Acute Fatty Liver of Pregnancy: Experience of 8 Cases from a Tertiary Hospital

Swansea criteria score in acute fatty liver of pregnancy.

Risk of recurrent acute fatty liver of pregnancy: survey from a social media group

Acute fatty liver of pregnancy (AFLP) substantially contributes to maternal and neonatal morbidity and mortality. Other liver-associated pregnancy complications such as preeclampsia-associated HELLP (hemolysis, elevated liver enzyme, low platelet) syndrome may be difficult to differentiate from AFLP as these diseases overlap with regard to multiple clinical and laboratory features. The aim of this study was to investigate angiogenic profiles by measuring soluble fms-like tyrosine kinase-1 (sFlt-1) and placental growth factor (PlGF) in pregnancies compromised by AFLP and to compare them with those complicated by HELLP syndrome. Pregnant women affected by AFLP or HELLP syndrome were enrolled. The study population of women with HELLP syndrome was part of a larger data collection obtained in our clinic that has been used for previous work. Patients' angiogenic profiles were assessed by measuring sFlt-1 and PlGF serum levels. To assess the diagnostic potential of these angiogenic markers in AFLP, as well as discriminating it from HELLP syndrome, non-parametric tests were used and receiver operating curves were calculated. Six women with AFLP and 48 women with HELLP syndrome were included in the study. Patients with AFLP showed significantly higher sFlt-1 levels (median: 57570pg/mL; range 31609-147170pg/mL) than patients with HELLP syndrome (9713pg/mL; 1348-30781pg/mL; p<0.001). PlGF serum levels were higher in patients with AFLP compared with those with HELLP syndrome (197pg/mL; 127-487pg/mL vs. 40pg/mL; 9-644pg/mL, respectively; p<0.01). sFlt-1/PlGF ratios were not significantly different between AFLP and HELLP syndrome patients (192; 157-1159 vs. 232; 3-948, respectively; NS). In our study population, an sFlt-1 cut-off value of 31100pg/mL allowed differentiation between these two diseases with a sensitivity and specificity of 100%. A linear correlation was found between the cumulative numbers of Swansea criteria and sFlt-1 serum levels (r=0.97; p<0.01). AFLP is associated with very high sFlt-1 serum levels in particular in women fulfilling eight or more Swansea criteria. Besides the suggested Swansea criteria to diagnose AFLP, an sFlt-1 value above 31100pg/mL may be an additional biochemical feature improving discrimination between AFLP and HELLP syndrome. However, because of the small number of pregnancies affected by AFLP included in this work further studies are needed to corroborate our findings.

BackgroundCases of acute fatty liver of pregnancy are extremely rare but represent a serious complication as a life-threatening condition for both the mother and newborn in late-term pregnancy. Preoperatively, adequate management with multidisciplinary teams and timely delivery are potentially required to save materno-fetal life. Herein, we report a rare, inconspicuous case of acute fatty liver of pregnancy in third-trimester pregnancy and a review of the literature to underscore this hazardous entity.Case presentationA 28 year-old pregnant Vietnamese woman was transferred to our tertiary referral hospital for suspicion of acute fatty liver of pregnancy without prodromal signs, except for intermittent abdominal pain at 39 weeks 3 days of gestational age. In accordance with the Swansea criteria, laboratory findings contributed to confirming the diagnosis of acute fatty liver of pregnancy. Immediately, an emergent cesarean section was rapidly performed. Postoperatively, the patient recovered gradually with rapid improvement of biochemical and hematological parameters. The woman and her neonate were discharged uneventfully after 9 days of hospitalization.ConclusionsAcute fatty liver of pregnancy may be asymptomatic and can progress quickly, affecting multiple organs. Early diagnosis is based on abnormal presentation of serum laboratory tests. In addition, clinicians ought to assess the diagnosis following the Swansea criteria. Importantly, supportive therapy, strict monitoring, and early termination of pregnancy, when appropriate, are still the mandatory protocols for the management of acute fatty liver of pregnancy.

Objective To identify the incidence, clinical features and prognosis of acute fatty liver of pregnancy (AFLP). Methods The clinical data of AFLP from all hospitals in Shenzhen, China in the past 8 years were collected. The incidence, clinical characteristics and outcomes of AFLP were analyzed. Results There were 982 761 pregnant women from 2003 to 2010 and 44 of them were diagnosed as AFLP. The incidence of AFLP was 1/22 335. In this cohort of 44 cases with AFLP, 23 patients complicated by gestational hypertension disease, and the incidence was 52%. The mean (SD)maternal age was (27.7±5.7) and the gestational age at onset was 30+ 2-40+ 3 weeks. Thirty-three cases (75%) of them were primipara and 9 cases (20%) were multiple pregnancies. The first symptom was gastrointestinal symptoms 37 cases (84%), aggravated jaundice 40 cases (91%), coagulopathy 39 cases (89%), hepatorenal syndrome 25 cases (57%), hepatic encephalopathy 9 cases (20%), and multi-organ dysfunction syndrome 5 cases (11%). Thirty-nine cases were cured while 5 women died. The maternal mortality was 11%. The fetal outcomes showed 45 livinginfants, 9 fetuses death, and 1 neonatal death. The prenatal mortality was 18%. Conclusions The first symptom of AFLP was gastrointestinal symptoms in late pregnancy and the AFLP was associated with jaundice, high liver enzymes, coagulopathy, and early-onset acute renal failure. Early diagnosis and timely terminating pregnancy may improve the outcomes. Key words: Pregnancy; Fatty live; Incidence

