajas de la colocación temprana de un catéter epidural.

Prevalencia de Síndrome de Hellp en el servicio de ginecología y obstetricia del Hospital Central del Instituto de Previsión Social. 2017 / Prevalence of Hellp Syndrome in the gynecology and obstetrics service of the Central Hospital of the Instituto de Previsión Social

Introducción: En la actualidad, la mortalidad materna va aumentando, debido a ciertas complicaciones producidas durante la gestación, una de ellas es el Síndrome de HELLP que es considerada una de las complicaciones más severas de las enfermedades hipertensivas del embarazo que mayoritariamente se presenta en embarazadas que cursan con preeclampsia. Objetivo: Determinar la prevalencia de Síndrome de HELLP en el Servicio de Ginecología y Obstetricia del Hospital Central del Instituto de Previsión Social durante el año 2017. Materiales y método: Estudio observacional descriptivo de corte transversal, muestreo probabilístico aleatorio simple. Fueron incluidas las fichas clínicas de las embarazadas que acudieron al servicio de Ginecología y Obstetricia del Hospital Central del Instituto de Previsión Social desde enero a diciembre del 2017. Resultados: Fueron incluidas en el estudio 177 embarazadas; de las cuales el 14,2% presentó Síndrome de HELLP. La mediana de edad fue de 28 años; el estado civil predominante fue unión libre en un 49,15%; el 62,71% de las gestantes procedieron de áreas urbanas, mayoritariamente del departamento Central y el 33,90% de ellas son ama de casa. La edad gestacional estuvo comprendida predominantemente entre las 33,2 a 37,6 semanas; la mediana del número de controles prenatales fue de 5. Con respecto a las enfermedades hipertensivas del embarazo la mayor parte de las gestantes presentaron preeclampsia. Conclusión: La prevalencia de Síndrome de HELLP en las embarazadas que acudieron al servicio de Ginecología y Obstetricia en el Hospital Central del Instituto de Previsión Social durante el año 2017 fue del 14,2%; dato similar a otros estudios realizados.

Anesthesia in pregnant women with HELLP syndrome: case report / Anestesia para gestante com síndrome HELLP: relato de caso

Abstract Background and objectives: HELLP syndrome, characterized by hemolysis, high levels of liver enzyme, and low platelet count, is an advanced clinical stage of pre-eclampsia, progressing to high maternal (24%) and perinatal (up 40%) mortality, despite childbirth care in a timely manner. The goal is to describe the anesthetic management of a case with indication to emergency cesarean. Case report: Female patient, 36 years old, gestational age of 24 weeks, with hypertensive crisis (BP 180/100 mmHg) and severe headache, was admitted to the operating room for a cesarean section after diagnosis of HELLP syndrome. Indicated for general anesthesia, we opted for total intravenous with intubation after rapid sequence induction with propofol and remifentanil in continuous target-controlled infusion, and rocuronium at a dose of 1.2 mg/kg. Maintenance was achieved with propofol and remifentanil. The surgical procedure was uneventful, the child was born with APGAR 1/5 and transferred to the NICU. At the end of surgery, the patient was extubated in the operating room and taken to the ICU. The postoperative period was uneventful with no changes worthy of note and the patient was discharged on the sixth postoperative day. Conclusion: When general anesthesia is the choice in parturient with HELLP syndrome, tracheal intubation with rapid sequence induction due to possible difficult airway, as well as the use of drugs to control the hemodynamic response can minimize the complications associated with the procedure, as occurred in this case.

