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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Problems of pregnancy in patients with liver disease are discussed. The effects of pregnancy on the disease course are generally limited to triggering of intensifiying icterus and pruritus; intrahepatic cholestasis may also occur. Increased incidences of miscarriage and prematurity have been reported in patients with liver cirrhosis, chronic hepatitis, cholestasis, and Dubin-Johnson syndrome, which is hereditary, and viral hepatitis in early pregnancy (increased incidence of chromosome abnormalities). Liver diseases constitute a relative indication for abortion, depending on the general state of the mother's health and her desire for the child. Problems of diagnosis and treatment are also considered.
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PMID:[Pregnancy in liver diseases]. 112 34

Perinatal transmission of hepatitis B (HB) virus occurs if the mother has had acute HB infection during late pregnancy or in the first months postpartum, or if the mother is a chronic HB antigen carrier. Vertical transmission from chronic carriers exceeds 90% and accounts for up to 40% of the world chronic carriers in endemic areas. Hepatitis in pregnancy is not associated with increased abortion rate, stillbirth, or congenital malformation. However, prematurity seems to be increased if hepatitis is acquired in the last trimester. Sixty percent of pregnant women who acquire acute HB infections at or near delivery will transmit the HB virus to their offspring. Although infection is rarely symptomatic, 70 to 90% of the babies will remain chronically infected into adult life and be prone to cirrhosis and hepatocellular carcinoma. Because of such high risks and the safety and efficacy (seroconversion 90 to 100%) of HB vaccine in preventing HB infection, it is recommended that HB vaccine be given to pregnant women at high risk. However, its safety to the fetus is not well documented. Only one human study reports the safety and efficacy of Heptavax, but only when administered (to 72 pregnant women) in the last trimester of pregnancy when embryopathy cannot occur. We report pregnancy outcome in ten women, mostly health care personnel or patients traveling to endemic areas exposed to the vaccine during the first trimester of pregnancy. No congenital abnormalities were observed and all the infants are physically and developmentally normal for their ages at 2 to 12 months. Although small, this cohort suggests safe use of the vaccine in early pregnancy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Hepatitis B vaccine in pregnancy: maternal and fetal safety. 182 84

The histologic features of liver biopsies from 20 children treated with total parenteral nutrition (TPN) are presented. All the children received TPN for no less than 2 wk. Conditions that led clinicians to use this form of treatment included prematurity, sepsis, and gastrointestinal surgical procedures. Fourteen children had a history of prematurity; in nine the birth weight was between 640 and 1300 g. Gestational age and birth weight were not available in five and 11 children, respectively. Ten of the 20 children died. Our findings suggest that the morphologic features observed in the liver can be correlated with the duration of TPN. Thus, we propose a time table of hepatic histologic findings beginning with cholestasis and culminating in cirrhosis.
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PMID:Total parenteral nutrition: a histopathologic analysis of the liver changes in 20 children. 800 42

To evaluate the incidence, clinical course, and possible risk factors of cholestasis in very low-birth-weight infants. A retrospective study of 143 very low-birth-weight infants was performed. Cholestasis was defined as direct-reacting bilirubin > 2 mg/dL for more than 14 days. The clinical course of cholestasis was described, and perinatal risk factors were evaluated for associations with the development and severity of cholestasis. Cholestasis was present in 31 infants (21.7%). The mean (SD) age of onset was 30.3(15.3) days after birth or 26.0 (15.6) days after receiving parenteral nutrition, and the mean (SD) duration was 77.1 (33.8) days. In half of the cholestatic infants, bilirubin continued to rise after discontinuing parenteral nutrition. One infant developed signs of liver cirrhosis and died, two infants died with progressive cholestasis, while the other 28 patients recovered. Analysis of risk factors revealed that birthweight and duration of fasting significantly correlated with the development of cholestasis, and that sepsis significantly influenced the severity of cholestasis. Cholestasis is a common complication of extreme prematurity. The clinical course seems benign but long-term sequelae are unknown. Immature liver function and absence of stimuli for intestinal motility and hormonal secretion predispose to decreased bile flow, while sepsis further impairs hepatic ductular secretion and aggravates cholestasis.
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PMID:Risk factors of cholestasis in very low-birth-weight infants. 885 50

