Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0015695 (fatty liver)
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Severe liver disease complicates pregnancy in only 0.1% of the cases. Viral hepatitis is the most common cause (40%). (Liver cirrhosis usually results in amenorrhea). Liver disease unique to pregnancy comprises "intrahepatic cholestasis of pregnancy" (Increased fetal risk), "acute fatty liver of pregnancy" (AFLP) and "HELLP-syndrome", both with high maternal and fetal risk when untreated. AFLP and HELLP-syndrome are diseases of the third trimester and show similar clinical signs of jaundice, coagulopathy and elevated liver enzymes. The immediate termination of pregnancy preferably by Caesarean section has been shown to improve both, maternal and fetal outcome. Imaging methods like ultrasound are invaluable in the differential diagnosis and detection of complications like subcapsular hematoma in the liver patients with HELLP-syndrome. Fulminant hepatic failure requires intensive care, liver transplantation is an additional therapeutic option. Recurrent AFLP has been reported recently.
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PMID:[Acute hepatopathies in pregnancy: diagnosis and therapy]. 152 90

Acute liver failure (ALF) in pregnancy is a common challenging clinical problem both in terms of correct diagnosis and management. Acute viral hepatitis is the most common cause of jaundice in pregnancy. The course of acute viral hepatitis is unaffected by pregnancy, except in patients with hepatitis E (HEV), particularly from endemic countries like India, where ALF carries a high mortality. In both HEV infection and herpes simplex infections, maternal and fetal mortality rates are significantly increased. ALF specific to pregnancy including pre-eclampsia, associated with hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome, acute fatty liver of pregnancy, and hepatic infarction result in increased maternal and fetal mortality if not recognized and acted on early. Early recognition of possible causes and prompt treatment are crucial for successful outcome of ALF in pregnancy. Treatment involves prompt delivery, whereupon the liver disease quickly reverses. This review article addresses the present understanding of ALF in pregnancy reviewing the common causes of ALF and their management in pregnancy.
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PMID:Acute liver failure in pregnancy: an overview. 1829 70

Acute liver failure (ALF) during pregnancy is very uncommon. Pregnancy-specific liver conditions like hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome and acute fatty liver of pregnancy can cause ALF at term or postpartum, but, typically occur during the third trimester. Most of these patients recover spontaneously after delivery, but, on occasion, they require liver transplantation in the postpartum period. However, ALF during the first and second trimester of pregnancy requiring antepartum liver transplantation is rare. Only fifteen cases of liver transplantation during pregnancy have been reported, and very few occurred during the first trimester. We report a Woman who developed acute liver failure during the first trimester of pregnancy and underwent successful liver transplantation at 11-week gestation, followed by successful delivery of the fetus at 30 weeks. To our knowledge, this is the earliest case of successful liver transplantation during pregnancy followed by successful fetal outcome. We discuss management of the patient and fetus before, during, and after liver transplantation and review the literature on antepartum liver transplant in pregnancy.
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PMID:Liver transplantation for acute liver failure at 11-week gestation with successful maternal and fetal outcome. 2322 16

Abnormalities in liver function tests appear in 3% of pregnancies. Severe acute liver damage can be an exclusive condition of pregnancy (dependent or independent of pre-eclampsia) or a concomitant disease. HELLP syndrome and acute fatty liver of pregnancy are the most severe liver diseases associated with pregnancy. Both appear during the third trimester and have a similar clinical presentation. Acute fatty liver may be associated with hypoglycemia and HELLP syndrome is closely linked with pre-eclampsia. Among concomitant conditions, fulminant acute hepatitis caused by medications or virus is the most severe disease. Its clinical presentation may be hyper-acute with neurological involvement and severe coagulation disorders. It has a high mortality and patients should be transplanted. Fulminant hepatic failure caused by acetaminophen overdose can be managed with n-acetyl cysteine. Because of the high fetal mortality rate, the gestational age at diagnosis is crucial.
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PMID:[Causes and management of severe acute liver damage during pregnancy]. 2620 75

Acute liver failure is a rare but life-threatening medical emergency in pregnancy whose true incidence remains unknown. Many cases of acute liver failure are caused by pregnancy-related conditions such as acute fatty liver of pregnancy and HELLP syndrome. However, acute deterioration in liver function can also be caused by drug overdose, viral infections, and an exacerbation of underlying chronic liver disease. This article provides an overview of the normal liver changes that occur during pregnancy, and summarizes the most common conditions and general management strategies of liver failure during pregnancy.
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PMID:Liver Failure in Pregnancy. 2660 Apr 44

