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].

• Fatigue

  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]

  These enter the maternal circulation and are hepatotoxic long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency is a cause but not the only cause Clinical features vague abdominal pain, fatigue, nausea and vomiting may present with slowly [aic.cuhk.edu.hk]

  Symptoms Common symptoms are: Fatigue Nausea Vomiting Headache and abdominal pain in the epigastrium, which can progress to jaundice and liver failure with hepatic encephalopathy, renal dysfunction, and coagulopathy. [hellodoktor.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]

  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. [symptoma.com]

• Nausea

  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]

  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]

• 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]

• 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]

• Abdominal Distension

  She became tachypneic and tachycardiac with increased abdominal distension and decreased urine output. She landed in Disseminated Intravascular Coagulation. The drain output increased within first 24 hour of LSCS (2500 ml). [omicsonline.org]

• Jaundice

  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]

  A review of this disorder and other medical emergencies causing jaundice in pregnancy is presented. [ncbi.nlm.nih.gov]

  Diagnosing the etiology of jaundice is extremely important in pregnant patients as certain conditions like AFLP, HELLP syndrome and intra-hepatic cholestasis of pregnancy (ICP) may require early termination of pregnancy even in the presence of jaundice [omicsonline.org]

• 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. [gut.bmj.com]

• Kidney Failure

  The renal and liver functions normalized in all patients after recovery, including in those with pre-existing liver or kidney failure. [ncbi.nlm.nih.gov]

  Kidney failure occurs rarely. [infobaby.org]

• Headache

  The prodromic phase was variable; patients complained of nausea, abdominal pain, malaise, polyuria-polydipsia syndrome and headaches followed by jaundice. The laboratory results indicated coagulopathy, liver function abnormalities and hypoglycemia. [ncbi.nlm.nih.gov]

  Symptoms include nausea and vomiting, abdominal pain, yellowing of the skin and eyes ( jaundice ), frequent thirst (polydipsia), increased urination (polyuria), headache, and altered mental state. [medicinenet.com]

  Again she returned with complaint of severe sustained headache. Computed tomography showed subdural hemorrhage and drainage of hematoma was performed immediately. Finally the patient recovered from all of these critical conditions. [acta.tums.ac.ir]

  Symptoms may include: Nausea and vomiting Abdominal pain, especially in the right upper side Malaise (general discomfort) Headache Tiredness Confusion Jaundice (yellow coloring of skin, eyes, and mucous membranes) The symptoms of acute fatty liver of [stanfordchildrens.org]

  Symptoms may include: nausea and vomiting abdominal pain, especially in the right upper side malaise (general discomfort) headache tiredness confusion jaundice (yellow coloring of skin, eyes, and mucous membranes) The symptoms of acute fatty liver of [medcentral.org]

• Somnolence

  Considering the patient's increasing somnolence and absence of cervical dilatation, it was decided to perform an emergency lower segment caesarean section (LSCS) without a trial of labor. [ijccm.org]

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

  Liver biopsy was done on the 5th to 15th postpartum day in 8 cases. [ncbi.nlm.nih.gov]

  Clinical findings, a thorough laboratory evaluation, and a liver biopsy are vital components of the diagnostic workup, while imaging studies are of limited benefit. [symptoma.com]

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]

Liver transplantation is rarely required for treatment of the condition, but may be needed for mothers with severe DIC, those with rupture of the liver, or those with severe encephalopathy. [en.wikipedia.org]

Treatment - Acute fatty liver of pregnancy Treatment involves stabilizing the mother and delivery of the baby. Resources - Acute fatty liver of pregnancy Not supplied. [checkorphan.org]

The condition was previously thought to be universally fatal, but aggressive treatment by stabilizing the mother with intravenous fluids and blood products in anticipation of early delivery has improved prognosis. [checkorphan.org]

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]

Clinical and istopathologic features, possible etiologic factors and possible treatment for the mother and fetus are discussed. [ncbi.nlm.nih.gov]

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. [gut.bmj.com]

This review examines recent literature on the epidemiology, pathogenesis, diagnosis, and treatment of acute fatty liver of pregnancy. [readbyqxmd.com]

As these triglyceride-rich particles cause endothelial dysfunction, they may contribute to the pathophysiology of AFLP. [ncbi.nlm.nih.gov]

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]

Our review summarizes the current knowledge in pathophysiology, diagnostic approach and management of this disorder. [uthealth.influuent.utsystem.edu]

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]

Can I do anything to prevent this condition? Unfortunately, we do not know enough about the risk factors for this condition to determine any possible preventive strategies. Are AFLP and fatty liver disease the same thing? No! [sampan.org]

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.