Acute fatty liver of pregnancy

Abnormal and rapid accumulation of fat in the liver during pregnancy. It usually manifests during the third trimester and may be associated with mutations in the long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) gene. OMIM: 609016

Also Known As:

Networked: 224 relevant articles (2 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Abruptio Placentae (Placental Abruption)
2.	Fatty Liver
3.	Hemolysis
4.	Pre-Eclampsia (Preeclampsia)
5.	HELLP Syndrome

Experts

1.	Nelson, David B: 4 articles (04/2022 - 07/2014)
2.	Ibdah, Jamal A: 4 articles (01/2018 - 11/2002)
3.	Cunningham, F Gary: 3 articles (04/2022 - 07/2014)
4.	Minakami, Hisanori: 3 articles (03/2014 - 12/2002)
5.	Rakheja, Dinesh: 3 articles (01/2008 - 07/2002)
6.	Strauss, Arnold W: 3 articles (05/2005 - 11/2002)
7.	Bennett, Michael J: 3 articles (06/2003 - 05/2002)
8.	Shah, Silvi: 2 articles (07/2022 - 11/2020)
9.	Erez, Offer: 2 articles (01/2022 - 10/2015)
10.	Thachil, Jecko: 2 articles (01/2022 - 10/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Acute fatty liver of pregnancy:

