Manejo anestésico en gestante con hígado graso agudo / Anesthetic management in pregnant women with acute fatty liver

Introducción: El hígado graso agudo del embarazo es una complicación poco frecuente y potencialmente fatal. Objetivo: Describir el manejo anestésico en una gestante portadora de enfermedad del hígado graso agudo. Presentación del caso: Se trata de una gestante de 19 años de edad con antecedentes de hipotiroidismo posquirúrgico, anunciada para procedimiento quirúrgico de urgencia para realizarle cesárea por presentar hígado graso agudo del embarazo. Conclusiones: El hígado graso agudo del embarazo obliga a realizar diagnóstico precoz y tratamiento agresivo. La atención médica interdisciplinaria y el tratamiento de soporte son decisivos en la evolución favorable. La cesárea es el método más seguro de la terminación del embarazo y se debe recomendar para reducir el riesgo de resultados adversos. La administración de anestesia general orotraqueal para la cesárea de urgencia garantiza un abordaje quirúrgico seguro y eficaz.

Introduction: The acute fatty liver of pregnancy is a rare and potentially fatal complication. Objective: To describe anesthetic management in a pregnant woman who carries acute fatty liver disease. Case report: This is a 19 year-old pregnant woman with a history of postoperative hypothyroidism, announced for emergency surgical procedure to perform cesarean section due to acute fatty liver of pregnancy. Conclusions: The acute fatty liver of pregnancy requires early diagnosis and aggressive treatment. Interdisciplinary medical care and supportive treatment are decisive in the favorable evolution. Caesarean section is the safest method of pregnancy termination and should be recommended to reduce the risk of adverse outcomes. The administration of orotracheal general anesthesia for emergency cesarean section ensures a safe and effective surgical approach.

Preeclampsia de presentación grave asociada a hígado graso agudo del embarazo / Severe preeclampsia associated with acute fatty liver of pregnancy / Pré-eclâmpsia grave associada a fígado gorduroso agudo da gravidez

La preeclampsia se puede asociar a una patología poco frecuente como es el hígado graso agudo del embarazo. Se reporta el caso clínico de una paciente de 35 años, tercigesta, cursando embarazo gemelar que presenta preeclampsia con elementos de gravedad, asociada a hígado graso agudo del embarazo. Se realiza diagnóstico y tratamiento precoz de ambas patologías, presentando buena evolución materno-fetal.

Preeclampsia can be associated with acute fatty liver of pregnancy, a rare disease. This report describes the case of a 35-year-old patient, gravida 3, pregnant with twins, who presented with severe pre-eclampsia associated with acute fatty liver of pregnancy. Early diagnosis and treatment of both pathologies was performed, resulting in good maternal-fetal evolution.

A pré-eclâmpsia pode estar associada a uma patologia rara, como o fígado gorduroso agudo da gravidez. Neste relato, apresentamos uma paciente de 35 anos, terciária, em gestação gemelar, apresentando pré-eclâmpsia grave, associada a esteatose hepática aguda na gestação. É realizado diagnóstico e tratamento precoces de ambas as patologias, apresentando boa evolução materno-fetal.

A comparison of clinical characteristics between acute fatty liver of pregnancy and hemolysis, elevated liver enzymes and low platelets syndrome / 中华危重病急救医学