Resumo Justificativa e objetivos: A síndrome HELLP, caracterizada por hemólise, elevação dos níveis de enzimas hepáticas e plaquetopenia, representa estágio clínico avançado da pré-eclâmpsia, cursando com elevada mortalidade materna (24%) e perinatal (até 40%), apesar da assistência ao parto de forma oportuna. O objetivo é descrever o manejo anestésico de um caso com indicação de cesariana de emergência. Relato de caso: Paciente do sexo feminino, 36 anos, idade gestacional 24 semanas, com crise hipertensiva (PA 180/100 mmHg) e cefaleia intensa, é encaminhada ao Centro Cirúrgico para operação cesariana após diagnóstico de síndrome HELLP. Indicada anestesia geral, optou-se por venosa total com intubação após indução sequencial rápida, com propofol e remifentanil em infusão contínua alvo-controlada e rocurônio na dose de 1,2 mg/kg. A manutenção foi obtida com propofol e remifentanil. O procedimento cirúrgico transcorreu sem anormalidades, a criança nasceu com APGAR 1/5 e foi encaminhada à UTI Neonatal. Ao final da cirurgia, procedeu-se à extubação na sala cirúrgica e a paciente foi encaminhada ao CTI. O pós-operatório decorreu sem alterações dignas de nota e a paciente teve alta no sexto dia pós-operatório. Conclusão: Quando se opta pela anestesia geral em parturiente com síndrome HELLP, a intubação traqueal com técnica de indução sequencial rápida em função de possível via aérea difícil, bem como o emprego de drogas que controlem a resposta hemodinâmica, podem minimizar as complicações associadas ao procedimento, como ocorreu no presente caso.

Actualización en el diagnóstico y manejo del daño hepático agudo grave en el embarazo / Causes and management of severe acute liver damage during pregnancy

Abnormalities in liver function tests appear in 3% of pregnancies. Severe acute liver damage can be an exclusive condition of pregnancy (dependent or independent of pre-eclampsia) or a concomitant disease. HELLP syndrome and acute fatty liver of pregnancy are the most severe liver diseases associated with pregnancy. Both appear during the third trimester and have a similar clinical presentation. Acute fatty liver may be associated with hypoglycemia and HELLP syndrome is closely linked with pre-eclampsia. Among concomitant conditions, fulminant acute hepatitis caused by medications or virus is the most severe disease. Its clinical presentation may be hyper-acute with neurological involvement and severe coagulation disorders. It has a high mortality and patients should be transplanted. Fulminant hepatic failure caused by acetaminophen overdose can be managed with n-acetyl cysteine. Because of the high fetal mortality rate, the gestational age at diagnosis is crucial.

Diagnóstico posparto de la enfermedad de Wilson en gestante con síndrome HELLP y coagulopatía / Postpartum diagnosis of Wilson disease in a pregnant woman with HELLP syndrome and coagulopathy

El síndrome HELLP es una entidad clínico-analítica definida por anemia hemolítica microangiopática, elevación de enzimas hepáticas y trombocitopenia. La afectación hepática del síndrome es común a diversas hepatopatías. Algunas, inherentes a la gestación (hígado graso agudo del embarazo, colestasis intrahepática gestacional) y otras relativas a enfermedad intercurrente (hepatitis agudas virales) o hepatopatía crónica previa. Se presenta un caso clínico de diagnóstico posparto de hepatopatía crónica por enfermedad de Wilson (EW), que comienza en el tercer trimestre de gestación con síndrome HELLP asociado a insuficiencia hepática y coagulopatía. Se revisan los posibles diagnósticos diferenciales y la literatura científica al respecto. Conclusión. Es importante la realización de un adecuado diagnóstico diferencial del síndrome HELLP, ya que no siempre es secundario a la preeclampsia gestacional, pudiendo ser el punto de partida para el diagnóstico de otras hepatopatías agudas o crónicas subyacentes (AU)

HELLP syndrome is a clinical-analytical entity defined by microangiopathic hemolytic anemia, elevated liver enzymes, and thrombocytopenia. Liver involvement in this syndrome is common to several liver diseases. Some of these liver diseases are inherent to pregnancy (acute fatty liver of pregnancy, gestational intrahepatic cholestasis), while others are related to intercurrent disease (acute viral hepatitis) or to previous chronic liver disease. We report a case of postpartum diagnosis of chronic liver disease secondary to Wilson disease, with onset in the third trimester of pregnancy and HELLP syndrome associated with acute liver failure and coagulopathy. We review the differential diagnosis and the scientific literature on the topic. Conclusion. A correct differential diagnosis of HELLP syndrome is important because this syndrome is not always secondary to gestational preeclampsia and can be the starting point for the diagnosis of other acute or chronic liver diseases (AU)

Hígado graso agudo del embarazo y síndrome HELLP: aspectos diagnósticos y terapéuticos / Acute fatty liver of pregnancy and HELLP syndrome: diagnostic and therapeutic aspects

HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count) and acute fatty liver of pregnancy (AFLP), cause liver dysfunction in the third trimester of pregnancy and determine risk of maternal and fetal mortality. Many maternal complications can occur, including acute renal failure and coagulopathy. Early diagnosis, prompt delivery and adequate supportive care are the key to a good outcome in most of the patients. In some cases, despite an appropriate conventional management, the disease may progress to multiple organ dysfunction, progressive liver insufficiency or severe hepatic complications (subcapsular hematoma, liver rupture), requiring adjunctive medical therapy, such as plasma exchange, surgery or liver transplantation.

El síndrome HELLP (hemolysis, elevated liver enzymes, and low platelet count) y el hígado graso agudo del embarazo (acute fatty liver of pregnancy -AFLP), son causa de disfunción hepática en el tercer trimestre del embarazo y condicionan riesgo de mortalidad materna fetal. Se pueden presentar diversas complicaciones como insuficiencia renal aguda y coagulopatía, entre otras. El diagnóstico oportuno, la interrupción del embarazo y un manejo de sostén adecuado permiten sacar adelante a la mayoría de las pacientes. En algunos casos, a pesar de un manejo convencional adecuado, la enfermedad puede evolucionar con disfunción multiorgánica, insuficiencia hepática progresiva o complicaciones hepáticas graves (hematoma subcapsular, rotura hepática), requiriendo de terapia médica adyuvante, como la plasmaféresis, cirugía o incluso trasplante hepático.

Manejo conservador de un hematoma subcapsular hepático en un síndrome de HELLP / Conservative management of a subcapsular liver hematoma in HELLP syndrome

La sospecha clínica, el diagnóstico rápido y un abordaje multidisciplinar son fundamentales para mejorar la morbimortalidad de las pacientes con hematoma subcapsular hepático. El tratamiento de esta entidad abarca desde la conducta expectante bajo vigilancia clínica y radiológica, la embolización de las arterias hepáticas o la cirugía en caso de inestabilidad hemodinámica, persistencia de sangrado o incremento del dolor. Presentamos el caso de un síndrome de HELLP posparto complicado con un hematoma subcapsular hepático y que se resolvió exitosamente con embolización de la arteria hepática derecha (AU)

To improve morbidity and mortality in patients with subcapsular liver hematoma, clinical suspicion, a rapid diagnosis and multidisciplinary treatment are fundamental. The treatment of this complication includes expectant management with clinical and radiological observation, embolization of the hepatic arteries, and surgical treatment if there is hemodynamic instability, persistent bleeding or increasing pain. We report a case of postpartum HELLP syndrome that was complicated by a subcapsular liver hematoma, successfully treated by embolization of the right hepatic artery (AU)

Tratamiento conservador de la rotura espontánea hepática en un paciente con el síndrome de hemólisis, elevación de enzimas hepáticas y descenso de las plaquetas / Medical magement of the hepatic spontaneous rupture in pacient with HELLP syndrome

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Diagnóstico e conduta na Síndrome HELLP / Diagnosis and management of HELLP Syndrome

A síndrome HELLP é definida classicamente pela presença de hemólise, elevação das enzimas hepáticas e plaquetopenia durante a gravidez. Acredita-se que essa síndrome seja uma variante da pré-eclâmpsia grave, apesar de nem sempre ocorrer hipertensão arterial. A síndrome HELLP ocorre em cerca de 1 a 2 casos a cada 1.000 gestações, e sua importância reside na elevada morbimortalidade materna e fetal. Dessa forma, o diagnóstico oportuno poderá livrar a mãe e o feto dos efeitos deletérios da doença. O tratamento definitivo consiste no parto, entretanto, a conduta dependerá da idade gestacional, da vitalidade fetal e das condições clínicas maternas. Neste artigo revisam-se os principais indícios diagnósticos da síndrome HELLP, bem como as condutas para o melhor benefício materno-fetal.