1.	Fibrinogen (Factor I)FDA Link 11/01/1994 - "Acute fatty liver of pregnancy should be suspected in all patients with symptoms of preeclampsia in the presence of hypoglycemia, low fibrinogen, and prolonged prothrombin time, particularly in the absence of severe abruptio placentae. " 07/01/2014 - "Fibrinogen regeneration with placental abruption was rapid, whereas it remained depressed for 4-5 days with acute fatty liver of pregnancy; fibrin-fibrinogen split products were also cleared more rapidly after abruption than women with acute fatty liver (P<.001 for interaction for both using random effects modeling). "
2.	Fibrin Fibrinogen Degradation ProductsIBA 07/01/2014 - "Fibrinogen regeneration with placental abruption was rapid, whereas it remained depressed for 4-5 days with acute fatty liver of pregnancy; fibrin-fibrinogen split products were also cleared more rapidly after abruption than women with acute fatty liver (P<.001 for interaction for both using random effects modeling). "
3.	EnzymesIBA 06/01/2022 - "[A comparison of clinical characteristics between acute fatty liver of pregnancy and hemolysis, elevated liver enzymes and low platelets syndrome]." 04/01/2022 - "Comparing acute fatty liver of pregnancy from hemolysis, elevated liver enzymes, and low platelets syndrome." 04/12/2021 - "Comparison of maternal and neonatal outcomes between acute fatty liver of pregnancy and hemolysis, elevated liver enzymes and low platelets syndrome: a retrospective cohort study." 01/01/2019 - "To compare serum levels of angiogenesis-related factors between 14 women with HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome and a woman with acute fatty liver of pregnancy (AFLP). " 09/01/2017 - "Maternal hemolysis, elevated liver enzymes and low platelet count syndrome and acute fatty liver of pregnancy were noted in two and one mothers, respectively. "
4.	Fatty Acids (Saturated Fatty Acids)IBA 10/01/2022 - "Acute fatty liver of pregnancy (AFLP) is a rare liver disorder that is caused by defects in mitochondrial beta (β) oxidation of fatty acids. " 01/22/2018 - "Role of 3-Hydroxy Fatty Acid-Induced Hepatic Lipotoxicity in Acute Fatty Liver of Pregnancy." 12/01/2017 - "Acute fatty liver of pregnancy is a rare mitochondrial hepatopathy characterised by microvesicular fatty infiltration, and is believed to be due to impaired fatty acid oxidation. " 08/01/2017 - "[Acute fatty liver of pregnancy and mitochondrial fatty acid oxidation. " 10/01/2013 - "Defects in fatty acid oxidation secondary to various enzymatic deficiencies have been associated with acute fatty liver of pregnancy. "
5.	Dacarbazine (DIC)FDA LinkGeneric 12/01/1989 - "Hypotheses of acute veno-occlusive disease caused by the DIC, and acute fatty liver of pregnancy are discussed." 05/01/1982 - "[Acute fatty liver of pregnancy: report of a case complicating DIC and acute pancreatitis (author's transl)]." 01/01/1978 - "A patient with acute fatty liver of pregnancy associated with disseminated intravascular coagulation (DIC) is reported. " 08/01/2011 - "The most common obstetric causes of admission were massive postpartum haemorrhage (n=42, 20.29%) and pregnancy associated hypertension (n=36, 17.39%), followed by acute fatty liver of pregnancy (AFLP, n=27, 13.04%), obstetric disseminated intravascular coagulation (DIC, n=23, 11.11%). " 01/01/2012 - "We describe here a primigravida with acute fatty liver of pregnancy and a multipara with placental abruption who each developed uterine inertia complicated by postpartum DIC that required total hysterectomy. "
6.	Oxidoreductases (Dehydrogenase)IBA 01/01/2009 - "Acute fatty liver of pregnancy and neonatal long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency." 02/01/2004 - "Maternal acute fatty liver of pregnancy and the associated risk for long-chain 3-hydroxyacyl-coenzyme a dehydrogenase (LCHAD) deficiency in infants." 04/01/2001 - "There is a correlation between pregnancy complications such as acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency. " 09/01/2018 - "Acute fatty liver of pregnancy is a heterogeneous disorder which may occur in the absence of a detectable gene mutation for the enzyme long-chain L-3-hydroxyacyl-CoA dehydrogenase. " 03/01/2011 - "Acute fatty liver of pregnancy has been found to be associated with a defect of long-chain 3-hydroxyacyl coenzyme A dehydrogenase in the fetus, and mothers and their offspring should undergo DNA testing at least for the main associated genetic mutation (c.1528G>C)."
7.	Anesthetics (Anesthetic Agents)IBA 01/01/2019 - "Acute Fatty Liver of Pregnancy: Pathophysiology, Anesthetic Implications, and Obstetrical Management." 06/27/2017 - "Objective: To retrospectively analyze the clinical characteristics of patients with acute fatty liver of pregnancy (AFLP), and to discuss perioperative and anesthetic management. " 06/27/2017 - "[Retrospective analysis of anesthetic and perioperative management in patients of acute fatty liver of pregnancy]." 06/01/2010 - "Case report: anesthetic management of acute fatty liver of pregnancy in the postpartum period." 12/01/2007 - "Few clinical diagnoses of acute fatty liver of pregnancy (AFLP) are established immediately upon admission, while anesthetic interventions are frequently required on an emergent basis. "
8.	thiamine triphosphorate (TTP)IBA 12/01/2012 - "Particular attention should be paid to specific conditions that lead to AKI during the second and third trimesters, such as preeclampsia, HELLP syndrome, acute fatty liver of pregnancy, and TTP-HUS. " 01/01/2020 - "The cause of this endothelial aggression can be multiple: thrombocytopenic thrombotic purpura (TTP), HELLP syndrome, antiphospholipid syndrome, atypical hemolytic and uremic syndrome or acute fatty liver of pregnancy. " 12/01/2014 - "Interdisciplinary teams of physicians were able to exclude other imitators of preeclampsia, such as hemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), APS, lupus and acute fatty liver of pregnancy. " 09/01/2011 - "In particular, severe preeclampsia/HELLP syndrome, acute fatty liver of pregnancy (AFLP) and thrombotic thrombocytopenic purpura (TTP) are all frequently complicated by AKI, and share several clinical features which pose diagnostic challenges to the clinician. " 11/01/2005 - "We discuss TTP and its variants, hemolytic uremic syndrome, disseminated intravascular coagulation, heparin-induced thrombocytopenia, antiphospholipid syndrome, Evans syndrome, preeclampsia/eclampsia, HELLP syndrome, acute fatty liver of pregnancy, and multiorgan failure."
9.	Coenzyme A (CoA)IBA 03/01/2011 - "Acute fatty liver of pregnancy has been found to be associated with a defect of long-chain 3-hydroxyacyl coenzyme A dehydrogenase in the fetus, and mothers and their offspring should undergo DNA testing at least for the main associated genetic mutation (c.1528G>C)." 06/01/2003 - "Pregnancies in which the fetus had long-chain hydroxyacyl-CoA dehydrogenase deficiency have been complicated by life-threatening HELLP syndrome (haemolysis, elevated liver enzymes and low platelets) or AFL (acute fatty liver of pregnancy) during third trimester. " 06/03/1999 - "Acute fatty liver of pregnancy and the HELLP syndrome (hemolysis, elevated liver-enzyme levels, and a low platelet count) are serious hepatic disorders that may occur during pregnancy in women whose fetuses are later found to have a deficiency of long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase. " 01/01/2008 - "In some cases, severe preeclampsia and related disorders of acute fatty liver of pregnancy and hemolysis, elevated liver enzymes, low platelets syndrome are associated with inherited defects in mitochondrial beta-oxidation of fatty acids, especially a deficiency of long-chain 3-hydroxyacyl coenzyme A dehydrogenase (LCHAD). "
10.	Transaminases (Aminotransferases)IBA 11/01/2021 - "Letter to the Editor: Serum Aminotransferase Activity Beyond 100 Times the Upper Limit of Normal Value Does Not Suggest Acute Fatty Liver of Pregnancy Alone." 05/01/1994 - "All had raised liver transaminases and biopsy-proven acute fatty liver of pregnancy. " 02/01/1986 - "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. " 10/01/2004 - "Minor elevations in aminotransferases may be a harbinger of life-threatening processes, such as acute fatty liver of pregnancy (AFLP) or hemolysis, elevated liver enzyme levels, low platelet count (HELLP) syndrome. "

