Edit concept Question Editor Create issue ticket

Acute Fatty Liver of Pregnancy

AFLP

Acute fatty liver of pregnancy is uncommon, but a possibly life-threatening disorder of pregnancy that primarily develops in the third trimester. Jaundice, fever, abdominal pain, anorexia, and nausea are some of the main complaints, whereas liver failure and coagulopathy can lead to both maternal and fetal death in the absence of proper therapy. Clinical findings, a thorough laboratory evaluation, and a liver biopsy are vital components of the diagnostic workup, while imaging studies are of limited benefit.


Presentation

Described as a rare entity in clinical practice (seen in 1 in 10,000-15,000 pregnancies) [1], acute fatty liver of pregnancy (AFLP) is a disorder of still incompletely understood etiology that is encountered in the third trimester of pregnancy or in early postpartum period [1] [2] [3] [4] [5]. Microvesicular steatosis (accumulation of fat) in the liver, but without any inflammation or necrosis, is the main pathophysiological event that is responsible for the development of symptoms and associated complications, but the mechanisms leading to its occurrence remain unclear [1] [6]. The clinical presentation is nonspecific - jaundice is the most common symptom, followed by fever, nausea, vomiting, anorexia, malaise, abdominal pain and tenderness in the upper right or middle epigastric area [1] [3] [7]. Additional findings include proteinuria, hypertension, and edema (which manifest as polyuria and polydipsia, and are highly suggestive of preeclampsia), whereas altered consciousness, seizures, respiratory insufficiency that requires assisted ventilation, as well as ascites, may develop rapidly without early therapy [2]. Furthermore, various complications of acute fatty liver of pregnancy have been described in the literature - liver failure, gastrointestinal bleeding (as a result of coagulopathies that arise from impaired liver function), renal failure, pancreatitis, hypoglycemia, disseminated intravascular coagulation (DIC) and hepatic encephalopathy [1] [3]. Despite the fact that the majority of women improve their general condition within the first month postpartum [3] [8], deaths from this condition have been reported, especially if preeclampsia or other comorbidities are concomitantly present [1].

