Acute fatty liver of pregnancy.

Acute fatty liver of pregnancy (AFLP) is a rare, potentially fatal complication that occurs in the third trimester or early postpartum period. Although the exact pathogenesis is unknown, this disease has been linked to an abnormality in fetal fatty acid metabolism. Early diagnosis of AFLP sometimes can be difficult because it shares features with other common conditions such as pre-eclampsia, viral hepatitis and cholestasis of pregnancy. However, a 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. Supportive care and expeditious delivery are essential to optimal maternal-fetal outcomes and remain as the mainstay treatment for AFLP.     

Acute fatty liver of pregnancy: a clinicopathologic study of 35 cases

Acute fatty liver of pregnancy is a disease of the third trimester which is generally considered to be rare and to have a grave prognosis. This study found an optimistic outlook for patients with acute fatty liver of pregnancy due to early termination of the pregnancy as well as the recognition of milder cases. In prospectively followed women, a maternal mortality of 8% and a fetal mortality of 14% were observed. The disorder also appears to be more common than previously suspected and should be considered in all women with liver dysfunction in late pregnancy, even if they are anicteric. Histologically, the characteristic fine droplet steatosis usually produces distinct vacuolization in sections stained with hematoxylin-eosin. However, early in the course of the illness, liver cells have a ballooned appearance and the presence of lipid is masked. When accompanied by a significant necroinflammatory reaction, this stage may be difficult to distinguish from acute viral hepatitis. Whenever acute fatty liver of pregnancy is suspected, a small piece of the biopsy should be reserved for special stains to confirm the presence of lipid in frozen sections. Significant loss of hepatic parenchyma is a regular accompaniment of acute fatty liver of pregnancy and is due to hepatocytolysis, acidophilic degeneration and liver cell atrophy. Extramedullary hematopoiesis and giant mitochondria are often present; the latter change is probably an adaptive or degenerative response to an altered metabolic environment. Despite the frequent presence of signs and symptoms of toxemia in patients with acute fatty liver of pregnancy, no histologic overlap was observed, suggesting that they represent distinct etiologic entities.     

Acute fatty liver in pregnancy

When confronted with liver abnormalities during the third trimester of pregnancy, one should consider acute fatty liver of pregnancy. The differential diagnosis with (pre-)eclampsia and HELLP syndrome is sometimes difficult. In these cases a liver biopsy is helpful though rarely performed during pregnancy. After delivery of the child the liver test abnormalities will ultimately disappear. Recent publications reveal that a dysfunction in the beta-oxidation of mitochondrial fatty acids may contribute to the aetiology of this rare disorder. We describe a case of acute fatty liver in pregnancy, with liver dysfunction (decreased albumin, prolonged prothrombin time) slowly returning to normal after delivery. Testing for disorders in beta-oxidation of mitochondrial fatty acids did not reveal abnormalities in mother or child.     

Acute fatty liver of pregnancy:Over six months follow-up study of twenty-five patients

AIM:To evaluate the prognosis of patients with acute fatty liver of pregnancy(AFLP)6 mo or longer after discharge.METHODS:The records of pregnant patients diagnosed with AFLP at Beijing Ditan Hospital over a 16-year period were reviewed in November 2012.Patients weremonitored using abdominal ultrasound,liver and kidney functions,and routine blood examination.RESULTS:A total of 42 patients were diagnosed with AFLP during the study period,and 25 were followed.The mean follow-up duration was 54.5 mo(range:6.5-181 mo).All patients were in good physical condition,but one patient had gestational diabetes.The renal and liver functions normalized in all patients after recovery,including in those with pre-existing liver or kidney failure.The ultrasound findings were normal in12 patients,an increasingly coarsened echo-pattern and increased echogenicity of the liver in 10 patients,and mild to moderate fatty liver infiltration in 3 patients.Cirrhosis or liver nodules were not observed in any patient.CONCLUSION:Acute liver failure and acute renal failure in AFLP patients is reversible.Patients do not require any specific long-term follow-up after recovery from AFLP if their liver function tests have normalized and they remain well.     

Acute fatty liver of pregnancy

We have cared for two women with acute fatty liver of pregnancy in the past two years. Both patients survived as did three of their four babies. This compares with published mortality rates of up to 85% with this condition. Both patients presented with symptoms and signs of impending liver failure during the third trimester of pregnancy. Both patients were jaundiced, had elevated aminotransferases, leukocytosis, hepatic encephalopathy, and disseminated intravascular coagulation. The diagnosis was confirmed by liver biopsy in both. We attribute the unusually good outcome of these women and their children to early recognition of their disorder and prompt delivery once the diagnosis was considered.This work was supported in part by Training Grant AM 07024 from the National Institutes of Health.     

