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Query: UMLS:C0015695 (fatty liver)
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Objective: To investigate the early recognition and management of acute fatty liver of pregnancy (AFLP) to improve the maternal and fetal survival.Study Design: Eight cases presenting with AFLP managed in our hospital during the past 212 years were studied retrospectively with emphasis on presenting symptoms, laboratory findings, and the time for liver biopsy. Also, to report the maternal and fetus outcomes in such cases.Result: The mean gestational age at onset was 34 +/- 2 weeks (range 30-37 weeks). All cases were primigravida. In the early stages, all presented with malaise, nausea, vomiting, and epigastric distress followed by jaundice in the third trimester of pregnancy. Three of eight presented with polyuria and polydipsia. Laboratory findings: all had raised transaminases and serum bilirubin (2.9-29.9 mg/dL), hypoalbuminemia (22.4-30 g/L), hypofibriogenemia (< 180 mg/dL), prolonged prothrombin time, and prolonged partial thromboplastin time. Maternal complication was frequent, including hepatic encephalopathy (6), ascites (6), hypoglycemia (5), hematemesis (2), postpartum hemorrhage (5), and preeclampsia (4). Cesarean was performed in 3 cases. One mother died of fulminant hepatic failure, the other cases were survival. There were no fetal deaths. Liver biopsy was done in 8 cases. It is suggested that percutaneous liver biopsy should not be done until the coagulation tests become normal, the amounts of ascites decrease and platelet counts increase after delivery.Conclusion: With increasing awareness, especially in the early recognition of AFLP cases and prompt progressive management, including early termination of pregnancy, and using large-dose infusion of fresh frozen plasma or albumine alternatively, the prognosis of AFLP is obviously improved.
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PMID:Acute fatty liver of pregnancy: an experience in diagnosis and management of eight cases. 1083 62

The authors diagnosed disturbance of liver-function associated with severe thrombopenia in a pregnant woman in the third trimester. Principally, acute fatty liver of pregnancy can be characterized by existing symptoms, e.g. nausea, vomiting, epigastric pain, jaundice, hyperbilirubinemia, moderately elevated SGOT and SGPT levels, thrombopenia, leukocytosis, low fibrinogen level and disseminated intravascular coagulopathy, but hepatomegaly, purpura and petechia on lower and upper extremities, and high ALP and GGT levels during postpartum period do not confirm suspicion of this diagnosis. The present report draws attention to the difficulties of differential diagnosis of pregnancy-induced elevated liver enzymes diseases associated with low platelets, as there are several identical pathophysiological processes. Although causes and exact pathophysiology of disorders are unknown, similar symptoms during the process of diseases leave the question open whether they are different diseases or whether they are different manifestations of the same disease, and what kind of relationship exists between these diseases and preeclampsia. This case suggests careful evaluation of the whole clinical picture, moreover it is emphasized that prompt, aggressive treatment of hemostatic disturbance and the expeditious delivery can save maternal life.
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PMID:[Atypical process of acute disturbance of liver function with severe thrombocytopenia in the third trimester]. 1100 36

Lactic acidosis and hepatic steatosis caused by mitochondrial toxicity of nucleoside reverse transcriptase inhibitors (NRTI) is a rare cause of liver disease with a high mortality rate. This report describes a male, HIV-positive patient with a 4-week history of nausea, vomiting and abdominal pain. His medication consisted of prednisone 5 mg od (because of auto-immune thrombocytopenia), didanosine (for 2 years) and stavudine (for 3 months). Laboratory studies showed cholestasis and elevation of aminotransferases. Lactic level was not measured. Liver biopsy revealed steatosis and cholestatic hepatitis. In the absence of other causes of liver disease a probable diagnosis of stavudine-induced hepatic toxicity was made. After discontinuation of NRTI, he recovered completely. Because lactic acidosis had not been confirmed, stavudine was restarted and within 1 week the lactate level increased significantly. Therefore stavudine was discontinued again. One year later the patient is doing well on a double protease inhibitor regimen. In conclusion, clinicians treating patients with NRTI should be aware of the risk of lactic acidosis and hepatic steatosis. When this is suspected, all NRTI must be stopped. The diagnosis can be made when elevated lactate levels and hepatic steatosis are present in the absence of other causes of liver disease.
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PMID:Hepatic steatosis and lactic acidosis caused by stavudine in an HIV-infected patient. 1106 65