The objective of this study was to evaluate the maternal and perinatal outcome of women with liver dysfunction during pregnancy. The study involved a prospective observational study design and was carried out at the Dow University of Health Sciences and Civil Hospital Karachi, Pakistan. A total of 800 women, who delivered during the study period from January 2006 to September 2006, constituted the study population. Pregnant women with liver dysfunction underwent evaluation for the aetiology of their liver dysfunction, including screening for hepatitis E. Thirty-five women were identified with liver dysfunction. Fourteen (40%) presented in the second trimester and 21 (60%) presented in the third trimester. Twenty-two of the 35 women (63%) had isolated acute hepatitis E; five (14%) had HELLP (haemolysis, elevated liver enzymes and low platelet count) syndrome; two (6%) had intrahepatic cholestasis of pregnancy (IHCP), two had acute fatty liver of pregnancy (AFLP) and two women had hepatitis A. A specific diagnosis was not reached in two women who died prior to delivery. In women with hepatitis E, the mean values of bilirubin and alanine transaminase were 12 mg/dL and 675 U/L, respectively. Abnormal coagulation parameters were present in 20 (57%) of the women and in 18 of 22 (82%) with hepatitis E. Fulminant hepatic failure (FHF) was seen in four patients. Seven women (20%) underwent caesarean section, 26 (74%) delivered vaginally and two women died undelivered. There were six maternal deaths in the study population; two were due to hepatitis E, one each from HELLP and AFLP, and two remained undiagnosed. The overall perinatal mortality within the group was 43%. Hepatitis E was the most common cause of FHF and maternal death in pregnant women with liver dysfunction.
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PMID:Liver dysfunction in pregnancy: an important cause of maternal and perinatal morbidity and mortality in Pakistan. 2758

Anorexia nervosa (AN) has the highest mortality rate of all psychiatric illnesses due to the widespread organ dysfunction caused by the underlying severe malnutrition. Starvation causes hepatocyte injury and death leading to a rise in aminotransferases. Malnutrition-induced hepatitis is common among individuals with AN especially as body mass index decreases. Acute liver failure associated with coagulopathy and encephalopathy can rarely occur. Liver enzymes may also less commonly increase as part of the refeeding process due to hepatic steatosis and can be distinguished from starvation hepatitis by the finding of a fatty liver on ultrasonography. Individuals with AN and starvation-induced hepatitis are at increased risk of hypoglycemia due to depleted glycogen stores and impaired gluconeogenesis. Gastroenterology and hepatology consultations are often requested when patients with AN and signs of hepatitis are hospitalized. It should be noted that additional laboratory testing, imaging, or liver biopsy all have low diagnostic yield, are costly, and potentially invasive, therefore, not generally recommended for diagnostic purposes. While the hepatitis of AN can reach severe levels, a supervised increase in caloric intake and a return to a healthy body weight often quickly lead to normalization of elevated aminotransferases caused by starvation.
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PMID:Hepatic Complications of Anorexia Nervosa. 2893 25

Cell death represents a basic biological paradigm that governs outcomes and long-term sequelae in almost every hepatic disease condition. Acute liver failure is characterized by massive loss of parenchymal cells but is usually followed by restitution ad integrum. By contrast, cell death in chronic liver diseases often occurs at a lesser extent but leads to long-term alterations in organ architecture and function, contributing to chronic hepatocyte turnover, the recruitment of immune cells and activation of hepatic stellate cells. These chronic cell death responses contribute to the development of liver fibrosis, cirrhosis and cancer. It has become evident that, besides apoptosis, necroptosis is a highly relevant form of programmed cell death in the liver. Differential activation of specific forms of programmed cell death might not only affect outcomes in liver diseases but also offer novel opportunities for therapeutic intervention. Here, we summarize the underlying molecular mechanisms and open questions about disease-specific activation and roles of programmed cell death forms, their contribution to response signatures and their detection. We focus on the role of apoptosis and necroptosis in acute liver injury, nonalcoholic fatty liver disease (NAFLD), nonalcoholic steatohepatitis (NASH) and liver cancer, and possible translations into clinical applications.
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PMID:Apoptosis and necroptosis in the liver: a matter of life and death. 3025 76