Diagnosis Of Acute Fatty Liver Research Articles

Missing or making the timely diagnosis of acute fatty liver of pregnancy (AFLP): lessons learned

OBJECTIVE To review the presenting signs, symptoms, laboratory findings, clinical courses, complications, and maternal outcomes in women determined on medicolegal review to have had acute fatty liver of pregnancy (AFLP). METHODS We retrospectively studied case files from women with medical and/or hypertensive complications of pregnancy which had been reviewed by the first author, deidentified and divested of legal documents so that only medical-related information was retained for later research and teaching purposes. Main outcomes are 12 lessons learned from this review to be offered for the future benefit of clinicians encountering patients with AFLP. RESULTS Eleven women with AFLP were identified within the 161 file database. Signs and symptoms present in 91–100% of women with AFLP were altered sensorium, a fast resting maternal pulse >100/min, gastrointestinal complaints, muscle pain and evidence of fetal compromise/stillbirth. Laboratory findings in 91–100% revealed abnormal liver, renal and coagulation tests, hyperuricemia, and very low serum glucose and albumin. A correct initial diagnosis of AFLP was made for only three women; the most common incorrect presumptive diagnosis was HELLP syndrome. Four women survived. Twelve major findings are discussed. CONCLUSION Rapid, timely and thorough evaluation of third trimester women presenting with A (Altered mental status changes/extreme sleepiness), F (Fast resting pulse >100 in the absence of fever or heart causation), L (Liver-related gastrointestinal complaints) and P (Pain as myalgias and body aches), often in association with evidence of fetal compromise or stillbirth, should initiate efforts to investigate a possible diagnosis of AFLP. Adoption of the concepts summarized including core initial laboratory testing, immediate patient transfer to tertiary care, and aggressive correction of consumptive coagulopathy could form the basis of a patient safety bundle to guide future management for patients with AFLP.

Acute Fatty Liver of Pregnancy: Rare, but Potentially Fatal

Patient: Female, 34-year-oldFinal Diagnosis: Acute fatty liver of pregnancySymptoms: Jaundice • nausea • prostration • vomitingMedication: —Clinical Procedure: —Specialty: Obstetrics and GynecologyObjective:Rare diseaseBackground:Acute fatty liver of pregnancy is an obstetric emergency characterized by liver dysfunction, which can lead to severe maternal and fetal complications.Case Report:A 34-year-old woman, 37 weeks and 2 days pregnant, reported symptoms of nausea, vomiting, jaundice, and prostration. Laboratory findings revealed liver dysfunction and coagulopathy. A clinical diagnosis of acute fatty liver was made and an emergency cesarean section was performed. The postoperative period was complicated by disseminated intravascular coagulation, acute hepatic and renal insufficiency, and pancreatitis.Conclusions:Early recognition of this pathology, the interruption of pregnancy, and intensive therapy led to a favorable outcome.

Acute fatty liver of pregnancy: a case report

BackgroundAcute Fatty Liver of Pregnancy (AFLP) is a rare, catastrophic disease affecting women in the third trimester of pregnancy or in the post-partum period. It is usually a diagnosis of exclusion and requires a strong index of suspicion for a timely diagnosis and prompt intervention.Case presentationWe report a case of AM, an 18 year patient, in her first pregnancy at 35 weeks gestation who presented with nausea, vomiting and jaundice. She had a vaginal delivery following spontaneous preterm labour. A clinical diagnosis of acute fatty liver of pregnancy was made on the 3rd day post-delivery. The post-delivery course was complicated by a deterioration of clinical symptoms with worsening hepatorenal function and development of encephalopathy. The patient died 3 days after admission and the diagnosis was confirmed on post-mortem and histology.ConclusionDelay in the diagnosis is associated with morbid complications with high mortality and this case highlights the importance of a high index of suspicion of the condition in women presenting with jaundice in pregnancy.

Physiological changes of pregnancy and the Swansea criteria in diagnosing acute fatty liver of pregnancy

The Swansea criteria are used to assess the likelihood of acute fatty liver of pregnancy. There are significant physiological changes in normal pregnancy in several of the pathology parameters used in the Swansea criteria. This may impact the sensitivity and specificity of the Swansea criteria. Five of the 11 case series reporting laboratory values in acute fatty liver of pregnancy used values divergent from the Swansea criteria. When using the Swansea criteria for diagnosis of acute fatty liver of pregnancy, using pregnancy-specific and/or laboratory-specific reference intervals is recommended. Simpler diagnostic criteria using parameters of hepatocellular damage and hepatic synthetic dysfunction may be an alternative to the Swansea criteria, and further studies investigating the sensitivity and specificity of these parameters would be useful.