Type B lactic acidosis is a rare and often fatal complication seen in patients receiving the nucleotide analogues zidovudine, stavudine, didanosine, and lamivudine. We describe a case of a 51-year-old human immunodeficiency virus (HIV)-positive woman receiving three nucleotide analogues. She presented with nausea, vomiting, abdominal pain, and hepatic steatosis. Signs of mitochondrial toxicity were demonstrated by diffuse myopathy and pancreatitis. Serum riboflavin levels documented a deficiency that was treated with 50 mg of riboflavin daily. Immediately after treatment, serum blood urea nitrogen level, lactic acid levels, and arterial blood pH all returned to normal values. Her signs of mitochondrial toxicity also improved after treatment with riboflavin. Successful reversal of the patient's type B lactic acidosis after riboflavin therapy suggested that riboflavin deficiency plays a direct role in the development of nucleotide analogue-induced lactic acidosis. It is impossible to predict which patients are predisposed to the development of this syndrome. For this reason, it may be important to screen and treat riboflavin deficiency in patients on nucleoside analogues.
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PMID:Emerging role of riboflavin in the treatment of nucleoside analogue-induced type B lactic acidosis. 1178 75

Anti-retroviral therapy has still many difficulties for the continuous viral suppression, although it has markedly improved the prognosis of the patients with HIV/AIDS. It is essential that anti-retroviral therapy requires strict adherence of the patients. More than 95% of adherence to have medicine is required for the success of the therapy. Severe and frequent adverse reactions, many pill burden, food restriction and patients' poor recognition to the therapy influence adherence. Of those, adverse reaction of the drugs is the strongest factor for disturbing adherence. Digestive tract symptoms such as nausea, vomiting, abdominal pain and/or diarrhea are common and affect to reduce adherence. Recently, lactic acidosis/hepatic steatosis and lipodystrophy syndrome have been recognized as novel and important adverse reactions with anti-retroviral medicines. Physicians should be aware of the importance of adherence, and assist the patients to improve it with multiple approaches.
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PMID:[Challenges of the anti-retroviral therapy]. 1196 83

Acute liver disease was diagnosed in three pregnant patients: two 30-year-old women had a 'haemolysis, elevated liver enzymes, low platelets' (HELLP) syndrome and acute fatty liver of pregnancy, respectively, and a 20-year-old woman had acute liver failure due to acute hepatitis B. The first two patients had a caesarean section, the third one delivered her child, which died spontaneously shortly after birth at a gestational age of 23 weeks. She was then treated by liver transplantation. All three patients left the hospital in good condition. Liver diseases in pregnancy may be pregnancy-related, e.g. the HELLP syndrome and acute fatty liver of pregnancy, but they may also be coincidental phenomena, e.g. viral hepatitis. The HELLP syndrome is often associated with pre-eclampsia, and presents with epigastric pain and thrombocytopenia with haemolysis. Acute fatty liver disease and acute liver failure due to hepatitis present with liver insufficiency characterised by anorexia, nausea, coagulopathy, hypoglycaemia and elevated serum ammonia levels. Management depends on the diagnosis and the gestational age; pregnancy complicated by acute fatty liver disease should be terminated while pregnancy complicated by the HELLP syndrome early in pregnancy may be maintained to improve the outcome of the foetus. In acute liver failure due to viral hepatitis, termination of pregnancy alone does not affect the disease.
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PMID:[The pregnant patient with acute liver disease]. 1253 8

