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

Acute fatty liver of pregnancy (AFLP) is a serious maternal illness occurring in the third trimester of pregnancy with significant perinatal and maternal mortality. Till recently, it has been considered a mysterious illness. In this editorial, we review the recent advances in understanding the pathogenesis of AFLP and discuss the studies documenting a fetal-maternal interaction with a causative association between carrying a fetus with a defect in mitochondrial fatty acid oxidation and development of AFLP. Further, we discuss the impact of these recent advances on the offspring born to women who develop AFLP, such that screening for a genetic defect can be life saving to the newborn and would allow genetic counseling in subsequent pregnancies. The molecular basis and underlying mechanism for this unique fetal-maternal interaction causing maternal liver disease is discussed.

Effects of plasma exchange combined with continuous renal replacement therapy on acute fatty liver of pregnancy

BACKGROUND: Acute fatty liver of pregnancy (AFLP) in the third trimester or early postpartum period can lead to fatal liver damage. Its traditional therapy is not very effective in facilitating hepatic recovery. The safety and effect of plasma exchange (PE)in combination with continuous renal replacement therapy(CRRT) (PE+CRRT) for AFLP still needs evaluation.METHODS: Five AFLP patients with hepatic encephalopathy and renal failure were subjected to PE+CRRT in our department from 2007 to 2012. Their symptoms, physical signs and results were observed, and all relevant laboratory tests were compared before and after PE+CRRT.RESULTS: All the 5 patients were well tolerated to the therapy. Four of them responded to the treatment and showed improvement in clinical symptoms/signs and laboratory results and they were cured and discharged home after the treatment One patient succeeded in bridging to transplantation for slowing down hepatic failure and its complications process after2 treatment sessions. Intensive care unit stay and hospital stay were 9.4 (range 5-18) and 25.0 days (range 11-42), respectively.CONCLUSION: PE+CRRT is safe and effective and should be used immediately at the onset of hepatic encephalopathy and/or renal failure in patients with AFLP.

Acute fatty liver of pregnancy associated with severe acute pancreatitis:A case report

Acute fatty liver of pregnancy is a rare disease that affects women in the third trimester of pregnancy. Although infrequent, the disease can cause maternal mortality. The diagnosis is not always clear until the pregnancy is terminated, and significant complications, such as acute pancreatitis, can occur. Pancreatic involvement typically only occurs in severe cases after the development of hepatic and renal impairment. To date, little knowledge is available regarding how the disease causes pancreatitis. Treatment involves supportive measures and pregnancy interruption. In this report, we describe a case of a previously healthy 26-year-old woman at a gestational age of 27 wk and 6 d who was admitted with severe abdominal pain and vomiting. This case illustrates the clinical and laboratory overlap between acute fatty liver of pregnancy and pancreatitis, highlighting the difficulties in differentiating each disease. Furthermore, the hypothesis for this overlapping is presented, and the therapeutic options are discussed.

Case Report-Early Detection of Acute Fatty Liver of Pregnancy in Urgent Care Department with Nonspecific Symptoms

Acute fatty liver of pregnancy is a rare but serious disorder affecting pregnancy. The rarity of this situation along with nonspecific symptoms makes it difficult to diagnose and thus causes catastrophic events. Biochemical parameters are required to diagnose this condition and differentiate it from other life threatening causes of pregnancy like HELLP syndrome and Preeclampsia. To avoid complications it is imperative to diagnose this condition at the earliest by doing blood test investigation and symptomatic management and delivery. We present a case in our hospital which came to urgent care with nonspecific symptoms and was diagnosed on blood tests and managed promptly and thus averted a catastrophic event.

Progress in the treatment of acute fatty liver of pregnancy and management of perioperative anesthesia review

Acute fatty liver of pregnancy is a rare and critical disease in obstetrics,mainly characterized by liver failure,severe coagulopathy and other clinical manifestations,with rapid progression and high mortality.This article discusses the epidemic characteristics,pathogenesis,clinical manifestations,laboratory examination,clinical diagnosis and treatment of acute fatty liver during pregnancy.This paper summarizes the related contents of perioperative anesthesia in anesthesia methods,drug selection,coagulation regulation,organ protection and postpartum disease outcome.

Acute Fatty Liver of Pregnancy： A Retrospective Analysis o 56 Cases

Background： Acute fatty liver of pregnancy （AFLP） is a rare but life-threatening complication occurring in the third trimester. It is often fatal to both mother and fetus. The complicated clinical manifestations as well as an insufficient understanding of the disease make the precise diagnosis and effective treatment of AF LP challenging. A full understanding of the risk factors, clinical features, and test findings of AFLP is critical for its timely diagnosis and treatment. Methods： We performed a retrospective study of 56 patients with AFLP between June 2008 and July 2013. We analyzed the clinical features, laboratory results, perioperative management, and patient outcomes. Results： The initial symptoms varied considerably, with nausea and vomiting （13/56, 23%） being the most common. Liver-function indexes were remarkable, including elevated levels of serum alanine aminotransferase （262.16 ± 281.71 U/L）, aspartate aminotransferase （260.98 ± 237.91 U/L）, lactic dehydrogenase （1011.76 ± 530.34 U/L）, and direct bilirubin （85.59 ± 90.02 μmol/L）. Coagulation disorders were indicated by abnormal levels offibrinogen （245.95 ± 186.11 mg/dL）, D-dimer （2.46 ±4.01 mg/L）, and fibrin degradation products （43.62 ±48.71 mg/L）. The main maternal complications were hypoproteinemia （75%）, coagulopathy （54%）, and acute renal failure （39%）. Multivariate logistic regression analysis identified prothrombin time （PT; odds ratio [OR] = 1.558, 95% confidence interval [CI] =1.248-1.946, P= 0.016） and international normalized ratio （INR; OR = 40.034, 95% CI= 2.517-636.693, P = 0.009） as risk factors. The perinatal infant death rate was related to gestational age at delivery （OR = 1.298, 95% CI- 1.040-1.618, P = 0.021）, direct bilirubin （OR = 1.05, 95% CI = 1.008 1.094, P = 0.020）, and fibrin degradation products （OR - 0.973, 95% CI = 0.950 0.996, P= 0.021）. Conclusions： Nausea and vomiting may be the most common symptoms of AFLP. Indexes of liver dysfunction and coagulation disorders should also be considered. PT and INR are risk factors for fatal complications in patients with AFLP, and perinatal mortality is linked to the level of fibrin degradation products. Timely delivery is crucial to controlling the development of AFLR