Acute fatty liver and HELLP syndrome: two distinct pregnancy disorders

Objective Acute fatty liver of pregnancy(AFLP) is a rare but very urgent obstetric disease in clinical. It is a common cause of liver failure in pregnancy and often needs to be admitted to the department of critical care medicine because of the rapid development of acute hepatic dysfunction and severe acute renal dysfunction. The etiology and pathogenesis of this disorder is not very clear although there have been many studies on it before. Meanwhile, the relatively high mortality requires a better recognition in order to better guide clinical decision making. Our previous multicentre retrospective study on AFLP demonstrated that total bilirubin and serum creatinine were independent risk factors for perinatal maternal mortality. And we aim to further assess maternal outcomes and risk factors in AFLP patients treated without plasma exchange or renal replacement therapy based on previous data we collected. Methods Retrospective cohort study of 133 hospitalized patients with AFLP was collected from four Chinese tertiary hospitals during the period between January 2009 and April 2014. One hundred thirty three patients were divided into two subgroups containing patients treated without plasma exchange (PE) or renal replacement therapy (CRT) and patients treated with PE or/and CRT. Logistic regression was used to analyze independent risk factors for maternal mortality of AFLP treated without PE or CRT. Results The maternal mortality rate was 12.0% in subgroup of patients treated without PE or CRT. And in subgroup of patients treated with PE or/and CRT, the maternal mortality rate was 26.8%. Independent risk factors for maternal mortality of AFLP treated without PE or CRT were direct bilirubin (OR = 1.012; 95% CI, 1.002–1.022) and serum creatinine (OR = 1.022; 95% CI, 1.007–1.036). Conclusion Although less liver and kidney damage in AFLP treated without PE or CRT, direct bilirubin and serum creatinine remained to be independent risk factors for maternal mortality. Thus, the level of bilirubin and serum creatinine might not be necessary for AFLP to decide whether to give plasma exchange or dialysis treatment.

Objective To investigate the clinical features of acute fatty liver of pregnancy, and to improve the early diagnosis. Methods The clinical data of 13 patients of acute fatty liver of pregnancy from January 2014 to June 2015 were retrospectively analyzed. Clinical manifest, chemical examination and imaging examination were analyzed. Results The lategestation, upper digestive tract symptom, liver, renal and blood clotting function abnormity were common characteristics of all patients of acute fatty liver of pregnancy, but heightened blood ammonia, blood uric acid and leukocytes or decreased blood glucose and ultrasonic sound abnormity appeared only in proportion of these patients. Conclusions Lategestation, upper digestive tract symptom, liver, renal and blood clotting function abnormity are five capital standard of early diagnosis of acute fatty liver of pregnancy; by knowing this, we can diagnose it early and improve the outcome. Key words: Acute fatty liver of pregnancy; Early diagnosis; Capital standard

Acute fatty liver of pregnancy in 3 tertiary care centers

816: Hepatic and renal dysfunction with acute fatty liver of pregnancy: expected duration of recovery after delivery

A population-based study of acute fatty liver of pregnancy: incidence and maternal and neonatal outcomes

Background Liver disease is a rare complication of pregnancy that can lead to several consequences and require specific intervention with implications for both the mother and fetus. This study is aimed at assessing the clinical profile and associated complications of liver diseases unique to pregnancy at St. Paul's Hospital Millennium Medical College, Addis Ababa, Ethiopia. Methodology. This study is a retrospective cohort study of all identified cases admitted to the obstetrics ward and intensive care unit (ICU) from January 2018 to December 2020 at St. Paul's Hospital Millennium Medical College, Addis Ababa, Ethiopia. Medical records were reviewed for clinical features, biochemical profiles, and fetomaternal complications. Data were analyzed using SPSS version 26. A chi-square test was done to look for an association with a p value less than 0.05 considered statistically significant, and an odds ratio was determined to assess the effect size. Results From 95 cases identified, preeclampsia/eclampsia with liver dysfunction accounted for 43 (45%), followed by hemolysis elevated liver enzyme and low platelet (HELLP syndrome) 35 (36.8%), hyperemesis gravidarum with liver dysfunction 9 (9.5%), acute fatty liver of pregnancy (AFLP) 7 (7.4%), and intrahepatic cholestasis of pregnancy 1 (1.1%). When compared to HELLP syndrome, AFLP showed significantly higher median (IQR) values (p < 0.05) for total bilirubin 13.3 (7.3-16.3), direct bilirubin 9.73 (6.87-11.9) mg/dL, prothrombin time 23 (20.4-25.7) seconds, international normalization ratio 2.2 (1.9-2.4), white blood count 23.8 (17.8-26.6)∗103/μL, creatinine 3.5 (2.44-5.6) mg/dL, and lower hemoglobin level of 7.9 (6.2-10) g/dL. There were 4 (4.2%) maternal hospital deaths, with a case fatality rate of HELLP syndrome being 8.6% and 14.3% in AFLP. The overall hospital fetal mortality was 33 (34.7%). In this study, 42 patients with HELLP syndrome and AFLP had an increased risk of maternal ICU admission (OR = 25.5, 95% CI: 5.48-118.6, p value = 0.001), acute kidney injury requiring dialysis (OR = 12.2, 95% CI: 1.46-102.2, p value = 0.009), placental abruption (OR = 14.2, 95% CI: 1.72-117.1, p value = 0.004), and stillbirth (OR = 7.2, 95% CI: 2.38-21.7, p value = 0.001). Conclusion Preeclampsia with liver dysfunction and HELLP syndrome accounted for the majority of cases. It also demonstrated key biochemical characteristics that can be used to distinguish between HELLP syndrome and AFLP. Emphasis has to be given to the risk of requiring maternal ICU admission, dialysis, abruption of the placenta, and stillbirths while managing patients diagnosed with HELLP syndrome and AFLP.