Retrospective analysis of anesthetic and perioperative management in patients of acute fatty liver of pregnancy

Objective: To retrospectively analyze the clinical characteristics of patients with acute fatty liver of pregnancy (AFLP), and to discuss perioperative and anesthetic management. Methods: A retrospective review was conducted on the records of pregnant patients with a diagnosis of acute fatty liver of pregnancy in Peking University Third Hospital from January 2007 to December 2015. 12 cases were identified. The clinical features, preoperative laboratory findings, types of delivery, anesthetic techniques for cesarean section, and the outcomes of parturients and fetus were collected and analyzed. Results: Among the 12 cases, 91.7% were primigravid, 50% had twin pregnancies, and one was diagnosed with concomitant preeclampsia. The common clinical features included nausea (6 cases, 54.5%), vomiting (5 cases, 45.5%), jaundice (5 cases, 45.5%), malaise (3 cases, 27.3%), epigastric discomfort (2 cases, 18.2%), anorexia (2 cases, 18.2%) and regurgitation (1 case, 9.1%). Laboratory tests mainly showed impaired hepatic function and hypoglycemia. Cesarean deliveries were performed in 10 of the 11 patients diagnosed antepartum. Cesarean section was performed under neuraxial anesthesia (1 case) or general anesthesia (9 cases). The patient transferred to our center after delivery and diagnosed postpartum died. All the patients diagnosed antepartum survived. 6 out of the 18 fetuses were transferred to the pediatric department due to preterm, low birth weight, intrauterine restriction or asphyxiation, and were all survived. Conclusions: AFLP is one of the most severe complications in parturients. Prognosis can be improved with early diagnosis and prompt termination of pregnancy. Hepatic function, coagulation status and urgency of delivery should be well considered to choose the appropriate anesthetic method, and anesthetic management should be individualized.

Acute Fatty Liver of Pregnancy Complicated by Quadruplets

Poster Presentation Background The diagnosis of acute fatty liver of pregnancy (AFLP) prior to surgical incision is critical. Case A 26‐year‐old G2P1 woman pregnant with quadruplets at 32 weeks gestation presented to triage with general malaise, pain in the right thigh, and feeling unwell. The woman had normal range blood pressures and no complaint of visual changes, epigastric pain, or jaundiced appearance. She was admitted for observation to rule out deep vein thrombosis (DVT). Routine labs were ordered with a comprehensive metabolic panel that indicated high enzymes, low platelets, and low hemoglobin and hematocrit (H&H). The decision to proceed with delivery was made due to diagnosis of HELLP syndrome. Clotting studies and a disseminated intravascular coagulation (DIC) panel were ordered and drawn at this time. The woman was sent to the operating room (OR) for delivery and prepped for an epidural. The diagnosis of AFLP was made in the OR. The procedure was stopped prior to epidural placement, and the woman was moved back to the obstetric intensive care unit for correction of coagulopathy. Consultation was initiated with transfusion medicine and transfusion of 12 units apheresis platelets, two units of packed red blood cells (PRBC), 16 pooled cryoprecipitated anti‐hemophilic factor (cyro), two jumbo fresh frozen plasma (FFP) units, and six liquid plasma units was ordered. We increased fibrinogen from 60 to 347 during the course of 6 hours, and the decision was made to move the woman to the OR and proceed with the cesarean with an intraoperative transfusion of three apheresis platelets, two units of PRBCs, six pooled cyros, and two jumbo FFP units. The delivery of the quadruplets and delivery of placenta were successful with good hemostasis. The woman experienced a total estimated blood loss of 1400 ml. After birth, the quadruplets were transported to the NICU, and the women was ventilated and placed in the obstetric intensive care unit. Careful evaluation for renal failure, hepatic encephalopathy, and/or other sequelae of AFLP was ongoing. The woman was extubated 24 hours postoperatively with stable renal function and urine output. Conclusion On postoperative day 2, the woman began to experience agitation, deterioration in mental status, and decreased ability to communicate. Fibrinogen decreased from 327 to 225, international normalized ratio (INR) increased from 2.2 to 2.5, ammonia increased from 33 to 51, and creatinine was 2.60. A care team including renal specialists, critical care providers, maternal/fetal medicine and obstetric intensive care unit nurses agreed to transfer the woman to a long‐term acute care facility with an in‐house live transplant team for ongoing support and care. The diagnosis of AFLP prior to surgical incision proved critical to this woman's survival. The multidisciplinary care team worked together to provide optimal care and prevented what could have been a dire outcome.