Acute fatty liver of pregnancy： An update on pathogenesis and clinical implications

INTRODUCTION Acute fatty liver of pregnancy (AFLP) is a maternal liver disease unique to pregnancy. It was first described in 1934 as “yellow acute atrophy of the liver[1]” and was described as a specific clinical entity in 1940[2]. AFLP is a rare, buts…     

Acute fatty liver of pregnancy. A reassessment based on observations in nine patients

Acute fatty liver of pregnancy was diagnosed in nine patients over a 10-year period. Eight patients had severe hepatic dysfunction typical for this syndrome and one had subclinical disease but typical hepatic histologic findings. All patients survived with little fetal wastage; all had preeclampsia. Histologic findings included cholestasis, hepatocellular necrosis, and inflammation, as well as microvesicular fat. Histologic findings from biopsy specimens of four of seven patients were initially misinterpreted as hepatitis. This disorder may have both a broad clinical and histologic spectrum; it is probably not rare but often misdiagnosed, perhaps as viral hepatitis. The concurrence of this disorder with toxemia of pregnancy suggests that these entities may be pathophysiologically related.     

Acute fatty liver during pregnancy: report of case

Acute fatty liver during pregnancy constitutes a rarely clinical entity with unknown pathogenesis and etiology. Its clinical picture is similar to preeclampsia including liver failure manifestations. Here in me expose a clinical case of a young woman 35 week first pregnancy with mild preeclampsia symptoms. We include a review of the literature.     

Acute fatty liver of pregnancy showing microbial infection in the liver

A 24-year-old, nulliparous woman in her 30th week of pregnancy was admitted due to threatened premature delivery. Ritodrin chloride relieved the premature contraction of the uterus but jaundice and drowsiness appeared 7 weeks later. Laboratory data revealed disseminated intravascular coagulation (DIC) with intrahepatic cholestasis, and ultrasound examination showed fatty liver. The patient was diagnosed with acute fatty liver of pregnancy (AFLP). Emergency delivery by Caesarean section was performed at 37 weeks of pregnancy and the liver function and DIC improved immediately. Liver biopsy 13 days after delivery showed nuclear swelling and cytoplasmic ballooning with mild fatty deposition. These findings were relatively compatible with acute AFLP. Higher magnification and electron microscopy revealed intracytoplasmic bacteria and fungus in the residual stage. The bacterial infection could be considered related to AFLP.     

妊娠急性脂肪肝与麻醉——13例临床分析

目的探讨妊娠急性脂肪肝（AFLP）的临床特点、围术期处理、母亲及围产儿结局,以便早期诊断和及时治疗,改善预后。方法对近3年我院收治的13例AFLP患者的症状、实验室检查、临床过程、围术期处理以及母亲和围产儿结局进行回顾性分析。结果AFLP76．92％发生于初产妇,男性胎儿占69．23％,前驱症状为乏力、纳差、恶心、呕吐、黄疸,实验室检查示凝血病、肝功能异常、低血糖、低蛋白血症、尿胆红素阴性,产妇死亡2例,占15．4％,无围产儿死亡。麻醉方法采用椎管内麻醉（10例,76．9％）,全身麻醉（2例,15．4％）。结论妊娠急性脂肪肝是发生于妊娠晚期的一种严重并发症,早期诊断、尽快终止妊娠和围术期积极的对症支持治疗是提高母儿预后的关键。麻醉方法应个体化,有明显凝血病的患者以全身麻醉首选。     

Acute exacerbation in patients with liver cirrhosis: a clinicopathological study.

The incidence, clinicopathologic features and etiology of acute exacerbation occurring in patients with liver cirrhosis were assessed prospectively among 332 hepatitis B surface antigen (HBsAg) positive and 71 HBsAg negative patients. During an 11-year period and a mean follow-up duration of 26.8 months, 148 acute exacerbation occurred in 107 HBsAg positive patients and 32 episodes occurred in 18 HBsAg negative patients. The calculated annual incidence was 11.5%. The clinical, laboratory and histologic features were similar to those in patients with chronic hepatitis. Confluent hepatic necrosis and alphafetoprotein elevation over 100 ng/ml occurred frequently, particularly in HBeAg positive patients. In general, acute exacerbations in HBsAg negative patients were less severe than their HBsAg positive counterparts. Of the exacerbations in HBsAg positive patients, 54.8% of the HBeAg positive ones and 38.6% of the HBeAg negative ones were attributable to hepatitis B virus reactivation, while 4.8% and 7.9%, respectively, were due to hepatitis delta virus superinfection. The others might be the results of hepatitis non-A, non-B virus superinfection or increased piecemeal necrosis. The immediate outcome of acute exacerbations in cirrhotic patients was usually good, although 13.8% developed hepatic decompensation and 4.4% died. Further follow-up study is required to evaluate the long-term effect of the frequent occurrence of bridging hepatic necrosis, high elevation of alphafetoprotein and hepatic decompensation during acute exacerbation in cirrhotic patients.     

Acute fatty liver of pregnancy complicated with anterior pituitary insufficiency

Acute fatty liver of pregnancy complicated with anterior pituitary insufficiency in a 24-year-old nullipara woman who presented fever and progressing liver damage after the delivery by Cesarean section is described. The liver biopsy revealed severe fatty changes with microvesicular fat drops in the hepatocytes. Serum growth hormone and adrenocorticotropic hormone levels were low, and did not respond to the stimulation. The daily urinary excretion of 17-hydroxycorticosteroid was also low. Acute fatty liver of pregnancy and antehypophyseal insufficiency were diagnosed. Secondary adrenal failure was also suspected. The co-existing hypercoagulable state could cause an ischemic attack on the pituitary gland.