Objective To explore the value of Swansea criteria on diagnosis and severity evaluation of acute fatty liver of pregnancy (AFLP).Methods Fifty-two AFLP patients were admitted to Shandong Provincial Hospital Affiliated to Shandong University between January 1,2000 and December 31,2011.All these cases were retrospectively reassessed by Swansea criteria.According to the severity,prognosis and whether continuous blood purification treatment was needed,these cases were classified as mild and severe cases.Differences between groups were detected by x2 or t test.Bivariate correlation analysis was used for Swansea criteria compliance and postnatal hemorrhage and days in hospital.Results After reassessing by Swansea criteria,31 cases could be diagnosed as AFLP (20 cases met seven or more criteria,11 cases met six criteria)and the other 21 cases could not (16 cases met five criteria,three cases met four,and two cases met three).For the 16 cases that met five Swansea criteria,they were confirmed as AFLP based on postnatal follow-up.The five cases that met four or three criteria were confirmed as AFLP because no other disease could explain their status.Among the patients who met seven or more Swansea criteria,the incidence of intrauterine fetal death was 40％ (8/20),and 65％ (13/20) needed continuous blood purification.These were higher than in patients who met six or fewer Swansea criteria [9％ (3/32) and 28％ (9/32),x2=6.921 and 6.857,P=0.014 and 0.011].Postpartum hemorrhage was positively correlated with Swansea criteria compliance (r2=0.286,P=0.040).Conclusion Patients who meet five Swansea criteria can be diagnosed as AFLP.Swansea criteria can be applied to the severity evaluation of AFLP. Key words: Pregnancy complications; Fatty liver; Severity of illness index

As proposed criteria (Swansea criteria) for the diagnosis of acute fatty liver of pregnancy (AFLP) do not include antithrombin (AT) activity, diagnosis of AFLP may be delayed. The aim of this review is to underscore problems in the differential diagnosis of AFLP and the syndrome of hemolysis, elevated liver enzymes and low platelet counts (HELLP syndrome) and to facilitate prompt diagnosis of AFLP. Published works dealing with liver dysfunction in pregnancy, HELLP syndrome and AFLP were reviewed. AFLP and HELLP syndrome shared common clinical, laboratory, histological and genetic features, and differential diagnosis between them was often difficult. However, HELLP syndrome was likely to occur in patients with hypertension, but AFLP occurred often in the absence of hypertension. In addition, AFLP was exclusively associated with pregnancy-induced antithrombin deficiency (PIATD). Approximately 50% of patients with AFLP did not have thrombocytopenia at presentation. As the Swansea criteria for AFLP did not include PIATD, diagnosis of AFLP was delayed until manifestation of life-threatening complications; 60% of women were admitted to intensive care and 15% to a specialist liver unit. In conclusion, incorporation of AT activity of less than 65% into the diagnostic criteria for AFLP may facilitate suspicion and prompt diagnosis of AFLP, decrease uncertainty regarding the diagnosis of AFLP, and contribute to better investigation and understanding of the process leading to the development of liver dysfunction.

Acute fatty liver of pregnancy is associated with significant maternal and fetal morbidity and mortality. Defects in fatty acid metabolism appear to play a role in pathogenesis, and several pathogenic variants leading to enzyme deficiencies have been associated with the development of acute fatty liver of pregnancy. The risk of recurrence in subsequent pregnancies is unknown. A review of 10 small case series of women with acute fatty liver of pregnancy disclosed only one case of recurrence in 35 subsequent pregnancies. This contrasted with a survey where 5 of 24 women reported recurrent acute fatty liver in subsequent pregnancies. Retrospective and prospective studies examining subsequent pregnancy outcomes following a diagnosis of acute fatty liver of pregnancy would be useful to better define risk and aid in preconception counselling of women who have had a diagnosis of acute fatty liver of pregnancy and wish to consider further pregnancies.