Objective:To compare and analyze the clinical characteristics between acute fatty liver of pregnancy (AFLP) and the hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome.Methods:This is a retrospective cohort study. The clinical data of 13 cases with AFLP and 34 cases with HELLP syndrome were collected from three tertiary referral centers in Yunnan (the First Affiliated Hospital of Kunming Medical University, the Second Affiliated Hospital of Kunming Medical University, and Yan'an Hospital of Kunming City) from January 2016 to December 2021. The patients were diagnosed to AFLP and HELLP syndrome according to the Swansea criteria and the Tennessee classification system. The general characteristics, clinical features, laboratory results within 24 hours after admission, complications, maternal and neonatal outcomes were compared to analysis the differences between the two groups.Results:① Maternal characteristics: compared with HELLP syndrome group, AFLP group had lower body mass index (BMI) and blood pressure at admission (both P < 0.01). ②Clinical features: the most common symptoms in AFLP patients were skin jaundice, abdominal pain, nausea and vomiting, edema. The main manifestations of patients with HELLP syndrome were albuminuria, hypertension, edema, headache. Some patients had multiple symptoms concurrently. ③ Laboratory results: compared with HELLP syndrome group, the levels of platelet count (PLT), total bilirubin (TBil), direct bilirubin (DBil), γ-glutamyl transferase (γ-GGT), alkaline phosphatase (ALP), total bile acid (TBA), serum creatinine (SCr) and international standardized ratio (INR) in AFLP group were significantly increased within 24 hours after admission [PLT (×10 9/L): 107.69±51.13 vs.76.71±43.25, TBil (μmol/L): 121.60 (83.20, 170.00) vs.15.25 (7.22, 29.05), DBil (μmol/L): 86.50 (58.60, 104.00) vs. 4.30 (2.22, 10.10), γ-GGT (U/L): 87.00 (37.00, 127.00) vs. 41.00 (19.00, 64.42), ALP (U/L): 199.10 (109.00, 349.20) vs. 125.50 (90.50, 155.25), TBA (μmol/L): 51.50 (16.20, 117.40) vs. 4.15 (2.02, 6.95), SCr (μmol/L): 155.80 (129.00, 237.00) vs. 79.00 (65.43, 113.70), INR: 1.28 (1.17, 1.63) vs. 0.94 (0.88, 1.08), all P < 0.05], prothrombin time (PT) was significantly prolonged [seconds: 16.10 (14.50, 19.20) vs. 12.40 (11.43, 13.40), P < 0.05]. The level of blood glucose (GLU), fibrinogen (FIB) and the activity of antithrombin Ⅲ (ATⅢ) decreased significantly [GLU (mmol/L): 5.18±1.33 vs. 6.33±1.19, FIB (g/L): 1.96±1.46 vs. 3.81±1.58, ATⅢ (%): 40.61±25.84 vs. 66.39±24.11, all P < 0.05]; ④ Complications: compared with HELLP syndrome group, the incidence of patients with hypoglycemia [30.77% (4/13) vs. 0% (0/34)], acute liver failure [53.85% (7/13) vs. 5.88% (2/34)], acute renal insufficiency [69.23% (9/13) vs. 8.82% (3/34)], coagulopathy [76.92% (10/13) vs. 38.24% (13/34)], disseminated intravascular coagulation (DIC) [53.85% (7/13) vs. 5.88% (2/34)], and multiple organ dysfunction syndrome (MODS) [53.85% (7/13) vs. 5.88% (2/34)] were significantly higher in AFLP group (all P < 0.05). ⑤ Maternal and neonatal outcome: all patients delivered after admission. The total length of hospital and intensive care unit stay were significantly longer in the AFLP group than in the HELLP syndrome group [days: 17.00 (11.00, 25.00) vs. 9.00 (7.00, 12.00), 12.00 (4.00, 22.00) vs. 3.91 (0, 7.00), both P < 0.01]. Two AFLP patients died, including one due to intracranial venous thrombosis and one due to multiple organ failure and cardiopulmonary arrest. There were no deaths in the HELLP syndrome group. Conclusions:There are significant differences in maternal characteristics, laboratory results and complications between AFLP and HELLP syndrome. TBil, γ-GGT, SCr, FIB, INR and ATⅢ activity may help to distinguish the two diseases.

Temporal Evolution of Maternal Mortality: 1980-2019 / Evolução temporal da mortalidade materna: 1980-2019

Abstract Objective To determine the profile of maternal deaths occurred in the period between 2000 and 2019 in the Hospital de Clínicas de Porto Alegre (HCPA, in the Portuguese acronym) and to compare it with maternal deaths between 1980 and 1999 in the same institution. Methods Retrospective study that analyzed 2,481 medical records of women between 10 and 49 years old who died between 2000 and 2018. The present study was approved by the Ethics Committee (CAAE 78021417600005327). Results After reviewing 2,481 medical records of women who died in reproductive age, 43 deaths had occurred during pregnancy or in the postpartum period. Of these, 28 were considered maternal deaths. The maternal mortality ratio was 37.6 per 100,000 live births. Regarding causes, 16 deaths (57.1%) were directly associated with pregnancy, 10 (35.1%) were indirectly associated, and 2 (7.1%) were unrelated. The main cause of death was hypertension during pregnancy (31.2%) followed by acute liver steatosis during pregnancy (25%). In the previous study, published in 2003 in the same institution4, the mortality rate was 129 per 100,000 live births, and most deaths were related to direct obstetric causes (62%). The main causes of death in this period were due to hypertensive complications (17.2%), followed by postcesarean infection (16%). Conclusion Compared with data before the decade of 2000, there was an important reduction in maternal deaths due to infectious causes.

