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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

Acute fatty liver of pregnancy, with a case history where an early diagnosis could have been made, and a review of the French literature. Acute fatty liver of pregnancy, or Sheehan's syndrome is a rare but very serious complication of pregnancy. The disease is demonstrated by vomiting, abdominal pain and a high level of uric acid in the blood before jaundice is noted. Within a few days the triad of jaundice, pruritus and encephalopathy occur. These are often associated with toxaemia of pregnancy and with polyuria and polydipsia. A raised white blood count and a high level of bilirubinemia are almost always present. The outlook is very serious when haemorrhage appears. This malignant form of the disease is characterised by liver and kidney failure. Liver biopsy confirms the diagnosis. The prognosis is related to an early diagnosis and is good when labour is induced or caesarean section performed. Acute fatty liver of pregnancy is an emergency from the diagnostic as well as the therapeutic angles.
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PMID:[Acute fatty liver of pregnancy. Diagnostic value of hyperuricemia in the pre-jaundice stage]. 322 Oct 52

Acute fatty liver of pregnancy, or Sheehan's syndrome, is a rare complication of pregnancy, occurring in about 0.5% cases of jaundice in pregnancy. The anatomo-clinical picture and the biology of the condition is described using as basis a personal case history and the 28 cases to be found in the French literature. The condition shows, vomiting, abdominal pain and tachycardia in an apyrexial patient in the 3rd trimester of the pregnancy in which the disease occurs. The date shows itself a few days after the prodromal symptoms and is characterised by the triad of pruritus, jaundice and encephalopathy. All cases show an increased white blood count and hyperbilirubinaemia. If the condition goes on to become very serious one has to look for signs of haemorrhage and of liver and renal failure. Liver biopsy confirms the diagnosis. Prognosis will be improved if moderate forms are recognized early and if the condition is watched carefully. It may be necessary to stop the pregnancy before the serious conditions of the illness show up. Labour induced at the 37th week after checks had been made for fetal pulmonary maturity seemed to be a good way of treating the condition even if it has not yet become desperate. The final prognosis for the mother and the baby is always favourable if the baby is born alive.
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PMID:[Acute hepatic steatosis of pregnancy. Apropos of a case and review of the French literature]. 359 1

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

Twelve episodes of acute fatty liver of pregnancy (AFLP) were diagnosed in 11 patients during the past 18 years in a general hospital in Santiago, Chile, with a prevalence of 1 per 15,900 deliveries. Acute fatty liver of pregnancy started between the 31st and 38th weeks of pregnancy, with malaise, vomiting, jaundice, and lethargy as the main clinical manifestations. Polydipsia (in nine episodes) and skin pruritus (in seven episodes) were unusual clinical findings. In two patients, pruritus started two and four weeks before AFLP, suggesting that an intrahepatic cholestasis of pregnancy preceded AFLP in those patients. Considering the current prevalence of both diseases in Chile, their association should be considered fortuitous. In another patient, two consecutive pregnancies were affected by AFLP, raising to three the number of reported patients with recurrent AFLP. In 11 episodes, liver biopsies supported the diagnosis of AFLP by showing small and midsized vacuolar cytoplasmic transformation as the most prominent histopathological feature. Positive intracellular fat staining was found in the four samples analysed. Studies by electron microscopy showed megamitochondria with paracrystalline inclusions in four samples. All the mothers survived, but fetal mortality was 58.3%. Several extrahepatic complications delayed maternal recovery for up to four weeks after delivery. 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.
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PMID:Acute fatty liver of pregnancy: a clinical study of 12 episodes in 11 patients. 830 28

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


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