Constitutional Symptom
  • The life-threatening risk of AFLP mandates the physician to include this condition in the differential diagnosis of nonspecific constitutional symptoms accompanied by jaundice.[symptoma.com]
Vomiting
  • 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.[ncbi.nlm.nih.gov]
  • Nausea and vomiting were the most common symptoms (75%). There were 2 maternal deaths (12.5%) and 3 fetal deaths (15%).[ncbi.nlm.nih.gov]
  • The most common presenting symptom was nausea/vomiting (88.5%), jaundice (71.4%), and abdominal pain (51.4%). Maternal morbidity included hypoglycemia (94.2%), renal failure (94.2%), coagulopathy (77.1%), ascites (48.5%), and encephalopathy (40%).[ncbi.nlm.nih.gov]
  • RESULTS: The initial symptoms varied considerably, with nausea and vomiting (13/56, 23%) being the most common.[ncbi.nlm.nih.gov]
  • The importance of vomiting in late pregnancy is emphasized. A common feature seems to be a metabolic stress, possibly related to infection, which tips a susceptible liver into metabolic failure.[ncbi.nlm.nih.gov]
Nausea
  • Nausea and vomiting were the most common symptoms (75%). There were 2 maternal deaths (12.5%) and 3 fetal deaths (15%).[ncbi.nlm.nih.gov]
  • The most common presenting symptom was nausea/vomiting (88.5%), jaundice (71.4%), and abdominal pain (51.4%). Maternal morbidity included hypoglycemia (94.2%), renal failure (94.2%), coagulopathy (77.1%), ascites (48.5%), and encephalopathy (40%).[ncbi.nlm.nih.gov]
  • Jaundice, fever, abdominal pain, anorexia, and nausea are some of the main complaints, whereas liver failure and coagulopathy can lead to both maternal and fetal death in the absence of proper therapy.[symptoma.com]
  • RESULTS: The initial symptoms varied considerably, with nausea and vomiting (13/56, 23%) being the most common.[ncbi.nlm.nih.gov]
  • Abstract We report the case of a previously healthy 32-year-old woman nearing the third trimester of pregnancy who presented to the emergency department (ED) with acute jaundice, nausea, and vomiting.[ncbi.nlm.nih.gov]
Abdominal Pain
  • Jaundice, fever, abdominal pain, anorexia, and nausea are some of the main complaints, whereas liver failure and coagulopathy can lead to both maternal and fetal death in the absence of proper therapy.[symptoma.com]
  • She went to an outside hospital in her third trimester of pregnancy with abdominal pain, jaundice, peripheral edema, and diffuse intravascular coagulation.[ncbi.nlm.nih.gov]
  • Clinicians must have a high index of suspicion for this condition when a woman has nausea or vomiting, abdominal pain (particularly epigastric), jaundice, polyuria-polydipsia (without diabetes), increased serum transaminase activity or thrombocytopenia[ncbi.nlm.nih.gov]
  • Presentation is classically with malaise, nausea and vomiting, abdominal pain and rarely encephalopathy. Prolongation of laboratory clotting tests is an early feature. Ultrasound examination of the liver is performed to exclude biliary stasis.[ncbi.nlm.nih.gov]
  • A 21-year-old parous woman presented with nausea, genital bleeding and abdominal pain at 33 weeks of gestation. Laboratory data revealed hepatic failure and disseminated intravascular coagulopathy.[ncbi.nlm.nih.gov]
Loss of Appetite
  • Case 3 developed loss of appetite and general fatigue with jaundice 10 days before delivery. They underwent termination of pregnancy after diagnosis was made.[ncbi.nlm.nih.gov]
  • The most frequent symptoms of AFLP include: nausea or vomiting abdominal or epigastric pain excessive fatigue and sleepiness loss of appetite jaundice (in advanced cases) The development of epigastric pain (pain in the upper right area of the abdomen)[preeclampsia.org]
  • The symptoms include nausea, vomiting, loss of appetite, weakness, pain in the belly, ascites (as a consequence of portal hypertension), epigastric burning (as an evidence of esophagitis that usually occurs on the background of DIC syndrome) and a developing[infobaby.org]
  • Let your doctor know if you’ve been experiencing fatigue or loss of appetite. Also, tell your doctor about any history of alcohol, medication, and supplement use.[healthline.com]
Jaundice
  • A review of this disorder and other medical emergencies causing jaundice in pregnancy is presented.[ncbi.nlm.nih.gov]
  • The life-threatening risk of AFLP mandates the physician to include this condition in the differential diagnosis of nonspecific constitutional symptoms accompanied by jaundice.[symptoma.com]
  • The most common presenting symptom was nausea/vomiting (88.5%), jaundice (71.4%), and abdominal pain (51.4%). Maternal morbidity included hypoglycemia (94.2%), renal failure (94.2%), coagulopathy (77.1%), ascites (48.5%), and encephalopathy (40%).[ncbi.nlm.nih.gov]
  • Here we describe a patient presenting with stupor and jaundice after aspirin intake at 35 weeks of gestation. Supportive management and delivery resulted in uneventful discharge of the patient and the newborn.[ncbi.nlm.nih.gov]
  • Abstract Acute fatty liver of pregnancy is the least common and most serious cause of jaundice of pregnancy. It is rare, fewer than 100 cases having been reported (Koff, 1981).[ncbi.nlm.nih.gov]
Hepatomegaly
  • USG abdomen revealed hepatomegaly with fatty changes grade 2-3, medical renal disease, and minimal ascites. Vaginal packing removed after 24 hours and oozing was still present. Repacking was done.[najms.org]
  • Respiratory, cardiovascular and nervous system were normal.Ultrasonography revealed hepatomegaly with hypoechoic liver parenchyma and gallbladder wall edema. Growth of both fetuses corresponded to 30-32 weeks of gestation.[casereports.in]
  • Hepatomegaly and steatosis in HIV-infected patients receiving nucleoside analogue antiretroviral therapy.[doi.org]
Asterixis
  • Symptoms may rapidly progress from restlessness, confusion, and disorientation to asterixis, seizures, psychosis, and ultimately coma. [6] Other systemic effects include respiratory failure, sometimes requiring assisted ventilation, ascites, [4] and gastrointestinal[najms.org]
Pruritus
  • During the 26th week of a first pregnancy, a 25-year-old woman presented with pruritus suggesting an intrahepatic cholestasis of pregnancy. The pruritus, however, persisted despite the premature delivery of a normal newborn at the 35th week.[ncbi.nlm.nih.gov]
  • Polydipsia (in nine episodes) and skin pruritus (in seven episodes) were unusual clinical findings.[ncbi.nlm.nih.gov]
Skin Lesion
  • Early recognition is critical, as skin lesions may precede the development of pancreatitis. Often, as in our case, the effects of pancreatitis may be life threatening.[ncbi.nlm.nih.gov]

