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.     

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。     

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.     

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.     

Clinical characteristics of acute fatty liver of pregnancy in a tertiary Indonesian hospital

Acute fatty liver of pregnancy (AFLP) is a rare, often autosomal recessive disorder with a major risk for maternal and perinatal mortality and morbidity. In order to achieve a more favorable outcome, awareness of its clinical signs and symptoms and early recognition are of pivotal importance. Over a 5-year period, 18 patients were diagnosed with AFLP (one twin, 19 babies). The most common sign and symptoms were jaundice, hypoglycemia, nausea and vomiting, encephalopathy, and hypertension. Abnormal laboratory test results included elevated total/conjugated (direct) bilirubin, AST, ALT, PT, APTT, creatinine, leukocyte count, and hypoalbuminemia. Maternal and fetal mortality rate was high: 66.7% resulted in a maternal death and 57.9% in an intrauterine fetal demise (IUFD). The number of complications was found to correlate with maternal death (p?=?.042). Surviving AFLP patients had ≤3 complications, while patients with >3 complications on presentation had a high risk of maternal death (OR?=?5.0; 95% CI: 0.55–45.4). The presence of hypertension significantly increased the risk of maternal death (OR: 24.5; 95% CI: 1.1–542.8; p?=?.01). The risk of IUFD was primarily related to gestational age at delivery and birth weight. The high rate of jaundice as presenting symptom of AFLP suggests that Indonesian primary maternity care providers may often miss its important earlier signs and symptoms, in particular de novo onset of nausea and vomiting in late pregnancy.     

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.     

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。     

Maternal mortality and severe maternal morbidity from acute fatty liver of pregnancy in the Netherlands

Objective

To assess maternal death and severe maternal morbidity from acute fatty liver of pregnancy (AFLP) in the Netherlands.

Study design

A retrospective study of all cases of maternal mortality in the Netherlands between 1983 and 2006 and all cases of severe maternal morbidity in the Netherlands between 2004 and 2006, in which all 98 maternity units in the Netherlands participated. Maternal mortality ratio (MMR) and incidence of severe maternal morbidity were the main outcome measures.

Results

The MMR from direct maternal mortality from AFLP was 0.13 per 100,000 live births (95% CI 0.05-0.29). The incidence of severe maternal morbidity from AFLP was 3.2 per 100,000 deliveries (95% CI 1.8-5.7).

Conclusions

AFLP is a rare condition which still causes severe maternal morbidity and in some cases mortality. Referral to a tertiary care hospital for treatment of this uncommon disease should be considered.     

Noninvasive Swansea criteria are valuable alternatives for diagnosing acute fatty liver of pregnancy in a Chinese population

Background: This study aims to assess the diagnostic and prognostic value of Swansea criteria in diagnosing acute fatty liver of pregnancy (AFLP) in a Chinese population.

Methods: A retrospective study was conducted on 52 Chinese women diagnosed with AFLP. All selected cases were reassessed using the Swansea criteria with special focus on the noninvasive criteria, since performing a liver biopsy for this indication is rare in a Chinese population.

Results: Ninety point four percent of patients fulfilled five or more of the Swansea criteria. Thirty-one cases were positive for six or more Swansea criteria, but there were no significance differences between patients when using a cutoff criteria <6 or >6. When patients were positive for less than seven criteria, frequency of stillbirth, continuous blood purification (CBP) treatment, hysterectomy, and postpartum hemorrhage were not increased. However, patients who were positive for seven or more criteria had a significantly higher risk of stillbirth and a higher rate of CBP treatment (p?p?=?0.040). We observed a significantly elevated postpartum hemorrhage along with positivity of the Swansea criteria (p?=?0.040).

Conclusions: Swansea criteria without liver biopsy are good screening tools for AFLP diagnosis, and may be useful for assessing disease severity.     

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

Objective Acute fatty liver of pregnancy (AFLP) is a rare and serious entity associated with significant maternal and neonatal mortality and morbidity. We describe our experience with the clinical diagnosis, management and course patients with AFLP.Study design Medical records of patients with AFLP were reviewed over a 10-year period. Records were reviewed for symptoms, laboratory findings, clinical course, and maternal and perinatal outcomes.Results The incidence of AFLP was 1 in 7,270 births. The mean gestational age at onset was 34.6 ± 4.9 weeks. Eight percent of the patients were multiparous with more than three pregnancies. The prodromic phase was variable; patients complained of nausea, abdominal pain, malaise, polyuria-polydipsia syndrome and headaches followed by jaundice. The laboratory results indicated coagulopathy, liver function abnormalities and hypoglycemia. Emergency cesarean section was performed in two cases. The diagnosis has been assessed by transcutaneous hepatic biopsies processed in all patients between the 4th and 15th day (8.4 ± 4.3 days). Maternal morbidity included hypoglycaemia (40%), coagulopathy (50%) encephalopathy (30%) and renal failure (40%). There were no maternal deaths but fetal mortality is high 66%.Conclusion The early recognition of AFLP cases and prompt progressive management, including early termination of pregnancy and large dose infusion of fresh frozen plasma, the prognosis of AFLP is obviously improved.     