Nucleoside analog reverse transcriptase inhibitors (NRTI) have been used to treat HIV-infected patients for >10 years. Some severe adverse events have been attributed to mitochondrial dysfunction. Since 1991, cases of severe lactic acidosis have been reported in association with nucleoside therapy. Our objective was to report two cases of metabolic acidosis and hepatic steatosis in patients receiving stavudine (d4T) and to review the literature. A male and a female, 47 and 45 years of age, respectively, presented with abdominal pain, nausea, vomiting, and weakness after 9 and 6 months, respectively, of treatment with stavudine. At presentation, both patients had severe metabolic acidosis and liver failure. Ultrasonography showed hepatic steatosis (confirmed by biopsy in one case). All antiretroviral drugs were withdrawn and patients were treated with bicarbonate. Both patients developed fulminant liver dysfunction and multiple organ failure. We reviewed the literature and found 75 cases of lactic acidosis and hepatic steatosis associated with use of NRTI; 57 of these patients received d4T (76%). Of all cases reported in association with nucleoside therapy, 63% were females and mortality was 47%. General weakness, hepatic enzyme elevation, and liver steatosis are data that should alert physicians to this serious adverse event and to respond with prompt interruption of antiretroviral drugs and measurement of lactic acid in plasma. It is important to report serious adverse events in commercially released drugs to know prevalence in an exposed population. Physicians should be aware of risk and early signs of this serious adverse event.
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PMID:Metabolic acidosis and hepatic steatosis in two HIV-infected patients on stavudine (d4T) treatment. 1260 78

Hyperlactataemia is seen in 8-18.3% of HIV-infected patients taking nucleoside-analogue reverse transcriptase inhibitors (NRTIs). Recent epidemiological studies suggest that most episodes are transient and subclinical. However, symptomatic and occasionally life-threatening cases accompanied by metabolic acidosis and hepatic steatosis (ie, lactic acidosis syndrome) have also been described. Though yet to be fully elucidated, the proposed mechanism is NRTI-induced inhibition of mitochondrial DNA polymerase culminating in derangements in oxidative phosphorylation and lactate homeostasis. Signs and symptoms range from mild hyperlactataemia accompanied by nausea, abdominal discomfort, and weight loss to severe, intractable lactic acidosis complicated by coma and multi-organ failure. Significant progress has recently been made with regard to the natural history of NRTI-related hyperlactataemia. However, other important aspects of the disorder, such as its pathogenesis, predisposing conditions, and management, remain poorly understood. This article reviews the current published work on these issues, identifies areas of controversy, and addresses directions for future research.
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PMID:Hyperlactataemia syndromes associated with HIV therapy. 1452 59

Liver diseases specific of pregnancy, the most common hepatic complications of pregnancy, are always associated with a sometimes asymptomatic increase in serum aminotransferase activity. The most frequent of the liver diseases specific of pregnancy in normotensive pregnant women is cholestasis of pregnancy, the cause of generalised pruritus, and, in those with pregnancy-induced hypertension, preeclampsia which requires short-term cessation of pregnancy. Similar treatment is required by acute fatty liver of pregnancy the diagnosis of which must be done in the third trimester when recent polydipsia, nausea or vomiting occurs. Moreover, pregnancy increases the incidence and/or the severity of herpes simplex hepatitis (for which acyclovir therapy is urgently required) and hepatitis type E. Pregnancy may also unmask untreated cases of autoimmune hepatitis, Wilson's disease or Budd-Chiari syndrome.
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PMID:[Hepatic complications of pregnancy]. 1472 76

We report the case of a 26-year-old second gravida in the third trimester of pregnancy who presented with a history of nausea, repeated vomiting and jaundice. The patient was diagnosed as acute fatty liver of pregnancy. After delivery, the condition of the patient progressed to grade IV encephalopathy and did not improve despite all intensive clinical management measures. After 3 days in grade IV encephalopathy, the patient was infused 3 x 10(8) human foetal hepatocytes. The patient's level of consciousness started improving after 24 hours of foetal hepatocyte transfusion and she recovered completely within 7 days.
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PMID:Peritoneal transplantation of human fetal hepatocytes for the treatment of acute fatty liver of pregnancy: a case report. 1568 63


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