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Acute fatty liver of pregnancy is considered an uncommon disorder that may complicate the third trimester of pregnancy, with high fetal and maternal mortality rates. However, in recent years it has become clear that the course of the disorder can be less fulminant than previously believed. The diagnosis is also made more frequently. The case histories of three patients are described followed by a review of the clinical and diagnostic features. It is concluded that with all women showing symptoms of malaise, nausea, vomiting and upper abdominal pain in the third trimester of pregnancy liver enzymes should be measured. If evidence of liver dysfunction is found, acute fatty liver of pregnancy should be considered in the differential diagnosis. Only early recognition and prompt treatment will improve both fetal and maternal survival.
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PMID:[Acute fatty degeneration of the liver in pregnancy]. 239 79

The clinical features, laboratory investigations and histopathology of 12 patients with idiopathic acute fatty liver of pregnancy are presented. Repeated vomiting, starting in the last trimester, was the cardinal symptom. Seven patients had proteinuria, hypertension and peripheral oedema before jaundice appeared. Caesarian section and induction of labour led to a lower than expected maternal mortality (33 X 3 per cent) and foetal mortality (66 X 7 per cent). There was a high incidence of twin and male births. Neutrophilia, thrombocytopenia and normoblasts were a uniform feature and uric acid levels were universally high. These findings may be useful in diagnosis in conjunction with liver function tests. Hepatic histology showed pathognomonic microvesicular fat in swollen hepatocytes with central nuclei and centrilobular distribution. However, a diffuse pattern and the presence of significant inflammation and fibrin deposits led to an initial misdiagnosis in two patients. Histology of fetal livers and five placentae was normal. Seven subsequent normal pregnancies occurred in four patients. Acute fatty liver of pregnancy may be confused with acute hepatitis or toxaemia on both clinical and histological grounds. Accurate diagnosis should lead to improved management and lessen maternal and fetal mortality. This justifies more intensive and urgent investigation of nausea, vomiting and jaundice in the last trimester of pregnancy.
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PMID:Idiopathic acute fatty liver of pregnancy in 12 patients. 715 26

Acute fatty liver of pregnancy is a rare clinical entity unique to pregnancy that can lead to hepatic failure and encephalopathy and, if the diagnosis is delayed, to death for the baby and the mother. The characteristic histological picture demonstrates microvesicular fatty infiltration of hepatocytes. Acute fatty liver of pregnancy is a disease of the third trimester of pregnancy. The most significant clinical findings are nausea or vomiting, abdominal pain, jaundice, hepatic encephalopathy, increased transaminase levels, decreased platelet count, increased prothrombin time, and renal failure. Hypertension and proteinuria are common. Liver biopsy is not always necessary for diagnosis but may be useful in atypical cases. The primary therapy is early delivery and supportive care. Both the obstetric team and the medical consultants must have a high index of suspicion for this disease because early delivery is lifesaving and has transformed the prognosis for the mother and the baby. Collaboration between obstetricians and gastroenterologists is necessary to make the diagnosis and also to improve our understanding of this disease of unknown etiology.
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PMID:Acute fatty liver of pregnancy: the hepatologist's view. 805 22

Acute fatty liver of pregnancy is a rare clinical entity unique to pregnancy that occurs during the third trimester. 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. 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 in late pregnancy. The disease rarely recurs during a subsequent pregnancy. The cause is unknown, but some cases of acute fatty liver of pregnancy have been associated with a genetic deficiency of fatty acid beta-oxidation. Because of the possibility of this congenital deficiency, infants of affected mothers should undergo close follow-up from birth.
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PMID:Acute fatty liver of pregnancy. 963 7

Acute fatty liver of pregnancy (AFLP), rare and potentially fatal disease, is considered as obstetric emergency. The perinatologist must be familiar with this complication of pregnancy because early diagnosis, prompt delivery and supportive care is the mainstay of therapy. When pregnant woman has nausea or vomiting, abdominal pain and jaundice, increased transaminase activity, coagulopathy and hypoglycemia during third trimester AFLP should be suspected. Mothers who have experienced AFLP must be informed of the possibility recurrence and undergo close surveillance in the next pregnancy.
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PMID:[Acute fatty liver in pregnancy: treatment, prognosis, rules of management]. 1058 7