Treatment analysis of acute fatty liver of pregnancy complicated with hematogenous infection of CRKP

Acute fatty liver of pregnancy(AFLP)is an idiopathic disease with acute and critical onset.Although the incidence of AFLP is relatively low,its mortality remains high.When AFLP is complicated with multi-organ dysfunction syndrome(MODS)and severe infection,especially hematogenous infection caused by multidrug-resistant pathogens,treatment becomes extremely difficult with an even lower survival rate.In the present work,we reported a 31-year-old primipara woman who developed AFLP complicated with MODS and severe infection at 37+5 weeks of gestation and received treatment in our hospital.The result of fast blood culture was positive for carbapenem-resistant Klebsiella pneumonia(CRKP).Based on this finding in combination with the results of drug sensitivity test,the quadruple regime using scheme imipenem cilastatin sodium,tigecycline,polymyxin and fosfomycin was implemented,and the patient was successfully cured.Successful treatment of pregnant women with AFLP complicated with MODS and/or hematogenous infection of CRKP can be achieved by using individualized combined antibiotic therapies on the basis of fast blood culture and combined drug sensitivity test.In this case,the patient was in a critical situation.In the treatment process,clinicians,clinical pharmacists and microbiologists should cooperate with each other and discuss the treatment plan together according to the pathophysiological characteristics of the patient,which was one of the key factors for successful treatment of the patient.

Acute fatty liver of pregnancy: diagnosis and management of 8 cases

Objective To investigate the early recognition and management of acute fatty liver of pregnancy (AFLP) to improve maternal and fetal survival. Methods Eight cases of AFLP seen in our hospital during the past three years were studied retrospectively. Symptoms, laboratory findings, timing of liver biopsy, and maternal and fetus outcome were assessed. Results The mean gestational age at onset was 34 ± 2 weeks. All cases were primigravida. In the early stages, all patients presented malaise, nausea, vomiting and epigastric distress followed by jaundice in the third trimester of pregnancy. Laboratory findings:all had raised transaminases and serum bilirubin (32 .5 - 510. 8 umol/L), hypoalbuminemia (22 .4 -30.0 g/L ), hypofibrinogenemia (< 180 mg/dl ),prolonged prothrombin time and prolonged partial thromboplastin time. Maternal complication included hepatic encephalopathy (6 cases ), ascites (6 ),hypoglycemia (5), hematemesis (2), and postpartum hemorrhage (5) and preeclampsia (4). Emergency cesarean section was performed in 3 cases. One mother died of fulminant hepatic failure and the others survived. There was no fetus death. Liver biopsy was done on the 5th to 15th postpartum day in 8 cases. Conclusion With increasing awareness, especially in the early recognition of milder cases, and prompt progressive management including early termination of pregnancy by cesarean section and large dose infusion of fresh frozen plasma and albumin alternately, the prognosis of AFLP can be improved.

CSOG MFM Committee Guideline:Clinical Management Guidelines for Acute Fatty Liver of Pregnancy in China(2021)

Acute fatty liver of pregnancy(AFLP)is a rare but critical obstetric-specific disease with a high fatality rate,posing a serious threat to the safety of mothers and infants.These guidelines were specially formulated to standardize AFLP clinical pathways and to improve maternal and infant outcomes.Based on a two-round questionnaire survey,the guideline development team identified the following nine clinical issues that clinicians were most concerned about,and developed recommendations for each of them:prenatal outpatient screening for AFLP,diagnosis,preoperative risk assessment,delivery modes and timing,anesthesia methods,perinatal complications,selecting AFLP patients for artificial liver treatment,prognostic assessment,and monitoring during treatment.The guidelines cover the key issues related to AFLP diagnosis and treatment that concern clinicians.

Imaging of pregnancy-related liver diseases

Pregnancy-related liver diseases are the most frequent cause of liver dysfunction in pregnancy and pose a real threat to fetal and maternal survival.Although hepatobiliary disorders are uncommon in pregnancy,they may be severe enough to cause risk to both the mother and fetus.There are five different types of liver disease that are unique to pregnancy,including hyperemesis gravidarum,intrahepatic cholestasis of pregnancy,preeclampsia,hemolysis,elevated liver enzyme,and low platelet count syndrome,and acute fatty liver of pregnancy.Ultrasound,computed tomography,and magnetic resonance imaging play important roles in the rapid diagnosis and evaluation of complications.An accurate diagnosis can guide further treatment or the induction of labor to prevent intrauterine fetal death.This review highlights the pathogenesis,clinical features,imaging features,and treatment of pregnancy-related liver diseases with the aim of enhancing awareness of these rare but potentially life-threatening diseases of pregnancy.