Resumo Objetivo Determinar o perfil dos óbitos maternos ocorridos no período de 2000 a 2019 no Hospital de Clínicas de Porto Alegre (HCPA) e comparar com os óbitos maternos entre 1980 e 1999 na mesma instituição. Métodos Estudo retrospectivo que analisou 2.400 prontuários de mulheres entre 10 e 49 anos que morreram entre 2000 e 2019. O presente estudo foi aprovado pelo Comitê de Ética (CAAE 78021417600005327). Resultados Após revisão de 2.481 prontuários de mulheres que morreram em idade reprodutiva, 43 mortes ocorreram durante a gravidez ou no período pós-parto. Destas, 28 foram considerados óbitos maternos. A taxa de mortalidade materna foi de 37.6 por 100.000 nascidos vivos. Em relação às causas, 16 óbitos (57.1%) estiveram diretamente associados à gravidez, 10 (35.1%) estiveram indiretamente associados e 2 (7.1%) não estiveram relacionados. A principal causa de morte foi hipertensão na gravidez (31.2%) seguida de esteatose hepática aguda da gravidez (25%). No estudo anterior, publicado em 2003 na mesma instituição4, a taxa de mortalidade foi de 129 por 100.000 nascidos vivos, e a maioria dos óbitos estava relacionada a causas obstétricas diretas (62%). As principais causas de óbito neste período foram por complicações hipertensivas (17.2%), seguidas de infecção pós-cesárea (16%). Conclusão Em comparação com os dados anteriores à década de 2000, houve uma redução importante das mortes maternas por causas infecciosas.

Effect of abnormal liver function test on maternal and perinatal outcome in pregnancy: observational study

Background: The present study was conducted with the objective to assess the spectrum of liver disease in pregnancy, and its course and effect on maternal and fetal outcomes.Methods: The present study was conducted as a prospective follow up study in the department of Obstetrics and Gynaecology, J. K. Hospital for a period of 1 year. The pregnant women between the ages of 18-35 years presenting with symptoms suggestive of underlying liver disease were selected. Socio demographic details and clinical history was obtained from all the participants and they were subjected to liver function test (LFT). All patients were followed till 2 weeks post-partum period. The maternal and fetal outcomes were noted.Results: The following results were obtained: pregnancy-induced hypertension (PIH) was the most common cause of abnormal LFT (46.66%), about 57.5% patients delivered at term, 63.3% patients delivered vaginally, mostly cases delivered a term healthy neonate between 2.5-3.0 kg weight with Apgar score >7 at 5 minutes after birth and maternal complications were seen in 10.82% cases.Conclusions: Our study shows that though liver disease is uncommon in Indian pregnant women, but it is associated with high maternal and perinatal morbidity. A high index of suspicion of liver disease, early diagnosis, prompt referral to a higher centre when required, appropriate supportive management, and a proactive policy of early delivery when indicated may improve the maternal and fetal outcomes in pregnant women with liver disease. Thus LFT should be conducted as a routine investigation in all pregnant females during first and second trimester.

Progressing haemolysis, elevated liver enzymes, low platelet count syndrome: near miss case

A 25 years old women presented in labour room with 30 weeks’ pregnancy in labour with breech with previous two caesarean section and scar tenderness with foetal bradycardia (FHR 90) and jaundice. After caesarean section in emergency hour she started bleeding in abdominal drain while uterus was well contracted. On investigation and examination diagnosed as HELLP syndrome, managed promptly by medical teams of three departments (obstetrician, anaesthesia and medicine).