Acute fatty liver of pregnancy (AFLP) is a rare disorder of unknown aetiology that is diagnosed typically in the third trimester or early postpartum period. The incidence is estimated to be 1/6692-1/13,328. The obstetric team must have a high index of suspicion of this pathology, particularly in the presence of clinical and laboratory findings, such as nausea, vomiting, jaundice, increased serum transaminase levels, increased prothrombin time and hypoglycaemia. Early diagnosis followed by prompt delivery and supportive care provides significantly improved maternal and perinatal outcome. Delay in diagnosis of this obstetric emergency may lead to rapid progression to hepatic failure, disseminated intravascular coagulation (DIC), haemorrhage, encephalopathy, multiple organ failure and finally death. The case of a 34-year-old woman, gravida 3, para 2, with AFLP complicated with DIC is presented herein with a review of literature and discussion of its origin.
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PMID:Acute fatty liver of pregnancy complicated with disseminated intravascular coagulation and haemorrhage: a case report. 1587 33

Acute fatty liver of pregnancy is a rare clinical syndrome of pregnancy that occurs during the third trimester. Clinicians must have a high index of suspicion for this condition when a woman has nausea or vomiting during the last trimester. Early diagnosis and prompt delivery improve foetal and maternal prognosis. We report a case of a previously healthy 23-year-old woman who presented an acute fatty liver of pregnancy with intrauterine fetal death. Based on this experience as well as on medical literature, characteristics of this uncommon pathology are discussed.
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PMID:[Acute fatty liver of pregnancy: a rare pathology of the third trimester]. 1644 27

Acute fatty liver of pregnancy is an uncommon and potentially fatal disorder usually found in the third trimester of pregnancy and the early days postpartum. It is characterized by a micro vesicular stheatosis of the liver, which is reversible with complete regression of the disease. We present two clinical cases, in the first one the patient developed encephalopathy and hypoglycemia in her third day post cesarean section; the patient underwent surgical exploration because of a subaponeurotic hematoma and the diagnosis was confirmed by liver biopsy. The patient presented spontaneous resolution of the disease and was discharged home on her 21st day after delivery. The second case was a patient on her 37th week of pregnancy that arrived to the emergency room complaining of malaise, nausea and vomit, her laboratory analyses were remarkable hypoglycemia and hyperbilirubinemia. She underwent elective pregnancy termination with a favorable maternal and fetal outcome towards spontaneous resolution. It is critical to diagnose and treat this disease in a expedite manner in order to have a favorable maternal and fetal outcome. The mortality rate has decreased dramatically from 80% in 1965 to 100% survival in 1999 according to the latest reviews. This regression of the symptoms and signs along with normalization of the laboratory values has driven some authors to call this disease as the "reversible peripartum liver failure".
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PMID:[Fatty liver in pregnancy. A report of two cases and medical literature review]. 1688 59

Acute fatty liver of pregnancy (AFLP) is a rare, potentially fatal complication that occurs in the third trimester or early postpartum period. It generally appears between weeks 30 and 38. Usually the APLF symptoms start one to two weeks before hospitalization with nausea, emesis, general uneasiness, jaundice, epigastric pain and other symptoms. A careful history and physical examination, in conjunction with compatible laboratory and imaging results, are often sufficient to make the diagnosis, and liver biopsy is rarely indicated. The maternal outcome has improved enormously during the last decade. Early diagnosis, pregnancy termination and handling in special care or treating complications has lead to good maternofetal results.
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PMID:[Acute fatty liver of pregnancy. "Incidence, pathogenesis, diagnosis, treatment and prognosis"]. 1921 41

Liver disorders occurring during pregnancy may be specifically pregnancy-related, or may be due to an intercurrent or chronic liver disease, which may present in anyone, pregnant or not. This review focuses on the liver diseases unique to pregnancy. Hyperemesis gravidarum, which occurs during early pregnancy, may be associated with liver dysfunction. Intrahepatic cholestasis of pregnancy typically occurs during the second or third trimester. Pruritus and the associated biological signs of cholestasis improve rapidly after delivery. Mutations in gene encoding biliary transporters, especially ABCB4 encoding the multidrug resistance 3 protein, have been found to be associated with this complex disease. Ursodeoxycholic acid is currently the most effective medical treatment in improving pruritus and liver tests. Pre-eclampsia, which presents in late pregnancy frequently involves the liver, and HELLP syndrome (Hemolysis-Elevated Liver enzymes-Low Platelets) is a life-threatening complication. Prognosis of acute fatty liver of pregnancy has been radically transformed by early delivery, and clinicians must have a high index of suspicion for this condition when a woman presents nausea or vomiting, epigastric pain, jaundice, or polyuria-polydipsia during the third trimester. 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).
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PMID:Liver diseases unique to pregnancy: a 2010 update. 2131 Jun 83


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