Acute fatty liver of pregnancy in 3 tertiary care centers

OBJECTIVE: The purpose of this study was to determine the demographics, clinical presentation, and maternal and neonatal outcomes in patients who were diagnosed with acute fatty liver of pregnancy over a 10-year period. STUDY DESIGN: This was a multicenter retrospective study of women with the diagnosis of acute fatty liver of pregnancy. Records were reviewed for symptoms, laboratory findings, clinical course, and maternal and perinatal outcomes. RESULTS: Sixteen cases of acute fatty liver of pregnancy were identified. Three of the 16 cases had multiple gestations (18%). Eleven of the 16 cases were diagnosed in the antepartum period, and 5 cases were diagnosed within 4 days after delivery. Nausea and vomiting were the most common symptoms (75%). There were 2 maternal deaths (12.5%) and 3 fetal deaths (15%). CONCLUSION: We recommend that patients with persistent nausea, vomiting, or epigastric pain in the third trimester receive evaluation of liver enzymes, renal function, and a complete blood count to rule out the diagnosis of acute fatty liver of pregnancy.     

Acute fatty liver of pregnancy associated with pancreatitis: a life-threatening complication

OBJECTIVE: Inadequate data describe pancreatitis as a complication in women with acute fatty liver of pregnancy. Our purpose was to report our experience with this complication, which included maternal and perinatal outcomes. STUDY DESIGN: Medical records of patients with acute fatty liver of pregnancy and/or pancreatitis were reviewed over a 15-year period. Records were reviewed for symptoms, laboratory findings, clinical course, and maternal and perinatal outcomes. RESULTS: Persistent nausea and vomiting were the symptoms in 10 of 12 patients (83%). The average maternal age was 26.3 years (range, 21-37 years). There were two maternal deaths (17%); one death occurred despite liver transplantation. Maternal morbidity included encephalopathy (50%), acute respiratory distress syndrome (17%), and renal failure (33%) that resulted from acute tubular necrosis (3 cases) and acute cortical necrosis (1 case) that required renal transplantation. Of the 12 patients, 11 patients (91%) had an elevated serum lipase level, and another patient had an elevated serum amylase level (9%). Eight of 12 patients (67%) underwent radiologic studies, of which 7 patients (88%) were diagnostic for pancreatitis. Radiographic and autopsy findings noted pancreatic inflammation (3 cases), pseudocyst formation (3 cases), and hemorrhagic pancreatitis (1 case). The radiographic and serum laboratory abnormalities occurred after the onset of hepatic and renal abnormalities in all cases. CONCLUSION: Pancreatitis is a potentially lethal complication of acute fatty liver of pregnancy, and all patients with this diagnosis should undergo screening for the abnormality. Pancreatic abnormalities typically appear after hepatic and renal dysfunction.     

影响妊娠期急性脂肪肝预后的危险因素分析

【摘 要】 目的：探讨影响妊娠期急性脂肪肝（AFLP）患者预后的危险因素,为改善AFLP患者预后提供临床依据。方法：回顾性分析AFLP患者62例,以患者生存（45例）或死亡（17例）作为预后分类,对患者一般资料及各实验室指标进行单因素分析及多因素Logistic回归分析。结果：2组患者的红细胞计数（RBC）、红细胞比容（HCT）、白细胞计数（WBC）、血红蛋白（HGB）、血小板（PLT）、总蛋白（TP）、总胆红素（TBIL）、白蛋白（ALB）、直接胆红素（DBIL）、肌酐（Cr）、凝血酶原时间（PT）、纤维蛋白原（Fib）、活化的部分凝血酶时间（APTT）、发病到就诊时间≤7 d的例数、就诊到终止妊娠时间≤24 h的例数、发病到终止妊娠时间≤14 d的例数,差异有统计学意义（均P＜0.05）。就诊到终止妊娠超过24 h、发病到终止妊娠时间超过14 d是AFLP患者预后的危险因素。结论：就诊到终止妊娠时间和发病到终止妊娠时间是影响AFLP预后的重要因素。     

Three cases of acute fatty liver of pregnancy: Postpartum clinical course depends on interval between onset of symptoms and termination of pregnancy

We report our experience with three cases of acute fatty liver of pregnancy. Case 1 complained of hydrodipsia 4 days before delivery. Case 2 presented with nausea, vomiting and dizziness 6 days before delivery. Case 3 developed loss of appetite and general fatigue with jaundice 10 days before delivery. They underwent termination of pregnancy after diagnosis was made. Case 3 still developed hepatic encephalopathy, and finally she required liver transplantation. We hypothesise that the interval between the onset of symptoms and termination of pregnancy is an important factor for acuity of the disorder and patient morbidity or mortality.     