Review of maternal mortality in a tertiary care urban teaching hospital: 10 year retrospective study

Background: The objectives of this study were to calculate the maternal mortality ratio, causes for maternal death in our institution and the duration of hospital admission to death interval.Methods: The study included collecting and analyzing the details of maternal death in women who were admitted to St. Johns Medical College Hospital, Bengaluru, from January 2007 to December 2016. Results: Total maternal deaths were 61 and live births were 26,001 during the study period. The maternal mortality ratio (MMR) was 234.6 per 100,000 live births. Majority of maternal deaths occurred in women aged 18 - 35 years 56 (91.80%) women, primipara 45 (73.77%) and referred cases to our institution from other hospitals 52 (85.24%).                      Most of the women died in the postnatal period 54 (88.52%). Direct obstetric causes accounted for 44 (72.13%) maternal deaths and indirect causes 17 (27.86%) deaths. Preeclampsia and eclampsia were the leading causes for death 13 (21.31%) followed by acute fatty liver of pregnancy 12 (19.67%), hemorrhage 7 (13.11%) and sepsis 6 (9.83%). Anemia was present in 77.04% of women at the time of admission to our hospital. Thirty six (59.01%) women died within a week of admission to the hospital, in which 13 (21.31%) women died in less than 24hours of admission. Twenty five (40.98%) women died after a week of admission to hospital.Conclusions: Apart from the triad of preeclampsia, obstetric haemorrhage and sepsis, acute fatty liver of pregnancy has emerged as an important cause of maternal death. Most of the maternal deaths are preventable. Early detection of complications and timely referral to tertiary care hospital in St. Johns Medical College Hospital, Bengaluru, Karnataka, India decreases maternal morbidity and mortality.

Fatal outcome in acute fatty liver of pregnancy and review of literature

Acute fatty liver of pregnancy (AFLP) is an uncommon life-threatening disorder of pregnancy seen commonly in third trimester. When not diagnosed at right time it can lead to hepatic failure, encephalopathy, coagulopathy, maternal and fetal mortality. The clinical symptoms and signs are nonspecific, and it needs to be identified early in order to prevent poor outcome. The gold standard for diagnosis of AFLP is liver biopsy, which is difficult in an acute setting and in abnormal coagulation profile hence the diagnosis is usually based on clinical criteria   Early termination of pregnancy and good intensive care support are the mainstay of management. The authors here presented a case report where even early delivery and good critical care failed to prevent maternal mortality. Review of literature regarding etiopathogenesis, management and recurrences of AFLP are also discussed.

Analysis of changes in clinical characteristics and laboratory indexes of 54 cases of acute fatty liver of pregnancy / 中华肝脏病杂志

Objective@#To investigate the changes in clinical characteristics and laboratory indexes before and after the termination of pregnancy in patients with acute fatty liver of pregnancy (AFLP).@*Methods@#Patients with acute fatty liver of pregnancy who had been admitted to the Department of Obstetrics and Gynecology at the Second Affiliated Hospital of Chongqing Medical University and Chongqing Municipal People’s Hospital of Jiangbei District between 2007 and 2018 were selected. Clinical characteristics and complications during diagnosis and treatment, changes in blood coagulation, liver and kidney function, and postpartum recovery were collected for retrospectively analysis.@*Results@#54 cases with average gestational age of 35.0±1.7 weeks at third trimester of pregnancy with AFLP were treated. The most common gastrointestinal symptoms were yellow urine, nausea and vomiting. All patients had elevated bilirubin. 90.7% patients had changes in blood coagulation function and 68.5% had elevated serum creatinine. Transaminase levels were dropped rapidly within 1-2 days after the termination of pregnancy. Total bilirubin recovery was slow and partially recovered after 6-8 days. Serum creatinine and BUN increased slightly after delivery, reaching a peak at 3-4 days and then began to deplete. There was slight change in prothrombin time and fibrinogen after delivery, but returned to normal level after 5-6 days. The most common complications were AKI (74.1%), LF (42.6%), PPH (40.7%) and DIC (33.3%). Twenty-three of the 54 cases (42.6%) progressed to acute liver failure. AFLP complicated with ALF course was significantly longer than healthy controls, and the disease severity was significantly increased, with a mortality rate of 17.4% (4/23), and 0 in healthy controls. The difference was statistically significant.@*Conclusion@#Early diagnosis and termination of pregnancy are the key factors to determine the prognosis of pregnant patients with acute fatty liver. Blood coagulation function does not deteriorate after termination of pregnancy and renal function begins to recover after 4 days with slight restoration of liver function. The control of complications is an important factor to determine the prognosis of patients.

