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Query: UMLS:C0015695 (fatty liver)
 13,941 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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 is a rare but potentially fatal complication of the third trimester of pregnancy. Significant improvements in morbidity and mortality have been reported in the last several years. Despite accumulation of more data about the disease, the exact pathogenesis is unknown. Many women are initially misdiagnosed with other more common causes of liver dysfunction during pregnancy. It is possible that acute fatty liver is an atypical form of preeclampsia because 30% to 40% of women with acute fatty liver also have preeclampsia. Supportive care and expeditious delivery represent the only known treatment. More data are needed about acute fatty liver of pregnancy, but the rare nature of the disease and the likelihood that most cases are not reported in the literature limit the ability systematically to study causation, disease process, and treatment options. Because of the serious condition of most women who develop acute fatty liver of pregnancy, collaboration between critical care and perinatal care providers is essential for optimal maternal-fetal outcomes.
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PMID:Acute fatty liver of pregnancy. 959 60

In distinguishing normal from abnormal hepatic changes, the author described the expected changes in liver tests that occur during complicated pregnancy. This article reviews the forms of pre-existing liver disease that may affect or be affected by pregnancy, as well as liver diseases that tend to arise during pregnancy. Among the pre-existing liver diseases are autoimmune chronic active hepatitis, which may be activated by pregnancy and tends to be associated with an increased risk of still and premature births. Worsening of chronic hepatitis B and C has occasionally been observed. While some women with cirrhosis can sustain a normal pregnancy without any worsening of hepatic function, others develop liver failure; plus, women with cirrhosis are less fertile and have higher rates of both stillbirths and premature infants. Other liver disorders that may or may not be affected by pregnancy include Dubin-Johnson syndrome, Gilbert syndrome, benign recurrent intrahepatic cholestasis, Wilson's disease, hepatic adenomas, and focal nodular hyperplasia. Among the hepatic disorders that occur during pregnancy in normally healthy women and then resolve after delivery is intrahepatic cholestasis of pregnancy (also known as pruritus gravidarum, recurrent intrahepatic cholestasis of pregnancy, and obstetric hepatosis). Others include acute fatty liver of pregnancy and HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count), which may be part of the spectrum of disorders associated with pre-eclampsia/eclampsia. Pregnancy may also trigger the dissemination of herpes infection to the liver.
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PMID:Liver problems in pregnancy: part 2--managing pre-existing and pregnancy-induced liver disease. 973 96

Acute viral hepatitis is the most common cause of jaundice in pregnancy. The course of acute hepatitis is unaffected by pregnancy, except in patients with hepatitis E and disseminated herpes simplex infections, in which maternal and fetal mortality rates are significantly increased. Chronic hepatitis B or C infections may be transmitted to neonates; however, hepatitis B virus transmission is effectively prevented with perinatal hepatitis B vaccination and prophylaxis with hepatitis B immune globulin. Cholelithiasis occurs in 6 percent of pregnancies; complications can safely be treated with surgery. Women with chronic liver disease or cirrhosis exhibit a higher risk of fetal loss during pregnancy. Preeclampsia is associated with HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome, acute fatty liver of pregnancy, and hepatic infarction and rupture. These rare diseases result in increased maternal and fetal mortality. Treatment involves prompt delivery, whereupon the liver disease quickly reverses. Therapy with penicillamine, trientine, prednisone or azathioprine can be safely continued during pregnancy.
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PMID:Liver disease in pregnancy. 1006 7

Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is one of the recently discovered defects of mitochondrial fatty acid beta-oxidation. As a group, the beta-oxidation defects are among the most common inherited metabolic disorders, and LCHAD deficiency appears to be the most frequently diagnosed beta-oxidation defect in Finland. In the vast majority of patients, LCHAD deficiency is caused by a common autosomal recessive mutation G1528C. Like several beta-oxidation defects, it presents during infancy with hypoglycemic coma, hepatic steatosis, and hypocarnitinemia. Other manifestations are cardiomyopathy and rhabdomyolysis, which are frequent in defects of long-chain fatty acid oxidation. In addition, LCHAD deficiency has specific features, namely peripheral neuropathy and chorioretinopathy. Female carriers of LCHAD deficiency are prone to have preeclampsia-related pregnancy complications. Diagnosis is suggested by 3-hydroxylated acylcarnitine species in blood and the definitive diagnosis can be made by measuring intermediates of fatty acid beta-oxidation in fibroblasts or by detecting disease causing mutations. Analysis of the frequency of the G1528C mutation in Finland revealed carrier frequency of 1:240. Because of therapeutic and prenatal diagnostic opportunities in LCHAD deficiency, it is important to recognize this severe disorder early in its course.
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PMID:Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. 1022 30

