Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268318 (ICP)
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Liver disease in pregnancy is uncommon, acute viral hepatitis being the most frequent. The latter has a normal prognosis in pregnancy, with the possible exception of NANB hepatitis in India and North Africa. Immunization of neonates born of mothers suffering from acute or chronic HBV is essential and effective. Acute fatty liver of pregnancy has a better prognosis than previously thought, perhaps due to diagnosis of milder cases or improved intensive care. Its etiology is still unknown, but metabolic stress may be important. The confusion and overlap of AFLP, the HELLP syndrome, and liver disease of eclampsia suggest common etiological factors. Urgent delivery of the fetus is recommended in AFLP. The related condition of acute liver rupture may be diagnosed by ultrasound. Successful conservative management has been reported. Estrogens are involved in the pathophysiology of ICP, but this does not explain the profound racial differences in incidence. The nature of the sensitivity to estrogens is not understood, although reduced membrane fluidity, which may be counteracted by S-adenosyl-L-methionine, is one possible explanation. The increased fetal loss associated with ICP suggests that treatment should be more energetic than hitherto. In the worst affected individuals, fetal malnutrition secondary to maternal steatorrhea may be an important factor. In general, patients with chronic liver disease have increased maternal and particularly fetal mortality.
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PMID:Diagnosis and management of liver disease in pregnancy. 240 96

Patients with ICP should be considered to have a high-risk pregnancy. Once the diagnosis of ICP is suspected, usually because of generalized pruritus, it should be confirmed by liver function tests, and other causes of cholestasis should be ruled out. Treatment with UDCA is effective in ameliorating the cholestasis and is especially useful in severe forms or when there is a history of sudden fetal death in a previous pregnancy. The understanding of the pathogenesis of ICP has recently progressed as the result of the discovery of several defects in the MDR3 gene in isolated affected patients. More studies of this and other genes that regulate bile flow, linked with careful clinical observations to rule out unsuspected chronic liver disease not related to pregnancy, should lead to the discovery of the pathogenesis of this enigmatic disorder.
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PMID:Intrahepatic cholestasis of pregnancy. 1506 99

Abnormal liver tests occur in 3%-5% of pregnancies, with many potential causes, including coincidental liver disease (most commonly viral hepatitis or gallstones) and underlying chronic liver disease. However, most liver dysfunction in pregnancy is pregnancy-related and caused by 1 of the 5 liver diseases unique to the pregnant state: these fall into 2 main categories depending on their association with or without preeclampsia. The preeclampsia-associated liver diseases are preeclampsia itself, the hemolysis (H), elevated liver tests (EL), and low platelet count (LP) (HELLP) syndrome, and acute fatty liver of pregnancy. Hyperemesis gravidarum and intrahepatic cholestasis of pregnancy have no relationship to preeclampsia. Although still enigmatic, there have been recent interesting advances in understanding of these unique pregnancy-related liver diseases. Hyperemesis gravidarum is intractable, dehydrating vomiting in the first trimester of pregnancy; 50% of patients with this condition have liver dysfunction. Intrahepatic cholestasis of pregnancy is pruritus and elevated bile acids in the second half of pregnancy, accompanied by high levels of aminotransferases and mild jaundice. Maternal management is symptomatic with ursodeoxycholic acid; for the fetus, however, this is a high-risk pregnancy requiring close fetal monitoring and early delivery. Severe preeclampsia itself is the commonest cause of hepatic tenderness and liver dysfunction in pregnancy, and 2%-12% of cases are further complicated by hemolysis (H), elevated liver tests (EL), and low platelet count (LP)-the HELLP syndrome. Immediate delivery is the only definitive therapy, but many maternal complications can occur, including abruptio placentae, renal failure, subcapsular hematomas, and hepatic rupture. Acute fatty liver of pregnancy is a sudden catastrophic illness occurring almost exclusively in the third trimester; microvesicular fatty infiltration of hepatocytes causes acute liver failure with coagulopathy and encephalopathy. Early diagnosis and immediate delivery are essential for maternal and fetal survival.
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PMID:Liver disease in pregnancy. 1826 10

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

Intrahepatic cholestasis of pregnancy (ICP) is characterized by maternal pruritus, and elevated serum transaminases and bile acids. Genetic defects in at least 6 canalicular transporters have been found. Association studies stress the variability of genotypes, different penetrance, and influence of environmental factors. Serum autotaxin is a sensitive, specific, and robust diagnostic marker. Elevated maternal bile acids correlate with fetal complications. Long-term sequelae for mothers include the gallstone risk and chronic liver disease. There is an association between ICP and hepatitis C. Current treatment is ursodeoxycholic acid, owing to benefits on pruritus, liver function, safety, and decreased rates of adverse effects.
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PMID:New Insights on Intrahepatic Cholestasis of Pregnancy. 2659 98