Hígado graso agudo del embarazo. Reporte de caso / Acute fatty liver of pregnancy. Case report

Acute fatty liver of pregnancy is a rare condition that occurs most frequently in the third trimester. There is severe liver dysfunction, associated with renal impairment and prolonged clotting time. These hepatic and extra-hepatic complications can cause significant maternal morbidity and mortality, and the definitive treatment is the immediate interruption of pregnancy. We present a case of complicated acute fatty liver of pregnancy in a primigravid, 40-year-old patient who presented epigastralgia of sudden onset together with generalized lethargy, nausea and vomiting. Laboratory exams showed impaired hepatic and renal function along with hypoglycemia; we diagnosed acute fatty liver of pregnancy. The patient was treated to correct the metabolic alterations, and cesarean section was performed with improvement of laboratory parameters and complete postoperative recovery.

El hígado graso agudo del embarazo es una condición rara que ocurre más comúnmente en el tercer trimestre. Produce disfunción hepática severa asociada a alteración renal y tiempos de coagulación alargados. Se vincula a complicaciones hepáticas y extrahepáticas que pueden causar morbimortalidad materno-fetal significativa y el tratamiento definitivo es la interrupción inmediata del embarazo. Se presenta un caso de hígado graso agudo del embarazo en paciente de primigesta de 40 años, quien presentó epigastralgia de aparición súbita, junto con letargia, náuseas y vómitos. Los exámenes de laboratorio mostraron alteración de las pruebas de funcionalismo hepático y retal, junto con hipoglucemia, realizándose el diagnóstico de hígado graso agudo del embarazo. La paciente fue tratada para corregir las alteraciones metabólicas y se practicó cesárea, presentando mejoría de los parámetros de laboratorio y recuperación postoperatoria completa.

The characteristics of laboratory parameters evolution in patients with acute fatty liver of pregnancy and the effect of plasma exchange on those parameters / 中国中西医结合急救杂志

Objective To observe the variation tendency of laboratory indexes after delivery in pregnant women with acute fatty liver of pregnancy (AFLP) and the effects of plasma exchange (PE) on the patients prognoses and those parameters. Methods The data of 37 patients with AFLP admitted to the Department of Intensive Care Unit (ICU) of Anhui Provincial Hospital from January 2009 to June 2017 were retrospectively analyzed and the authors tried to clarify the natural course of AFLP; all the selected patients met the Swansea criteria of the AFLP diagnosis. The patients were divided into a PE group (9 cases) and a non-PE group (28 cases) according to whether they were treated with PE or not. At the same time, the effects of PE on liver function and coagulation parameters in the patients were analyzed. Results The most common clinical symptoms of 37 AFLP patients were as follows: nausea and vomiting (21 cases, incidence 56.8%), jaundice (21 cases, 56.8%), upper abdominal pain (14 cases, 37.8%), and more than 90% of patients had more than one symptom. All 37 patients with AFLP had abnormal liver function, their differences in the severity degrees were great, and the level of aspartate aminotransferase (AST) was (271.65±265.90) U/L;the levels of creatinine (SCr) of 72.9% patients (27 cases) were more than 140 mmol/L; the prothrombin times (PT) of 72.97% patients (27 cases) were more than 15 seconds; all patients had received blood product transfusion. Liver function and blood coagulation parameters mostly returned to normal within 1 week after delivery: AST rapidly decreased on the 1 - 2 days after delivery and then continued to decrease; all the cholesterol levels in the two groups decreased to the lowest levels within 3 - 4 days after delivery and then began to rise; the total bilirubin (TBil) levels kept on basically unchanging after delivery; in the patients with levels of platelet counts (PLT) on admission ≥ 150×109/L, on the second day after delivery, their counts returned to approximately 100×109/L, while in patients with PLT < 150×109/L on admission, on the sixth day after delivery, the counts began to slowly increase to about 100×109/L; the patients with fibrinogen (Fib) levels > 1.5 g/L on admission, the levels slightly decreased 2 days before delivery, then gradually recovered and stabilized after delivery, while the patients with Fib < 1.5 g/L, the levels began to rise slowly on the second day after delivery; prothrombin time (PT) and activated partial thromboplastin time (APTT) were basically return to the normal reference ranges on the fifth day after delivery. The PE therapy could significantly improve the patients' laboratory indexes: the levels of AST, TBil, PLT, PT were significant lower immediately and on the second day after PE compared with those levels before the PE [AST (U/L): 197 (114, 383), 116 (65, 311) vs. 239 (125, 430), TBil (μmol/L):109 (67, 126), 125 (103, 178) vs. 164 (99, 198), PLT (×109/L): 96.44±33.10, 89.89±33.03 vs. 126.22±40.83, PT (s): 17.82±5.93, 18.36±3.19 vs. 22.67±8.44, all P < 0.05]. Conclusion After terminating the delivery in time for about one week, the AFLP patients' various indexes may basically return to normal; PE therapy can improve the patients' liver function and coagulation indexes.