Differentiation of acute fatty liver of pregnancy from syndrome of hemolysis, elevated liver enzymes and low platelet counts

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 Complicated by Pancreatitis and Multiorgan Failure

Purpose: Introduction: Acute fatty liver is an uncommon but serious complication of pregnancy. It usually occurs in third trimester and was universally fatal until the advent of advanced supportive measures. Case: A 29 year old woman was transferred to our tertiary care center with multiorgan failure. She was found to be have liver failure, renal failure and coagulopathy when she first presented to a city hospital with nausea and vomiting in 32nd week of her first pregnancy. Vaginal ultrasonography revealed an intrauterine dead fetus which was evacuated by cesarean section. Upon arrival to our center, she had developed acute pancreatitis, disseminated intravascular coagulation, intraabdominal bleeding with hemorrhagic shock and hypoglycemia in addition to hepatic and renal failure. She was given aggressive supportive treatment and blood products replacement. Transjugular liver biopsy confirmed the diagnosis of acute fatty liver of pregnancy (AFLP). The patient went on to develop acute respiratory failure and sepsis and ultimately succumbed to multiorgan failure. Discussion: Liver dysfunction in pregnancy can range from benign cholestasis to severe preeclampsia, HELLP (Hemolysis, Elevated Liver enzymes, Low Platelets) syndrome and AFLP. AFLP usually begins in late third trimester with nausea, vomiting, anorexia and epigastric pain and can progress to jaundice, coagulopathy and hepatic encephalopathy. Key laboratory findings are hyperbilirubinemia with modest elevation in transaminases, leukocytosis, coagulopathy and hypoglycemia. It can be complicated by disseminated intravascular coagulation, sepsis, renal failure and, rarely, pancreatitis. It is challenging to differentiate AFLP from HELLP syndrome due to overlapping features and liver biopsy showing microvesicular steatosis is usually necessary to make the diagnosis of AFLP. Emergent delivery and aggressive supportive care is the key to successful outcomes. Resolution occurs spontaneously after termination of pregnancy. Conclusion: AFLP is a serious condition which usually presents with non specific symptoms. Prompt recognition, termination of pregnancy and advanced supportive care are critical determinants of disease outcome. Prognosis is guarded when it is complicated by multiorgan failure.

Acute Fatty Liver of Pregnancy in a Taiwanese Tertiary Care Center: A Retrospective Review

To evaluate the demographics, clinical presentations, laboratory findings, and maternal and fetal outcomes in patients with acute fatty liver of pregnancy. A retrospective review was conducted of the records of pregnant patients with a diagnosis of acute fatty liver in a tertiary medical center over a 22-year period. Eighteen patients with acute fatty liver of pregnancy were recruited, all of whom developed the disease in the third trimester. Eleven women (61%) were primigravid and four (22%) had twin pregnancies; six (33%) were diagnosed antepartum, and the other 12 (67%) were diagnosed postpartum. There were two maternal deaths (11%) and four fetal deaths (18%). The most common complications apart from severe liver dysfunction were acute renal failure (83%), hypoglycemia (61%), and disseminated intravascular coagulation (61%). Women who become acutely ill during the third trimester of pregnancy should undergo tests for acute fatty liver of pregnancy, including laboratory tests for assessing liver function and coagulation profile.

Acute fatty liver of pregnancy: A case report of an uncommon disease

A 24-year-old female at 34-week gestation, presented with malaise, nausea, vomiting, jaundice, and absent foetal movements. A clinical diagnosis of acute fatty liver of pregnancy was made. Although early caesarean section was performed, postoperative course was complicated by acute respiratory distress syndrome (ARDS) sepsis, and continuing coagulopathy. Supportive management in an intensive care unit resulted in successful outcome.

Acute fatty liver of pregnancy in 3 tertiary care centers

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. 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. 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%). 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.

