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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 occurrence of hepatobiliary disease with or without jaundice during pregnancy provides both the hepatologist and obstetrician with an interesting and urgent diagnostic challenge. Advances in our understanding and management of liver disorders unique to pregnancy and hepatobiliary disease in general have resulted in a significant improvement in the outcome for both mother and fetus. Certain disorders such as acute fatty liver of pregnancy and hepatic haemorrhage associated with toxaemia should be considered medical emergencies and delay in diagnosis of these conditions will probably adversely affect maternal and fetal outcome. A careful clinical history, physical examination, appropriate laboratory tests and radiological investigations should allow a diagnosis within 24-48 hours of presentation. Liver biopsy is rarely required. A careful history may provide important information. Does the patient have pre-existent liver disease? Has there been contact with hepatitis, intravenous drug abuse or any other factor predisposing to acute viral hepatitis? Does the patient have a family history of pruritus and/or jaundice to suggest intrahepatic cholestasis of pregnancy? Is the patient's alcohol consumption excessive? Has the patient received any hepatotoxic medications? Has there been abdominal pain and/or fever to suggest gallstones, hepatic bleeding or acute fatty liver of pregnancy? Laboratory investigations may give valuable diagnostic clues. Marked aminotransferase elevation would suggest acute viral or 'ischaemic' hepatitis. Haematological features of microangiopathic haemolysis would point towards toxaemia or AFLP. Hepatitis A and B serological tests may be helpful in viral liver disease. Radiological investigations may be indicated depending on the clinical context. Abdominal ultrasonography may be useful in the diagnosis of gallstones, biliary obstruction, liver tumours or intrahepatic bleeding. Fatty infiltration of the liver may be diagnosed by ultrasonography but computed tomography (CT) of the abdomen is probably more reliable for a diagnosis of acute fatty liver of pregnancy as it allows measurement of liver density which is typically reduced by fatty infiltration. CT scanning is also probably more valuable than ultrasound in assessing the extent of capsular rupture and haemorrhage into the liver and peritoneal cavity.
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PMID:Jaundice in pregnancy. 265 65

The authors analyse 115 cases of acute fatty liver of pregnancy, proven histologically. Characteristics of the condition is the finding of central nuclei in the hepatocytes containing microvesicular droplets. The disease occurs more frequently in primiparous women (54 per cent) and usually occurs in the third trimester of the pregnancy. A pre-icteric phase usually precedes the jaundice and during that time there is usually vomiting and/or nausa with abdominal pain or anarexia. In 92 per cent of case there is transient loss of consciousness with hepatic encephalopathy. Further tests show that there is more defective liver function than would be expected from the extent of cell lysis; and there is defective renal function. The worst complications are intestinal haemorrhages (48 per cent of cases)--genital bleeding (43 per cent of cases)--shock--diffuse intravascular coagulation and complications associated with coma. Maternal mortality at present runs at 25 per cent and fetal mortality at 60 per cent. The condition does not recur. Early evacuation of the uterus is recommended by most authors and does probably improve the outlook. The various hypotheses concerning the aetiology are discussed.
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PMID:[Acute fatty liver of pregnancy]. 354 2

Drinking pattern as well as clinical, biochemical and histological findings were recorded of 282 males with alcohol-induced liver disease (fatty liver in 103, hepatitis in 61, cirrhosis in 118). The proportion of persons under 50 years of age was significantly greater with alcoholic hepatitis (70%) than cirrhosis (46%). Mean daily alcohol consumption was clearly lower among those with fatty liver than hepatitis or cirrhosis (P less than 0.02). Duration of alcohol abuse was on average shorter in patients with fatty liver and hepatitis than with cirrhosis (excessive consumption of less than 15 years was 61% and 62%, respectively, in the former, 28% in the latter (P less than 0.02). Symptoms and clinical and biochemical findings did not help in differentiating between hepatitis without cirrhotic change and cirrhosis. The most marked differences between cirrhosis and hepatitis, on one hand, and fatty liver, on the other, related to the frequency of certain signs and symptoms: upper abdominal pain, hard consistency of the liver, generalized jaundice, bleeding from esophageal varices and ascites; among biochemical findings they were: elevation of serum-bilirubin concentration above 34 mumol/l (2 mg/dl), lowering of the Quick values and of albumin concentration. Mortality rate during hospital stay was lower among patients with hepatitis but no cirrhotic change (6.6%) than among those with cirrhotic change (31.4%). While the prognosis under abstinence was relatively more favourable in patients with mild or moderately severe hepatitis, nonicteric forms require closer attention than has been given them so far.
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PMID:[Alcoholic fatty liver, alcoholic hepatitis and alcoholic cirrhosis. Drinking behavior and incidence of clinical, clinico-chemical and histological findings in 282 patients]. 623 65