The whole series of artificial liver support system for the treatment of acute fatty liver in pregnancy: a case report / 中国实用护理杂志

Objective To conclude nursing experience of full series artificial liver support system in the treatment of acute fatty liver in pregnancy. Methods Development of artificial liver support system according to patient's condition, total continuous renal replacement therapy 7days, double plasma molecular absorb system 3 times, plasma replacement 3 times, molecular adsorbent circulation system 2 times. Results After treatment, the patient was discharged after 20 days in hospital. Conclusions The full range of artificial liver support system can effectively treat the patients with acute fatty liver in pregnancy, it will greatly reduce the mortality of patients with acute fatty liver in pregnancy.

Esteatose hepática aguda da gestação: relato de caso / Acute fatty liver of pregnancy: case report

A esteatose hepática aguda da gestação é uma patologia obstétrica rara e grave, associada a alta mortalidade materna e fetal. O seu diagnóstico é difícil devido a sobreposição clínica com outras patologias graves, como a pré-eclampsia e a síndrome HELLP. O relato de caso apresentado neste estudo descreve a ocorrência de esteatose hepática aguda da gestação em uma mulher de 40 anos, multípara, previamente hígida, que evolui com insuficiência hepática e renal. A identificação precoce desta patologia, a pronta interrupção da gestação e o suporte intensivo adequado após o parto, contribuíram para o bom prognóstico da paciente.

Acute fatty liver of pregnancy (AFLP) is a rare and serious obstetric pathology associated with maternal and fetal mortality. The diagnosis difficulty may be overlapped with other serious diseases, such as preeclampsia and HELLP syndrome. In this study, the case report described the occurrence of acute fatty liver of pregnancy in a woman, 40 years old, multiparous, previously healthy, evolving with liver and kidney failure. Early identification of this condition, the prompt termination of pregnancy and adequate intensive support after delivery contributed to the good prognosis of the patient and patient's recovery

Esteatosis hepatica aguda en el tercer trimestre de embarazo gemelar: reporte de caso / Acute hepatic esteatosis during third trimester twin pregnancy: case report

Introducción: La esteatosis hepática aguda del embarazo (EHAE) es una patología metabólica grave e infrecuente caracterizada por esteatosis hepática microvesicular que conduce a insuficiencia hepática aguda. Aparece generalmente en el tercer trimestre de gestación y se reconoce el embarazo gemelar como un factor de riesgo para su desarrollo. Presentación del caso: Paciente femenina de 25 años sin antecedentes médicos, 2 embarazos, 1 parto y 0 abortos, cursando embarazo gemelar de 31 semanas de gestación, consultó por presentar cuadro de dolor abdominal, vómitos e ictericia. Se sospechó hepatitis aguda y se decidió hospitalizar. Al ingreso una ecografía obstétrica precisó latidos fetales ausentes decidiéndose interrupción del embarazo de urgencia. La paciente evolucionó con síndrome confusional y epistaxis masiva requiriendo transfusión. Exámenes de ingreso evidenciaron insuficiencia renal, insuficiencia hepática y trombocitopenia. Ingresó a Unidad de Paciente Crítico con diagnóstico de síndrome de HELLP requiriendo soporte con ventilación mecánica. Persistió con mala evolución clínica por lo que se solicitó tomografía de abdomen y pelvis mostrando signos de pancreatitis aguda y hematoma subcapsular hepático. Exámenes de control objetivaron hiperbilirrubinemia, transaminasas elevadas, hipoalbuminemia, hipocolesterolemia y tiempo de protrombina elevado. Se planteó diagnóstico de esteatosis hepática aguda del embarazo. Ingresó a lista de espera para trasplante hepático. Posteriormente evolucionó con mejoría espontánea de la función hepática y se logró extubar tras dos semanas de hospitalización. Discusión: El desarrollo de esteatosis hepática aguda del embarazo es infrecuente y potencialmente grave debiéndose interrumpir el embarazo precozmente y manejarse en unidades de paciente crítico debido a su alta mortalidad.