Total parenteral nutrition (TPN)-induced liver disease develops in 40-60% of infants who require long-term TPN for intestinal failure. The clinical spectrum includes cholestasis, cholelithiasis, hepatic fibrosis with progression to biliary cirrhosis, and the development of portal hypertension and liver failure in a significant number of children who are totally parenterally fed. The pathogenesis is multifactorial and is related to prematurity, low birth weight, and duration of TPN. The degree and severity of the liver disease is related to recurrent sepsis including catheter sepsis, bacterial translocation, and cholangitis. Lack of enteral feeding leading to reduced gut hormone secretion, reduction of bile flow, and biliary stasis may be important mechanisms in the development of cholestasis, biliary sludge, and cholelithiasis. Although it is unlikely that modern TPN solutions have a major role in the etiology of TPN liver disease, manganese toxicity recently has been recognized in children with hepatic dysfunction on TPN. Although there is a definite relationship with the degree of manganese toxicity and hepatic decompensation, it is not yet clear whether this is a primary mechanism or whether the high levels are related to reduced biliary excretion of manganese. The management strategies for the prevention of TPN-induced liver disease include early enteral feeding, a multidisciplinary approach to the management of parenteral nutrition, and aseptic catheter techniques to reduce sepsis. The administration of ursodeoxycholic acid may improve bile flow and reduce gall bladder and intestinal stasis. As survival from isolated intestinal transplantation improves, this therapeutic option should be considered before TPN liver disease becomes irreversible and combined liver and small bowel transplantation is required.
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PMID:Liver complications of pediatric parenteral nutrition--epidemiology. 943 2

Intestinal failure-associated liver disease develops in 40% to 60% of infants who require long-term total parenteral nutrition (TPN) for intestinal failure and 15% to 40% of adults on home parenteral nutrition. The clinical spectrum includes hepatic steatosis, cholestasis, cholelithiasis, and hepatic fibrosis. Progression to biliary cirrhosis and the development of portal hypertension and liver failure occurs in a minority but is more common in infants and neonates than in adults. The pathogenesis is multifactorial. In infants it is related to prematurity, low birth weight, duration of PN, short bowel syndrome requiring multiple laparotomies, and recurrent sepsis. Other important mechanisms include lack of enteral feeding, which leads to reduced gut hormone secretion; reduction of bile flow and biliary stasis, which leads to the development of cholestasis; and biliary sludge and gallstones, which exacerbate hepatic dysfunction. In adults, IFALD is less common and related to age, length of time on PN, total caloric intake, and lipid or glucose overload. In preterm infants, a deficiency of taurine or cysteine may play a role, whereas in both adults and children, choline deficiency may exacerbate IFALD. Lipid emulsions, choline deficiency, and manganese toxicity are associated with both hepatic steatosis and cholestasis in adults and children. Management strategies for the prevention of intestinal failure-induced liver disease include early enteral feeding, a multidisciplinary approach to the management of parenteral nutrition, and aseptic catheter techniques to reduce sepsis. The addition of choline, taurine, and cysteine to PN solutions may also play a role. Oral administration of ursodeoxycholic acid may improve bile flow and reduce gallbladder stasis. Survival after either isolated small bowel or combined liver and small bowel transplantation is approximately 50% at 5 years, making this an acceptable therapeutic option in adults and children with irreversible liver and intestinal failure.
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PMID:Intestinal failure-associated liver disease: what do we know today? 1647 75