Forty five cases of chronic pancreatitis have been diagnosed between January 1966 to July 1983 in the Hospital A. Posadas. The diagnosis was confirmed by the presence of one or more of the following data: pancreatic calcifications positive in 35, abnormal secretin test 37, ultrasonography and computed tomography pathological findings 10. Surgical operations were carried out in 25 patients and biopsy taken in 5. Thirty nine (86.6%) were males, 6 (13.3%) females, the mean age in each group was 47.4 and 39.8 years. Chronic alcoholism was certain in 41 (91.9) patients, in the remainder 4 no other etiologic factors were found. The main clinical data were: Weight loss 38 (84.4%) diabetes 34 (75.5%) pain 33 (73.3% in 7 as acute pancreatitis) Steatorrhea 23 (51.1%) jaundice 16 (35.5%- 11 by extrahepatic biliary tree obstruction, 5 by hepatic cirrhosis) pseudocysts 12 (26.6%). The more common associated diseases were: hepatic cirrhosis 6, fatty liver 2 (17.7%) gastroduodenal ulcer 6 (13.3%) cancer 4 (8.8%--gastric 1, pancreatic 3). In order to study the frequency of the clinical data the patients were grouped according to the presence or absence of calcifications and the etiologic factor Symptoms and signs were matched and statistic analysis (coefficient association phi) was made. Only a moderate association between acute pancreatitis in no calcified group and diabetes in calcified group were found. The chronologic study of certains clinical data shows that acute pancreatitis, jaundice, pseudo-cyst and surgical operations were significative more frequent in the first five years while diabetes has little more frequency in the second five year period. Twenty six surgical operations were carried out in 25 patients; 20 (76.9%) due to complications, 6 (23.1%) secondary to pain (pancreatic resection 3, pancreatoyeyunostomy 2, exploration 1). Twenty three patients were lost to follow-up, 12 died and 10 are still alive. This last group was followed at regular period, 8 remained asymptomatic and 2 have intermittent abdominal pain related to alcoholic ingestion.
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PMID:[Chronic calcified pancreatitis. Our experience]. 639 6

A 39-year-old moderately obese and alcoholic man complained of abdominal pain and nausea. An ultrasound examination of the liver showed large hyperechoic areas surrounding a limited pseudotumorous zone of relatively decreased echogenicity. CT scan showed a decreased density (35 UH) in the hyperechoic areas, suggesting fatty liver which was confirmed histologically; conversely, the hypoechoic area appeared normal on CT scan. Six months later, after reduction of caloric intake and cessation of alcohol ingestion, ultrasound examination and CT scan of the liver were normal.
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PMID:[Irregular hepatic steatosis. Ultrasonic and computed x-ray tomographic aspects]. 665 77

Brown bowel syndrome is a rare intestinal disorder associated with the deposition of lipofuscin pigment in the smooth muscle cells. We report two such cases presenting with intestinal pseudo-obstruction, abdominal pain, and body weight loss. Both cases had malabsorption and fatty liver. Exploratory laparotomy revealed brownish discoloration of the small bowel wall and enlargement of mesenteric lymph nodes. Light microscopy, autofluorescence and ultrastructure studies confirmed the deposition of lipofuscin pigments in the intestinal muscle cells and reticuloendothelial cells of mesenteric lymph nodes. In addition, the calf muscle biopsy of case 1 displayed myopathy and fatty replacement. Skeletal muscle strength of both patients was partially restored after parenteral and oral vitamin E supplement and other conservative treatment, but gastrointestinal symptoms of both patients continued to deteriorate. Thus, brown bowel syndrome associated with prolonged and severe malnutrition and possibly vitamin E deficiency appears only partially responsive to vitamin E supplementation.
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PMID:Brown bowel syndrome: report of two cases. 791 59

We report a case of fatty infiltration of the liver in a 27-year-old Hispanic woman. She went to an outside hospital in her third trimester of pregnancy with abdominal pain, jaundice, peripheral edema, and diffuse intravascular coagulation. An emergent cesarean section was performed, and she developed symptoms of acute hemorrhagic pancreatitis and altered mental status. She was transferred to Harbor County Hospital, where a computed tomography scan showed a fatty liver, and a liver biopsy confirmed the diagnosis of fatty liver of pregnancy. Prompt recognition and immediate termination of pregnancy with intensive, supportive care are essentials in the management of fatty liver of pregnancy.
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PMID:Acute fatty liver of pregnancy: laparoscopy-assisted diagnosis. 804 69

Liver disease in pregnancy should be considered in 3 categories: pre-existing disease, disease peculiar to pregnancy and coincident acute liver or gall-stone disease. In addition the time of onset of diagnosis in terms of the trimester of gestation must be verified, as the diseases peculiar to pregancy have a characteristic time of onset. In the last trimester closes obstetric management is required for the constellation of abnormal liver function tests, nausea and/or vomiting and abdominal pain. This may be due to severe pre-eclampsia, HELLP (haemolysis, elevated liver enzymes and low platelets) syndrome or acute fatty liver of pregnancy with or without sub-capsular hepatic haematomas, amongst which there is an overlap. Early delivery is curative. A molecular basis consisting of long chain 3-hydroxyl CoA dehydroxegenase deficiency in heterozygote mothers underlies this clinical syndrome. Ursodeoxycholic acid is now established treatment for intra-hepatic cholestasis of pregnancy and appears to improve foetal outcome. Hepatitis B vaccination and immunoglobulin at birth prevents chronic hepatitis B in children of HBsAg (hepatitis B surface antigen) positive carrier mothers.
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PMID:Pregnancy and liver disease. 951 93

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


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