Workup

The life-threatening risk of AFLP mandates the physician to include this condition in the differential diagnosis of nonspecific constitutional symptoms accompanied by jaundice. But because this condition is strikingly similar to viral hepatitis, preeclampsia, cholestasis, HELLP syndrome (hemolysis, elevated liver enzymes, low platelet count) and several other hepatic disorders, a thorough investigation is necessary. Some authors have illustrated the important role of a properly obtained patient history and a detailed physical examination that can rule out some of the mentioned diseases based on clinical findings (For example, jaundice is rarely seen in preeclampsia, and cholestasis is not associated with nausea and vomiting) [1]. Laboratory findings, however, provide more definite diagnostic clues. Elevated liver transaminase levels (alanine and aspartate aminotransferases, or ALT and AST, respectively), hypoglycemia, leukocytosis, hyperbilirubinemia, hypoglycemia, increased prothrombin time (PT) and/or partial thromboplastin time (aPTT), increased values of creatinine, and hyperammonemia are some of the main laboratory criteria of acute fatty liver of pregnancy [1] [2]. Imaging studies, such as computed tomography (CT) and abdominal ultrasonography, are of limited use in the diagnosis of AFLP, which is why a liver biopsy is considered to be the gold standard, but it can only be performed in the setting of hemodynamic stability [1] [2] [3] [7].

Liver Biopsy
  • 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.[ncbi.nlm.nih.gov]
  • Liver biopsy was done on the 5th to 15th postpartum day in 8 cases.[ncbi.nlm.nih.gov]
  • Acute fatty liver of pregnancy was documented by a liver biopsy specimen stained with oil red O and observed under electron microscopy.[ncbi.nlm.nih.gov]
  • Diagnosis was established by liver biopsy in both cases. The unusual good outcome for both women can be attributed to early recognition and prompt delivery, once diagnosis was considered.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]

Treatment

  • Treatment efficacy was based primarily on survival and secondarily on liver and kidney functions 2 weeks after treatment. Adverse effects were also assessed at the same time point.[ncbi.nlm.nih.gov]
  • The authors present the clinical course, dynamics, diagnostics, and outcome of treatment in this unique case. In this patient the ultimate treatment chosen was the liver transplantation.[ncbi.nlm.nih.gov]
  • RESULTS: (1) Adverse reactions during PE were generally mild and tolerable, and no patients discontinued treatment due to adverse events; symptoms, physical signs, and liver and kidney functions improved significantly after PE (P CONCLUSION: Treatment[ncbi.nlm.nih.gov]
  • The CBPT was implemented based on the timely termination of pregnancy and general treatment.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]

Prognosis

  • The obstetric team must be familiar with this disease because early diagnosis and prompt delivery have dramatically improved prognosis, which was often fatal for both mother and child.[ncbi.nlm.nih.gov]
  • AIM: To evaluate the prognosis of patients with acute fatty liver of pregnancy (AFLP) 6 mo or longer after discharge.[ncbi.nlm.nih.gov]
  • CONCLUSION: Early diagnosis, prompt delivery and intensive supportive treatment are critical for improving the prognosis of AFLP.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • 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[ncbi.nlm.nih.gov]

Etiology

  • Abstract Acute fatty liver of pregnancy (AFLP) is a rare, but serious life-threatening clinical entity, the etiology of which is unknown. The clinical presentation of this disease is varied and timely diagnosis often difficult.[ncbi.nlm.nih.gov]
  • The etiology of fetal mortality is not well delineated. Reported is a patient with histologically proved acute fatty liver who developed fetal distress during therapy for coagulopathy.[ncbi.nlm.nih.gov]
  • Markedly elevated transaminases prompted a search for other etiologies, and acetaminophen toxicity was diagnosed. Liver biopsy revealed acute fatty liver of pregnancy and toxin-induced injury consistent with acetaminophen use.[ncbi.nlm.nih.gov]
  • The biochemical disorder evidenced in this family is discussed, in an attempt to shed light on the etiology of acute fatty liver of pregnancy.[ncbi.nlm.nih.gov]
  • Clinical and istopathologic features, possible etiologic factors and possible treatment for the mother and fetus are discussed.[ncbi.nlm.nih.gov]