妊娠期急性脂肪肝及其临床诊疗分析

目的：探讨妊娠期急性脂肪肝（AFLP）的发病特点、诊疗方案、并发症及转归,以提高母婴存活率。方法：回顾性分析近7年在我院确诊为AFLP的14例患者的分娩方式、诊疗经过及预后。结果：14例AFLP患者1例死亡,8例产后出血,2例多脏器功能衰竭,新生儿轻度窒息1例,重度窒息2例,死胎1例。结论：AFLP早期诊断、及早终止妊娠及各科室的相互配合是十分必要的。应严密监测及调节凝血功能、肝肾功能,防止AFLP患者产后出血及多脏器功能衰竭,提高母婴存活率。     

妊娠期急性脂肪肝的早期识别

妊娠期急性脂肪肝是罕见的妊娠期特发性疾病,严重危及孕产妇及围产儿的生命安全。早期识别和诊断是减少严重并发症、改善预后的关键。临床医师需重视妊娠期急性脂肪肝患者早期非特异的临床症状和高危因素,结合实验室检查,为临床早期诊断提供依据。     

妊娠期急性脂肪肝的诊断与治疗

妊娠期急性脂肪肝(AFLP)是妊娠晚期一种特发性的严重并发症,孕产妇及围产儿死亡率高。该病缺乏特异性临床表现,需与妊娠期肝内胆汁淤积症等相鉴别,早期易误诊。肝活检是诊断AFLP的金标准,但根据病史、临床表现结合实验室及影像学检查,不必肝脏穿刺也可以做出临床诊断。迅速终止妊娠并给予支持治疗是主要治疗措施,对改善AFLP患者母胎结局至关重要。     

血浆置换在妊娠期急性脂肪肝中的应用

妊娠期急性脂肪肝是一种特发于妊娠期凶险的肝功能障碍的并发症。血浆置换是治疗妊娠期急性脂肪肝的一种新手段。本文从血浆置换机制、适用指征、方法及注意事项等方面总结了其在妊娠期急性脂肪肝中的应用,从而提高医护人员的认识。     

妊娠期急性脂肪肝9例临床分析

目的:探讨妊娠期急性脂肪肝(AFLP)的临床特点,以便早期诊断和及时治疗。方法:对近10年我院收治的9例AFLP患者的临床资料进行回顾性分析。结果:所有患者白细胞及尿酸均升高,8例纤维蛋白原降低,8例转氨酶升高,7例胆红素升高,7例空腹血糖降低,6例尿素氮升高,5例血肌酐升高,5例白蛋白降低,5例血小板降低、凝血时间延长。孕产妇治愈8例,死亡1例,死亡率为11%,11例围生儿中,存活7例,死亡4例,死亡率为36%。结论:对高危人群行产前筛查,可降低AFLP发病率;早期诊断、尽快终止妊娠和支持治疗是提高AFLP母儿预后的关键。     

妊娠急性脂肪肝35例临床分析

目的探讨妊娠急性脂肪肝（acute fatty liver of pregnancy,AFLP）的早期诊断方法和治疗原则。方法回顾性分析了1999年1月至2009年12月在北京地坛医院收治的35例AFLP患者的临床资料,并全部进行了随访。结果 AFLP临床表现以消化道症状、肝损害、凝血功能障碍为主要特征,严重者伴多脏器功能受损。孕产妇及围产儿结局：3例孕产妇死亡,5例围产儿死亡。结论早期诊断和及时终止妊娠,加强支持疗法及综合治疗是关键,可有效地降低母婴死亡率。     

Acute fatty liver of pregnancy: a retrospective study of 32 cases in South China

Abstract

Objective: To describe 32 cases of acute fatty liver of pregnancy (AFLP) and identify the potential clinical predictors of maternal and fetal outcomes in South China.