Introduction: Acute hepatic esteatosis of pregnancy (AHEP) is a serious and uncommon metabolic pathology characterized by microvesicular liver steatosis leading to acute liver failure. It usually appears in the third trimester of gestation and twin pregnancy is recognized as a risk factor for its development. Case Report: A 25-year-old female patient with no medical history, 2 pregnancies, 1 childbirth and 0 abortions, having a twin pregnancy of 31 weeks' gestation, consulted for abdominal pain, vomiting and jaundice. Acute hepatitis was suspected and it was decided to hospitalize. On admission, an obstetric ultrasonography screened for absent fetal beats, deciding to interrupt the pregnancy. The patient evolved with confusional syndrome and massive epistaxis requiring transfusion. She presented renal failure, hepatic failure, and thrombocytopenia. He entered a Critical Patient Unit with diagnosis of HELLP syndrome requiring support with mechanical ventilation. It persisted with poor clinical evolution, requiring tomography of the abdomen and pelvis showing signs of acute pancreatitis and subcapsular hepatic hematoma. Control examinations aimed at hyperbilirubinemia, elevated transaminases, hypoalbuminemia, hypocholesterolemia and elevated prothrombin time. A diagnosis of acute fatty liver of pregnancy was made. He entered the waiting list for liver transplantation. Subsequently it evolved with spontaneous improvement of the liver function and was able to extubate after two weeks of hospitalization. Discussion: The development of acute fatty liver of pregnancy is infrequent and potentially serious due to early termination of pregnancy and management in critical patient units due to its high mortality.

Maternal Death due to Acute Fatty Liver of Pregnancy / 대한법의학회지

Acute fatty liver of pregnancy (AFLP) is one of the sudden unexpected causes in pregnancy and maternal deaths. It has been considered as a potential fatal disease in pregnancy, but the postmortem findings of AFLP is not well known. Because an unexpected maternal death may lead to a legal dispute, forensic pathologists should be aware of clinical presentations and postmortem findings of AFLP. Therefore, we presented our case and reviewed with literatures.

Hígado graso agudo del embarazo / Acute fatty liver of pregnancy

El hígado graso agudo del embarazo, descrito en 1934, es una complicación gestacional poco frecuente y potencialmente fatal. El daño hepático materno ocurre por depósito citoplasmático perinuclear de microvesículas de grasa en el hepatocito relacionada con deficiencia enzimática autosómica recesiva en la vía de la oxidación mitocondrial de los ácidos grasos del feto. El objetivo de este artículo es exponer un caso de hígado graso agudo del embarazo acontecido en nuestro medio con criterios clínicos compatibles así como estudio histopatológico confirmatorio. Se trata de una paciente de 43 años procedente y residente de la ciudad de El Alto, La Paz (Bolivia), presenta ictericia, coluria, se automedica con paracetamol, asociándose al cuadro clínico nauseas acompañadas con vómitos, ausencia de movimientos fetales de producto único de 35 semanas; durante su evolución en UTI presenta encefalopatía hepática grado II y hemorragia digestiva, fallece a los 3 días de internación en UTI, se realiza necropsia hepática compatible con hígado graso agudo del embarazo. Cabe destacar la realización de estudio histopatológico en el presente caso, la cual no se realiza de manera rutinaria en nuestro medio, el cual reporta esteatosis hepática macro y microvacuolar asociada a proceso inflamatorio crónico activo acentuado difuso compatible con hígado graso agudo del embarazo, lo cual apoya los criterios clínicos de Swansea.