Epidemiology

  • Several lines of evidence suggest that blockade of fatty-acid oxidation may also be the underlying cause of Reye's syndrome, and epidemiological studies have identified aspirin taken during a viral illness as a contributing factor to the development of[ncbi.nlm.nih.gov]
  • This study confirms an improvement in maternal prognosis in AFLP, discusses the possibility of an epidemiological association with intrahepatic cholestasis of pregnancy, and increases the number of patients reported with recurrent AFLP.[ncbi.nlm.nih.gov]
  • This review examines recent literature on the epidemiology, pathogenesis, diagnosis, and treatment of acute fatty liver of pregnancy.[readbyqxmd.com]
  • Author information 1 National Perinatal Epidemiology Unit, University of Oxford, UK. marian.knight@npeu.ox.ac.uk Abstract OBJECTIVES: To identify a national, population-based cohort of women with acute fatty liver of pregnancy (AFLP), to evaluate proposed[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • As these triglyceride-rich particles cause endothelial dysfunction, they may contribute to the pathophysiology of AFLP.[ncbi.nlm.nih.gov]
  • The concurrence of this disorder with toxemia of pregnancy suggests that these entities may be pathophysiologically related.[ncbi.nlm.nih.gov]
  • The concurrence of this disorder with toxemia of pregnancy suggests that these entities may be pathophysiologically related. FULL TEXT[doi.org]
  • The following case study reviews the clinical pathophysiology of this perinatal health problem and highlights the priorities for the care of infants born to mothers with acute fatty liver disease of pregnancy.[doi.org]

Prevention

  • Vitamin K1 is frequently used in the treatment and prevention of hypoprothrombinemia and hemorrhagic disease of the newborn. It also serves as an antidote to anticoagulants.[ncbi.nlm.nih.gov]
  • Therefore an early diagnosis and delivery, followed by close monitoring and optimized management of the impaired liver function with all associated problems are necessary to prevent maternal and foetal death.[ncbi.nlm.nih.gov]
  • It is suggested that intrahepatic cholestasis of pregnancy caused premature delivery, which in turn may have prevented the onset of severe maternal and fetal complications caused by acute fatty liver of pregnancy.[ncbi.nlm.nih.gov]
  • This obstetrical emergency can lead to death of both mother and child if not diagnosed in time to prevent coagulopathic complications.[ncbi.nlm.nih.gov]
  • PPEX utilization in patients with severe AFLP may enhance maternal recovery by preventing secondary sequelae from hepatic insufficiency until spontaneous healing can occur.[ncbi.nlm.nih.gov]

References

Article

  1. Ko HH, Yoshida E. Acute fatty liver of pregnancy. Can J Gastroenterol. 2006;20(1):25-30.
  2. Dey M, Reema K. Acute Fatty Liver of Pregnancy. N Am J Med Sci. 2012;4(11):611-612.
  3. Ibdah JA. Acute fatty liver of pregnancy: An update on pathogenesis and clinical implications. World J Gastroenterol. 2006;12(46):7397-7404.
  4. Zhang Y-P, Kong W-Q, Zhou S-P, Gong Y-H, Zhou R. Acute Fatty Liver of Pregnancy: A Retrospective Analysis of 56 Cases. Chin Med J (Engl). 2016;129(10):1208-1214.
  5. Xiong H-F, Liu J-Y, Guo L-M, Li X-W. Acute fatty liver of pregnancy: Over six months follow-up study of twenty-five patients. World J Gastroenterol. 2015;21(6):1927-1931.
  6. Natarajan SK, Thangaraj KR, Goel A, Eapen CE, Balasubramanian KA, Ramachandran A. Acute fatty liver of pregnancy: an update on mechanisms. Obstet Med. 2011;4(3):99-103.
  7. Dey M, Kumar R, Narula GK, Vadhera A. Acute fatty liver of pregnancy. Med J Armed Forces India. 2014;70(4):392-393.
  8. Reyes H, Sandoval L, Wainstein A, et al. Acute fatty liver of pregnancy: a clinical study of 12 episodes in 11 patients. Gut. 1994;35(1):101-106.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2019-07-11 20:12