Reversible peripartum liver failure: A new perspective on the diagnosis, treatment, and cause of acute fatty liver of pregnancy, based on 28 consecutive cases

Objective: We sought to describe our experience with the clinical diagnosis, management, and course of patients with acute fatty liver of pregnancy. Study Design: Twenty-eight cases of acute fatty liver of pregnancy at the Los Angeles County and University of Southern California Medical Center from 1982 to June 1997 were identified, and presenting symptoms, clinical course, laboratory values, maternal complications, and neonatal outcomes were studied. Results: The incidence of acute fatty liver of pregnancy was 1 in 6659 births. There were no maternal deaths. Initial presentation was at an average of 37 weeks of gestation with a characteristic prodrome of malaise, nausea, vomiting, and abdominal pain. No patient was admitted with the diagnosis of acute fatty liver of pregnancy. The condition was diagnosed most commonly on the second hospital day after laboratory results indicated coagulopathy, renal insufficiency, and liver function abnormalities. One patient underwent liver biopsy at cesarean delivery. Radiologic studies did not aid with the diagnosis. Twenty-one patients were admitted in spontaneous labor, and 16 labors were complicated by abnormal fetal heart rate patterns or meconium. There was 1 stillbirth and 1 neonatal death as a result of perinatal asphyxia. Maternal morbidity consisted of hypoglycemia, infection, renal insufficiency, coagulopathy, encephalopathy, and wound complications. All patients had evidence of disseminated intravascular coagulopathy with profoundly decreased antithrombin levels. All patients recovered normal liver function post partum. Conclusions: Reversible peripartum liver failure may be diagnosed and managed on the basis of clinical and laboratory criteria. With adequate support, these patients may have full recovery of hepatic function. (Am J Obstet Gynecol 1999;181:389-95.)

Acute Fatty Liver of Pregnancy: A Review of Maternal Morbidity in 13 Patients Seen Over 12 Years in Nova Scotia

OBJECTIVE: To review the maternal and fetal survival in all cases of acute fatty liver of pregnancy seen by the Division of Gastroenterology at Grace Maternity Hospital and the Victoria General Hospital from 1979-91.DESIGN: A retrospective review of the clinical data obtained from the medical charts of 13 patients with a liver biopsy-based histopathological diagnosis of acute fatty liver of pregnancy.SETTING: Grace Maternity Hospital, a tertiary care centre serving d1e Atlantic provinces. Twelve patients were subsequently transferred to Victoria General Hospital for postpartum management in the setting of the medical intensive care unit.MAIN OUTCOME MEASURES: Classically, acute fatty liver of pregnancy is complicated by over 70% maternal and fetal mortality rate. Recent reports have indicated significantly improved maternal and fetal survival because of more awareness, improved management and the identification of milder forms of the disease.RESULTS: In this study of 13 cases of acute fatty liver of pregnancy, maternal survival was 100% and fetal survival was 93%.CONCLUSION: The excellent maternal and fetal survival in this series is attributed co awareness, close collaboration between obstetricians and gastroenterologists, prompt diagnosis and delivery and the management of postpartum patients in an intensive care unit setting.

Magnetic Resonance Imaging and Computed Tomography Scan for the Diagnosis of Acute Fatty Liver of Pregnancy

Magnetic resonance imaging (MRI) and computed tomography were used to diagnose fatty liver of pregnancy in a patient with preeclampsia and thrombotic thrombocytopenia purpura. The clinical course included multiple system failure that necessitated mechanical ventilation, dialysis, and plasmapheresis. The MRI picture is described in this entity for the first time. Both imaging techniques are discussed and compared.

Fulminant hepatic failure caused by acute fatty liver of pregnancy treated by orthotopic liver transplantation

A previously healthy 35-year-old woman was seen at 37 weeks' gestation with a 10-day history of fever, vomiting, diarrhea and malaise. Serum laboratory findings included elevation of serum bilirubin and AST, prolongation of serum prothrombin time and a positive monospot. A tentative diagnosis of acute fatty liver of pregnancy was made, and a healthy male infant was delivered by emergency cesarean section because of fetal distress. Over the subsequent 3 days, acute progressive oliguric renal failure, disseminated intravascular coagulation, hypoglycemia requiring intravenous dextrose infusion and pancreatitis developed; her mental status progressed to stage III encephalopathy. Quantitative computed tomography estimated the liver volume to be 770 cm3. The decision to proceed with orthotopic liver transplantation was made on the basis of progressive clinical deterioration despite aggressive support and because of her small liver size. After transplant, the patient's multisystem failure rapidly reversed. Histopathological examination of the native liver demonstrated predominantly zone 3 microvesicular steatosis with characteristic ultrastructural changes consistent with acute fatty liver of pregnancy. Southern blot analysis for Epstein-Barr virus DNA was negative. We conclude that orthotopic liver transplantation should be considered for the small group of patients with fulminant hepatic failure associated with acute fatty liver of pregnancy who manifest signs of irreversible liver failure despite delivery of the fetus and aggresive supportive care.