Acute fatty liver of pregnancy, described in 1934, is a rare but potentially fatal gestational complication. The damage in mother's liver is produced by perinuclear cytoplasmic deposit of fat micro vesicles in hepatocyte related with autosomal recessive enzymatic deficiency in mitochondrial oxidative fatty acids pathway of fetus. The objective of this paper is present an acute fatty liver of pregnancy occurred with clinical criteria and confirmation histopathological study. The present case is about a 43-year old patient, resident of El Alto, La Paz (Bolivia), with jaundice, choluria, auto medicated with acetaminophen, curses also with nausea and vomiting, absence of fetal movements of 35-week fetus, during her evolution in ICU presents hepatic encephalopathy and digestive hemorrhage, dies at 3th day in ICU, hepatic necropsy confirms acute fatty liver of pregnancy. It's worth mentioning the histopathological study, scarcely performed in our country, which, in this case, reports macro and microvacuolar steatosis in liver, associated to an active diffuse chronic inflammatory process compatible with acute fatty liver of pregnancy, in concordance with clinical Swansea criteria.

Acute Fatty Liver of Pregnancy.

Acute fatty liver of pregnancy (AFLP) is a rare life-threatening complication of pregnancy. It usually occurs in late third trimester. There is usually severe liver dysfunction with hypofibrinogenemia, hypoalbuminemia, hypocholesterolemia and prolonged clotting times. The most critical component of caring for a woman with AFLP is the delivery of her fetus.

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.

Fallo multiorgánico en adolescente con hígado graso agudo del embarazo / Multiorganic failure in adolescent with acute fatty liver of pregnancy

El hígado agudo graso del embarazo es considerado como una rara y catastrófica disfunción hepatocelular que se asocia con la gestación. Presentamos el caso de una joven adolescente con síntomas de la enfermedad a las 38,5 semanas de gestación. Ella en un periodo de 10 días desarrolla cuadro de astenia, vómitos, ictericia, dolor abdominal difuso y patrón humoral de insuficiencia hepática. Fue necesario una cesárea de urgencia para beneficio materno, ingresando de inmediato en cuidados intensivos. El perfil clínico-humoral, estudios imagenológicos y de laparoscopia confirmaron la hipótesis diagnóstica. Presentó complicaciones obstétricas que motivaron múltiples reintervenciones. Con una evolución adversa y fallo de múltiples órganos, requirió ventilación mecánica y soporte hemodinámico, hasta que fallece tras 11 días de tratamiento intensivo. Confirmamos la alta mortalidad de la enfermedad cuando produce fallo multiorgánico, y la necesidad de un diagnóstico precoz y tratamiento intensivo que incluya la posibilidad del trasplante hepático.

The acute fatty liver of pregnancy is considered like a rare and catastrophic hepatocellular dysfunction associated with the pregnancy. We present the case of a young adolescent with symptoms of the disease when she had 38.5 weeks of gestation. In a period of 10 days she develops asthenia, vomits, jaundice, diffuse abdominal pain and laboratory profile of hepatic insufficiency. Was necessary an emergent caesarean for maternal benefit and she were admitted immediately on intensive care unit. The clinical and humoral profile, image studies and laparoscopy confirmed the diagnostic hypothesis. She presented many obstetrical complications that motivated several surgeries. With an adverse evolution and multiorganic failure, she also required a mechanical ventilation and hemodynamic support, until she dies after 11 days of intensive treatment. We confirm the high mortality of the disease when it produces multiorganic failure, and the necessity of a precocious diagnosis and intensive treatment, including the possibility of liver transplant.

Acute fatty liver of pregnancy: A case report / 대한산부인과학회지

Acute fatty liver of pregnancy are relatively rare but extremely dangerous, because they may quickly develop into a fulminant disease and become a serious life-threatening disorder for mother and fetus in the third trimester. Therefore, early diagnosis, prompt delivery and intensive supportive care the cornerstones in the management of acute fatty liver of pregnancy. Clinical findings in acute fatty liver of pregnancy vary because it may occur with varying degrees of clinical severity and in conjunction with other third trimester symptoms, making early diagnosis difficult. However, careful history and physical examination, in conjunction with compatible laboratory and imaging results, are often sufficient to make the diagnosis, and liver biopsy is rarely indicated. We have experienced a case of acute fatty liver of pregnancy presenting as early hepatic encephalopathy, renal failure which developed during the third trimester. We diagnosed acute fatty liver of pregnancy based on clinical presentation and laboratory abnormalities. Despite of prompt delivery and adequate supportive care management, this severe complication of pregnancy has had an adverse outcome for mother.