Methods: Thirty-two cases of AFLP previously treated in the First Affiliated Hospital of Nanchang University in the past 10 years were enrolled in the current study.

Results: Sex of fetus and delivery method was the potential clinical predictor of fetal outcome. The main manifestations of AFLP were jaundice (n?=?32), nausea and vomiting (n?=?28), malaise (n?=?25), and ascites (n?=?25), and its complications included acute renal failure (n?=?26), hepatic encephalopathy (n?=?17), infection (n?=?10), and postpartum hemorrhage (n?=?7). Evidence of fatty changes of liver was demonstrated by B ultrasound and computed tomography (CT). Eighteen patients were recovered due to rapid diagnosis, early termination of pregnancy and supportive treatments.

Conclusions: Male sex and vaginal delivery was the risk factor of fetal outcome. Detailed history taking and proper management of potential predictors are important in making the decision of prompt delivery and choosing the appropriate delivery method. Further studies are needed to expand our knowledge on this disease.     

Acute fatty liver of pregnancy and acetaminophen toxicity leading to liver failure and postpartum liver transplantation. A case report

BACKGROUND: Acute fatty liver of pregnancy is a rare entity. A MEDLINE English-language search from 1966 to the present revealed no reports of acetaminophen toxicity and acute fatty liver in pregnancy. CASE: An 18-year-old, African American woman, gravida 1, presented at 33 weeks' gestation with signs and symptoms consistent with acute fatty liver of pregnancy and fetal death. Markedly elevated transaminases prompted a search for other etiologies, and acetaminophen toxicity was diagnosed. Liver biopsy revealed acute fatty liver of pregnancy and toxin-induced injury consistent with acetaminophen use. The patient's condition deteriorated, resulting in fulminant hepatic failure and requiring postpartum orthotopic liver transplantation. CONCLUSION: The combination of acute fatty liver of pregnancy and acetaminophen toxicity resulted in acute liver failure. Attention to clinical and biochemical parameters can lead to diagnosis and management.     

Acute fatty liver of pregnancy: a rare pathology of the third trimester

Acute fatty liver of pregnancy is a rare clinical syndrome of pregnancy that occurs during the third trimester. Clinicians must have a high index of suspicion for this condition when a woman has nausea or vomiting during the last trimester. Early diagnosis and prompt delivery improve foetal and maternal prognosis. We report a case of a previously healthy 23-year-old woman who presented an acute fatty liver of pregnancy with intrauterine fetal death. Based on this experience as well as on medical literature, characteristics of this uncommon pathology are discussed.     

The association between acute fatty liver of pregnancy and fatty acid oxidation disorders

Acute fatty liver of pregnancy is a relatively rare but potentially fatal liver disorder of late pregnancy. Recent advances in molecular diagnostic procedures provide evidence of a genetic basis for this condition and a link to offspring disorders in fatty acid oxidation. This relationship implies the need for genetic testing and follow-up of at-risk women and their neonates.     

Diagnosis of early acute fatty liver of pregnancy

Acute fatty liver of pregnancy (AFLP) is usually diagnosed at autopsy, with severe hepatic failure, or after the appearance of extrahepatic disease. We present an atypical patient with mild AFLP in whom hepatic biopsy allowed earlier diagnosis. A low threshold for liver biopsy in patients not experiencing fulminant disease can afford diagnostic information prior to the onset of serious sequelae.     

Liver function test and pregnancy

The physiological changes in liver function in pregnancy are commonly transient, rarely permanent. Disorders arising in pregnancy, such as pre-eclampsia and eclampsia, acute fatty liver of pregnancy (AFLP), haemolysis, elevated liver enzyme and low pletelets (HELLP) syndrome, cholestasis, hyperemisis gravidarum and isolated cases of raised liver enzymes can have serious implications. Proper interpretation of liver function tests (LFTs) at an early stage can lead to timely management and may reduce complications in both mother and fetus. Normal LFTs do not always mean that the liver is normal. A number of pitfalls can be encountered in the interpretation of basic blood LFTs. The commonly used LFTs primarily assess liver injury rather than hepatic function. Abnormal LFTs may indicate that something is wrong with the liver, and they can provide clues to the nature of the problem but this is not always the case. The various biochemical tests, their pathophysiology, and an approach to the interpretation of abnormal LFTs are discussed in this review. Commonly available tests include alanine transaminase, aspartate transaminase, alkaline phosphatase, bile acid, serum bilirubin, serum albumin and prothrombin time.     