HELLP syndrome is classically defined by the presence of hemolysis, elevated liver enzymes and low platelets (thrombocytopenia), during pregnancy. It is considered a variant of severe pre-eclampsia, although hypertension might not always occur. HELLP syndrome occurs in approximately 1 to 2 cases per 1000 pregnancies, and its importance is related to the high maternal and fetal mortality. Thus the diagnosis should be able to deliver mother and fetus from the deleterious effects of the disease. The definitive treatment is the delivery, however HELLP prognosis will depend on the fast diagnosis, and the treatment must take into consideration the gestational age, the fetal and the maternal medical conditions. This review article focus on HELLP syndrome major evidences as well as on the best maternal-fetal benefitial behavior.

Síndrome HELLP: relato de caso / HELLP syndrome: case report

Este artigo relata o caso de paciente de 20 anos de idade, feoderma, grávida de 35 semanas e três dias, primigesta, com síndrome HELLP. Foi internada na Maternidade do Hospital das Clínicas da UFMG após apresentar lombalgia e vômitos. Foram constatadas hipertensão e proteinúria. Submeteu-se à propedêutica para síndrome HELLP, que revelou plaquetopenia, elevação de transaminases e de desidrogenase lática. A gestante foi submetida a cesariana segmentar sob anestesia geral. Evoluiu com hipotonia uterina e hemorragia puerperal. Recebeu concentrado de plaquetas e de hemácias. Teve alta após cinco dias, com exames laboratoriais normais.

This is the case report a 20-year-old pregnant woman, yellow-skinned (feoderma), with gestational age 35 weeks and 3 days, primigravida with a final diagnosis of HELLP syndrome. She was admitted at the Maternity section of Hospital das Clínicas da UFMG, after presenting symptoms of low back pain, abdominal pain and vomiting. The physical examination and tests found hypertension and proteinuria. The laboratory tests for HELLP syndrome revealed thrombocytopenia, elevated transaminases and lactate dehydrogenase (LDH). The patient underwent a caesarean section under general anesthesia. She presented hypotonic uterine inertia and postpartum hemorrhage. The patient was given platelet concentrates and red blood cells. She was discharged after the 5th day, with normal laboratory.

Síndrome de Hellp: una propuesta terapéutica: presentación de caso / Hellp´S Syndrome: a Therapeutic Proposal: case presentation

Se trató un caso relacionado con una paciente femenina, mestiza, 29 años de edad, buenos antecedentes de salud, que ingresó en el servicio de terapia intensiva durante 5 días por presentar enfermedad hipertensiva asociada a la gestación (Eclampsia) complicada con Síndrome de Hellp. Se presentó el cuadro clínico típico de hemólisis, trombocitopenia y elevación de enzimas hepáticas, donde se practicó el tratamiento básico, hemoderivados y se introdujo, por primera vez, una inmunoglobulina intacta de producción nacional (intacglobin) con excelentes resultados basados en los posibles mecanismos inmunológicos que en su etiología se invocan(AU)

A case of a female patient, half-breed, 29 years of age, good antecedents of health that was admitted to the service of Intensive Therapy during 5 days presenting hypertension associated to gestation (Eclampsia) complicated with Hellp´s Syndrome was treated. The case presented the typical clinical picture of haemolysis, thrombocytopenia and elevation of hepatic enzymes. The basic treatment for hypertensive conditions was used, plus hemo derivates; and an intact immunoglobulin of national production (intacglobin) was introduced for the first time with excellent results, based on the possible immunologic mechanisms that are invoked in their etiology(AU)

Síndrome HELLP: diagnóstico e conduta / HELLP Syndrome: diagnosis and management

A síndrome HELLP é definida pela presença de hemólise, elevação de enzimas hepáticas e trombocitopenia em gestante com toxemia. Sua incidência é estimada em aproximadamente 20 porcento dos casos de pré-eclampsia grave e está associada a grande morbidade materna e perinatal. O diagnóstico laboratorial da síndrome HELLP constitui temática controversa, não havendo consenso tanto quanto aos testes como aos valores a serem utilizados. A conduta das gestantes com síndrome HELLP deve levar em consideração a idade gestacional, a presença de complicações maternas, a vitalidade fetal e as condições do colo uterino. O tratamento ideal, assim como em qualquer caso de toxemia, é o parto. Recentemente, tem-se aventado que a utilização de altas doses de corticóides pode melhorar o desfecho materno, além do já comprovado benefício fetal.