Fetal-maternal interactions are critical determinants of maternal health during pregnancy and perinatal outcome. This review explores the causative relationship of a fetal disorder of mitochondrial fatty acid oxidation, long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, and the serious maternal liver diseases of pregnancy-preeclampsia, the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet counts), and acute fatty liver of pregnancy. Features of the metabolic adaptation necessitated during the fetal-neonatal transition; common phenotypes of pediatric fatty acid oxidation disorders, including neonatal hypoketotic, hypoglycemia and hepatic crisis; and clinical abnormalities of HELLP and acute fatty liver of pregnancy are presented. Evidence that a common mutation in the alpha-subunit (LCHAD) of trifunctional protein, E474Q, is always one of the mutant alleles in fetal isolated LCHAD deficiency associated with these disorders of pregnancy that cause high maternal, fetal, and newborn morbidity and mortality is reviewed. Recommendations for molecular testing for LCHAD deficiency in families with life-threatening maternal liver disease are given.
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PMID:Inherited long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and a fetal-maternal interaction cause maternal liver disease and other pregnancy complications. 1033 63

Maternal death during pregnancy, although uncommon, may result from a broad range of conditions. In this paper, a case of thrombotic thrombocytopenic purpura diagnosed by postmortem examination is presented. Thrombotic thrombocytopenic purpura is one of a subset of diseases that result in the formation of microthrombi within the vasculature, either as a primary or secondary manifestation. Other conditions included in the differential diagnosis during pregnancy are hemolytic uremic syndrome, systemic lupus erythematosus, preeclampsia-eclampsia and the HELLP syndrome, acute fatty liver of pregnancy, antiphospholipid antibody syndrome, and disseminated intravascular coagulation. The histologic manifestations of these diseases can be similar and in most cases do not provide adequate information to accurately differentiate these diseases in the postmortem period. This paper addresses the need for clinical history (i.e., symptomatology, trimester of onset) and antemortem laboratory testing in addition to a thorough autopsy to accurately differentiate among the conditions named previously. In the absence of an adequate clinical history and antemortem laboratory testing, the more general diagnosis of "thrombotic microangiopathy of pregnancy" is acceptable.
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PMID:Death due to thrombotic thrombocytopenic purpura in pregnancy: case report with review of thrombotic microangiopathies of pregnancy. 1041 63

The spectrum of complications with pre-eclampsia, which may include AFLP (acute fatty liver of pregnancy) as well as the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), is resolved by early delivery. However, the ravages of HUS/TTP (hemolytic uremic syndrome/thrombotic thrombocytopenic purpura) require therapy usually by plasma exchange. Overlap between these two groups of syndromes has occurred on rare occasions and usually requires the therapy of the predominant or more dangerous or threatening form. Such overlap can be appreciated and then treated successfully without residual morbidity. The index case is presented and an extensive review of the two groups of syndromes is provided.
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PMID:Persistant pre-eclampsia post partum with elevated liver enzymes and hemolytic uremic syndrome. 1044 Sep 42

Patients with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency present with a Reye-like syndrome, cardiomyopathy, or sudden unexpected death. We describe an unusual presentation in a patient with unsuspected LCHAD deficiency. The proband presented at 2 months of age with an acute infantile hypocalcaemia and vitamin D deficiency associated with occult, unexplained cholestatic liver disease. Sudden, unexpected death occurred at 8 months. Molecular analysis revealed homozygosity for the prevalent LCHAD (1528G > C, E474Q) mutation. The mother had pre-eclampsia during the third trimester of her pregnancy. In a subsequent pregnancy, she developed severe acute fatty liver of pregnancy (AFLP) and intrauterine fetal death at 33 weeks of gestation. In conclusion, infantile hypocalcaemia is an unusual phenotype associated with LCHAD deficiency. The maternal pregnancy history documents that fetal LCHAD deficiency is associated with a spectrum of maternal illnesses ranging from pre-eclampsia to life-threatening AFLP.
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PMID:Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcaemia. 1051 81

Transaminase level elevation during pregnancy should be viewed as abnormal and evaluated. A high index of suspicion for acute fatty liver of pregnancy should be maintained during the third trimester, since early delivery has radically transformed the maternal and fetal prognosis of this condition. Pruritus is the main symptom of intrahepatic cholestasis, which carries a risk for the fetus. Urinary tract infection can cause cholestasis or worsen intrahepatic cholestasis of pregnancy. In patients with preeclampsia, rapid delivery should be considered if there is evidence of HELLP syndrome. Patients with mild chronic viral hepatitis can usually carry a pregnancy to term without undue difficulty. Neonates born to HBsAg-positive mothers should receive HBV-Ig and vaccine at birth to prevent perinatal transmission of the HBV. In patients with chronic hepatitis C, serum transaminase levels often return to normal during pregnancy, although the virus remains detectable in the blood. Mother-to-infant transmission of the HCV is possible but fairly uncommon if the mother is HIV-negative.
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PMID:[Liver and pregnancy]. 1060 76


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