Therapies and Procedures

1.	Critical Care (Surgical Intensive Care) 01/01/1990 - "Acute fatty liver of pregnancy has a better prognosis than previously thought, perhaps due to diagnosis of milder cases or improved intensive care. " 03/01/1998 - "Because of the serious condition of most women who develop acute fatty liver of pregnancy, collaboration between critical care and perinatal care providers is essential for optimal maternal-fetal outcomes." 10/01/2008 - "This chapter summarizes the clinical presentation, pathophysiology, evaluation and management of six commonly encountered complications unique to pregnancy that require critical care management: obstetric haemorrhage; pre-eclampsia/HELLP (haemolysis-elevated liver enzymes-low platelets) syndrome; acute fatty liver of pregnancy; peripartum cardiomyopathy; amniotic fluid embolism; and trauma."
2.	Cesarean Section (Caesarean Section) 01/01/2016 - "Several studies have reported a positive association between caesarean section for expeditious pregnancy termination and perinatal outcomes in acute fatty liver of pregnancy (AFLP); however, the risks remain unclear and independent studies have reported conflicting findings. " 01/01/2016 - "Effect of caesarean section on maternal and foetal outcomes in acute fatty liver of pregnancy: a systematic review and meta-analysis." 02/01/2008 - "We treated a 34-year-old woman who developed acute fatty liver of pregnancy (AFLP) after delivery by caesarean section at 32 weeks of gestation. " 04/01/1995 - "Anaesthesia for caesarean section in a patient with acute fatty liver of pregnancy." 10/01/1991 - "Anaesthesia for caesarean section in a patient with acute fatty liver of pregnancy."
3.	Therapeutics 03/01/2008 - "On the basis of anamnesis, differential diagnosis, typical course and complications of the disease, and proper response to intensive supportive medical therapy, we diagnosed an acute fatty liver of pregnancy. " 01/01/2008 - "Acute fatty liver of pregnancy and molecular absorbent recirculating system (MARS)-therapy: a case report." 05/23/1992 - "[Acute fatty liver of pregnancy--differential diagnosis and supportive therapy]." 09/01/2018 - "Treating acute fatty liver of pregnancy with artificial liver support therapy: Systematic review." 01/01/2008 - "Postpartum plasma exchange as adjunctive therapy for severe acute fatty liver of pregnancy."
4.	Hysterectomy 01/01/2013 - "UKOSS studies from which data on case women were obtained included amniotic fluid embolism, acute fatty liver of pregnancy, eclampsia, peripartum hysterectomy, therapies for peripartum haemorrhage and uterine rupture. " 01/01/2012 - "We describe here a primigravida with acute fatty liver of pregnancy and a multipara with placental abruption who each developed uterine inertia complicated by postpartum DIC that required total hysterectomy. "
5.	Plasma Exchange 03/13/2023 - "To assess the prevalence, risk factors, clinical characteristics of Acute fatty liver of pregnancy (AFLP) patients, and outcomes of AFLP patients treated with plasma exchange (PE). " 03/13/2023 - "The assessment in patients with acute fatty liver of pregnancy (AFLP) treated with plasma exchange: a cohort study of 298 patients." 01/01/2021 - "Therapeutic plasma exchange in acute fatty liver of pregnancy: a case report and literature review." 02/01/2019 - "A successful use of therapeutic plasma exchange in a fulminant form of acute fatty liver of pregnancy." 01/01/2015 - "Effectiveness of combining plasma exchange with plasma perfusion in acute fatty liver of pregnancy: a retrospective analysis."