Complications of acute fatty liver of pregnancy treated with activated protein C

Introduction Acute fatty liver (AFL) of pregnancy is a difficult and challenging diagnosis. The treatment plan is usually supportive.Case report We present a case of a patient with AFL of pregnancy and Gram-negative endomyometritis and sepsis. Our treatment plan included the use of activated protein C (APC).Conclusion This is the first case report of the use of APC in a post-partum patient with AFL and sepsis.     

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。     

Association between gestational age and induction-to-abortion interval in mid-trimester pregnancy termination using misoprostol

Objective

The study was aimed to evaluate the effectiveness, outcome, and pain intensity of the vaginal administration of misoprostol for the induction of abortion between 13 and 24 gestational weeks.

Study design

A retrospective study was conducted at our tertiary medical center from January 2006 to December 2009 on 122 consecutive women who underwent termination of pregnancy (TOP) in the mid-trimester. They were given 400 mcg of vaginal misoprostol every 6 h, up to four doses. The induction-to-abortion interval and the level of pain experienced during the process were assessed. Success was defined by the fetus being expelled within 48 h.

Results

Vaginal misoprostol was effective in 84% (98/122) of patients. The median duration of the induction-to-abortion interval was 16 (5-48) h. The induction-to-abortion interval was correlated with gestational age, while inversely correlated with parity. A correlation was also found between gestational age and pain intensity at 12 h from induction.

Conclusion

Misoprostol is safe and effective in mid-trimester abortion induction. The induction-to-abortion interval is shorter and abortion less painful with lower gestational age. Higher parity is also associated with shorter induction to abortion interval.     

Acute fatty liver of pregnancy with survival of mother and child: a case report.

A case of acute fatty liver of pregnancy (obstetric acute yellow atrophy or acute fatty metamorphosis of the liver) is reported in which cesarean section was made and both mother and child survived. The authors suggest that the prognosis was improved by rapid termination of pregnancy. The course of the disease, differential diagnosis, treatment and prognosis are discussed.     

Implications of abnormal liver function in pregnancy and non-alcoholic fatty liver disease

Elevated liver enzyme levels can sometimes be encountered in asymptomatic pregnant women. Similar to non-pregnant subjects, women with elevated gamma glutamyltransferase or alanine aminotransferase in early pregnancy have increased risk of subsequent complications, especially gestational diabetes mellitus. In non-pregnant subjects, the commonest cause of abnormal liver function currently is non-alcoholic fatty liver disease. Risk factors include obesity, diabetes, and the metabolic syndrome. It can progress to hepatocellular carcinoma through the development of steatohepatitis, and has become the leading cause for liver transplantation in women. Found in as many as 16–18% of pregnant women, it is associated with an increased risk of pregnancy complications and abnormal foetal growth, which predisposes the offspring to the same problem subsequently. This condition probably explains the majority of the cases of “idiopathic” abnormal liver function in pregnancy, and should be looked out for in high-risk women owing to its implications on their long-term health outcome.     

血浆置换在治疗妊娠期急性脂肪肝中的应用价值

目的探讨血浆置换在治疗妊娠期急性脂肪肝的中的应用价值。 方法对2010年1月至2013年12月中山大学附属第三医院产科收治的17例采用血浆置换治疗妊娠期急性脂肪肝患者的临床资料进行回顾性分析,分析患者治疗前后的临床症状、体征、肝肾功能、凝血等指标,以及血浆置换出现的副作用。 结果(1)患者治疗前后各项检查指标的比较：白细胞(20.2±9.1)×10⁹/L与(15.2±5.9)×10⁹/L、天冬氨酸转氨酶(304.3±312.6)U/L与(81.4±99.5)U/L、丙氨酸转氨酶(332.5±348.3)U/L与(116.7±198.9)U/L、总胆红素(130.5±113.6)μmol/L与(84.3±35.5)μmol/L、凝血酶原时间(22.5±6.3)s与(19.6±6.7)s、尿素(9.6±7.8) mmol/L与(7.6±4.2)mmol/L、尿酸(515.3±167.0)μmol/L与(380.0±219.4)μmol/L,均得到明显改善(P值均<0.05);(2)血浆置换副作用小,未出现因不良反应而终止血浆置换治疗的病例。 结论血浆置换用于治疗妊娠期急性脂肪肝具有安全、有效等特点,在及时终止妊娠后使用血浆置换可有效遏制病情的发展。     

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。     

Effects of a prolonged, 72 hours,interval between mifepristone and gemeprost in second trimester termination of pregnancy: a retrospective analysis

Objective: To evaluate if the 72 hours interval between mifepristone and gemeprost has a similar efficacy compared to the 48 hours interval for second trimester termination of pregnancy

Study Design: Two-hundred and fifteen consecutive pregnant women, admitted to our hospital, for second trimester TOP, were included in this retrospective analysis. Standard protocol was followed for all patients. On the first day of the procedure oral mifepristone 200?mg was administered. After 72 (group A, n?=?78) or 48 hours (group B, n?=?113) women were admitted for administration of gemeprost 1?mg pessary as per protocol. The induction to abortion time was defined as the interval between the insertion of the first gemeprost pessary and the expulsion of the fetus.

Results: There are no significant differences in the number of pessaries in the two groups. The induction to abortion interval was longer in group A than in group B. Twenty-one women required surgical evacuation of the uterus for retained placenta or incomplete abortion without difference between groups.

Conclusion: A 48-hours interval between mifepristone and gemeprost leads to better results than a 72-hours interval, with a shorter abortion length and represents the elective method for second trimester TOP.