Jaundice in pregnancy

The occurrence of hepatobiliary disease with or without jaundice during pregnancy provides both the hepatologist and obstetrician with an interesting and urgent diagnostic challenge. Advances in our understanding and management of liver disorders unique to pregnancy and hepatobiliary disease in general have resulted in a significant improvement in the outcome for both mother and fetus. Certain disorders such as acute fatty liver of pregnancy and hepatic haemorrhage associated with toxaemia should be considered medical emergencies and delay in diagnosis of these conditions will probably adversely affect maternal and fetal outcome. A careful clinical history, physical examination, appropriate laboratory tests and radiological investigations should allow a diagnosis within 24–48 hours of presentation. Liver biopsy is rarely required. A careful history may provide important information. Does the patient have pre-existent liver disease? Has there been contact with hepatitis, intravenous drug abuse or any other factor predisposing to acute viral hepatitis? Does the patient have a family history of pruritus and/or jaundice to suggest intrahepatic cholestasis of pregnancy? Is the patient's-alcohol consumption excessive? Has the patient received any hepatotoxic medications? Has there been abdominal pain and/or fever to suggest gallstones, hepatic bleeding or acute fatty liver of pregnancy? Laboratory investigations may give valuable diagnostic clues. Marked aminotransferase elevation would suggest acute viral or ‘ischaemic’ hepatitis. Haematological features of microangiopathic haemolysis would point towards toxaemia or AFLP. Hepatitis A and B serological tests may be helpful in viral liver disease. Radiological investigations may be indicated depending on the clinical context. Abdominal ultrasonography may be useful in the diagnosis of gallstones, biliary obstruction, liver tumours or intrahepatic bleeding. Fatty infiltration of the liver may be diagnosed by ultrasonography but computed tomography (CT) of the abdomen is probably more reliable for a diagnosis of acute fatty liver of pregnancy as it allows measurement of liver density which is typically reduced by fatty infiltration. CT scanning is also probably more valuable than ultrasound in assessing the extent of capsular rupture and haemorrhage into the liver and peritoneal cavity.

Computed tomography in acute fatty liver of pregnancy

A case of the diagnosis of acute fatty liver of pregnancy proved by biopsy is described. In this case computed tomography showed decreased attenuation over the liver, and this attenuation is compatible with fatty infiltration. Computed tomography may be useful in the differential diagnosis of jaundice in pregnancy.

Acute fatty liver of pregnancy and diagnosis by computed tomography.

A 39 year old woman was admitted to a maternity unit at 34 weeks' gestation with nausea, vomiting, and jaundice. Her condition deteriorated, and she was transferred to hospital, deeply unconscious and hypotensive. The diagnosis of acute fatty liver of pregnancy was initially suggested by the typical history of prodromal malaise and vomiting and the rapid onset of hepatic encephalopathy with profound hypoglycaemia and only small increases in transaminase activities. Computed tomography was performed: there was no enlargement of the liver or spleen, but the attenuation value over the liver indicated appreciable fatty infiltration of the liver, establishing the diagnosis of acute fatty liver of pregnancy. Computed tomography is of value in the diagnosis of liver disease of late pregnancy, and this technique may become the method of choice for the investigation of acute fatty liver of pregnancy.

Acute Fatty Liver of Pregnancy

A 17-year-old-female at 39 weeks gestation, presented with pelvic pain, proteinuria and a decrease in fetal heart rate. Timely caesarean section was performed. The postoperative course was complicated by acute renal failure, acute pancreatitis, acute liver failure, anemia, thrombocytopenia, systemic inflammatory response syndrome (SIRS), prolonged coagulopathy, hyperbilirubinemia, ecclampsia, and psychosis. The clinical features and laboratory abnormalities, in conjunction with the timing of gestational age, led to the diagnosis of acute fatty liver of pregnancy. Prompt diagnosis and supportive care in an intensive care unit provided for a positive outcome.