Outcome of pregnancy with severe liver disease.

OBJECTIVES: To study the incidence, presentation, and outcome of patients with severe liver disease in an urban Indian population. METHOD: 26 patients with severe liver disease were identified in the study period of one year at a teaching tertiary care institute in Mumbai. Investigations included bedside Bleeding and Clotting Time (BT/CT), coagulation profiles and liver and renal function tests. Management was directed towards initial stabilization followed by early delivery in an intensive care setting. RESULT: 80.71% of the patients had HELLP (Hemolysis, Elevated Liver enzymes, Low Platelets) syndrome. The remaining were cases of acute fulminant hepatitis and acute fatty liver of pregnancy. The most consistent finding was thrombocytopenia (88.46%). Disseminated Intravascular Coagulopathy (DIC) was the most common complication (65%). BT/CT were 100% sensitive for the diagnosis of DIC. Maternal and perinatal mortality were 42.3% and 61.5% respectively. CONCLUSION: Intensive care facilities and an early diagnosis are essential for the management of mothers with severe liver disease. Prognosis is poor for patients with fulminant hepatitis and acute fatty liver. Screening for DIC is a must. Delay in recovery of biochemical parameters may indicate atypical disease. Patient education is essential at discharge.     

Acute fatty liver in the second trimester

Background: Acute fatty liver of pregnancy is a rare, potentially fatal disease that occurs in the late third trimester or early postpartum period. The case of a woman with acute fatty liver in the second trimester is presented.Case: A 35-year-old woman, gravida 5, para 4, presented at 22 weeks’ gestation with nausea, vomiting, malaise, weight loss, and moderately elevated liver transaminase levels. The differential diagnoses included viral gastroenteritis and cholelithiasis. During the next 12 days, her transaminase levels increased, jaundice developed, her sensorium changed, and coagulopathy appeared. After the patient was transferred to a tertiary care hospital, acute fatty liver of pregnancy was diagnosed. The woman rapidly improved after delivery.Conclusion: Although acute fatty liver of pregnancy usually occurs in the late third trimester or early postpartum period, it can occur in the second trimester.     

妊娠急性脂肪肝的临床诊断及治疗方法

目的 探讨妊娠急性脂肪肝的临床诊断及治疗方法。方法 对上海市公共卫生临床中心1988年1月至2007年7月收治的36例妊娠急性脂肪肝患者的临床资料进行回顾性分析。结果 （1）临床表现：36例妊娠急性脂肪肝患者均发生于妊娠晚期,有明显的临床症状（以恶心、呕吐为主）和实验室检查特征（36例患者全部出现白细胞计数、肝酶及血清总胆红素水平升高,全部出现凝血酶原时间延长及血清白蛋白水平降低）。肝脏B超的阳性检出率为57％（17／30）,肝脏CT的阳性检出率为73％（16／22）,肝脏CT的阳性检出率高于B超（P〈0．05）。（2）分娩方式：阴道分娩12例,其中产后出血发生率为42％（5／12）,孕产妇死亡率为50％（6／12）,围产儿死亡率为50％（6／12）,新生儿窒息发生率为58％（7／12）;剖宫产分娩24例,其中产后出血发生率为42％（10／24）,孕产妇死亡率为8％（2／24）,围产儿死亡率为13％（3／24）,新生儿窒息发生率为38％（9／24）。（3）剖宫产终止妊娠加内科综合支持治疗能明显降低产后出血发生率、孕产妇死亡率、围产儿死亡率、新生儿窒息发生率。剖宫产分娩的孕产妇死亡率及围产儿死亡率与阴道分娩比较,差异有统计学意义（P〈0．05）,剖官产分娩的产后出血发生率及新生儿窒息发生率与阴道分娩比较,差异无统计学意义（P〉0．05）。（4）全部死亡病例均是从发病至接受正规治疗时间超过7d的患者。结论 结合临床症状、实验窀检查特征、肝脏B超、CT检查等,力争在发病1周内明确诊断;立即剖宫产术终止妊娠、积极内科综合支持疗法是改善母儿预后的关键。