ABCB4 (MDR3), a lipid translocator, moves phosphatidylcholine from the inner to the outer leaflet of the canalicular membrane. Genetic mutations of ABCB4 lead to three distinct but related hepatobiliary diseases. Progressive familial intrahepatic cholestasis (PFIC) type 3 is a chronic cholestatic syndrome characterized by a markedly elevated gamma-glutamyltranspeptidase. Patients present with jaundice, pruritus, and hepatosplenomegaly. Periportal inflammation progresses to biliary cirrhosis and causes portal hypertension. Ursodeoxycholic acid (UDCA) normalizes liver function tests in approximately one half of treated PFIC type 3 patients. Partial responders or nonresponders eventually will require liver transplantation. Gallstone patients with ABCB4 mutations may have low phospholipid-associated cholelithiasis syndrome, characterized by cholesterol gallstones and intrahepatic microlithiasis, along with recurrent biliary symptoms, despite cholecystectomy. Patients with ABCB4 mutations also may develop intrahepatic brown pigment stones. UDCA may improve biliary symptoms even before the dissolution of stones occurs. Additional therapies such as farnesoid X receptor ligands/agonists and benzfibrates show future therapeutic promise. Intrahepatic cholestasis of pregnancy affects pregnant women with abnormal ABCB4. These women suffer from disabling pruritus and also may experience steatorrhea. Fetuses are at high risk for prematurity and stillbirths. The definitive treatment is delivery of the baby. In the interim, limited fat intake, fat-soluble vitamin supplementation, and UDCA with or without S-adenosylmethionine can provide symptomatic relief. Additional hepatobiliary diseases related to ABCB4 mutations are likely to be identified. This may result in the discovery of additional therapies for PFIC type 3, gallstones, and intrahepatic cholestasis of pregnancy.
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PMID:The Multiple Facets of ABCB4 (MDR3) Deficiency. 1822 10

As the treatment of cirrhosis improves, pregnancy in patients with cirrhosis is likely to become more common. Although maternal and fetal mortality is expected to similarly improve, pregnant patients with cirrhosis face unique risks. These include higher rates of spontaneous abortion and prematurity and a potential for life-threatening variceal hemorrhage, hepatic decompensation, splenic artery aneurysm rupture, and postpartum hemorrhage. Pregnancy outcome may be influenced by the underlying etiology of liver disease, as in viral and autoimmune hepatitis. Medications also impact the course of pregnancy, and must be tailored appropriately during this time.
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PMID:Pregnancy and cirrhosis. 1866 64

Parenteral nutrition liver disease (PNLD) develops in 40-60% of infants who require long-term PN for intestinal failure. The clinical spectrum includes hepatic steatosis, cholestasis, cholelithiasis, and hepatic fibrosis. Progression to biliary cirrhosis and the development of portal hypertension and liver failure occurs in a minority who require combined liver and intestinal transplantation. The pathogenesis is multifactorial and is related to prematurity, low birth weight, duration of PN, short bowel syndrome requiring multiple laparotomies and recurrent sepsis. Other important mechanisms include lack of enteral feeding which leads to reduced gut hormone secretion, reduction of bile flow and biliary stasis which leads to the development of cholestasis, biliary sludge and gallstones, which exacerbate hepatic dysfunction, especially in premature neonates with immature hepatic function. The use of lipid emulsions, particularly soy bean emulsions have been associated with hepatic cholestasis in children, although there are little data now to support toxicity from other PN components. Management strategies for the prevention of parenteral nutrition liver disease include consideration of early enteral feeding, a multidisciplinary approach to the management of parenteral nutrition with a specialized nutritional care team and aseptic catheter techniques to reduce sepsis. The use of specialized lipid emulsions such as fish oil emulsions and or SMOF (Soy bean/Medium Chain Triglyceride/Olive Oil/Fish oil) improves established cholestasis and may prevent the onset. Oral administration of ursodeoxycholic acid may improve bile flow and reduce gall bladder stasis, although there is little data to suggest that prophylactic use prevents the onset of PNLD. Survival following either isolated small bowel or combined liver and small bowel transplantation is approximately 50% at 5 years making this an acceptable therapeutic option in children with irreversible liver and intestinal failure.
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PMID:Preventing parenteral nutrition liver disease. 2092 19

Patients with liver failure have menstrual cycle irregularities or amenorrhea. Liver transplantation restores menstrual pattern among women with cirrhosis in childbearing years. It is now accepted that a planned pregnancy is possible among liver transplant recipients at least 1 year after liver transplantation, with stable allograft function and under immunosuppressive regimens, to minimize the risks of preterm delivery and pregnancy-induced hypertension. After 1 year, the risk of graft loss decreases and is not related to pregnancy. It is a high-risk pregnancy which requires a specific and regular multidisciplinary joint follow-up (obstetrician, hepatologist, and anaesthesiologist), which leads in most cases to successful outcome for mother and child. But, early prevention and multidisciplinary management of the most common complications (pregnancy-induced hypertension, preeclampsia, and fetal growth restriction) is essential. The prematurity rate, maternal morbidity and mortality are higher than in the general population. Usual immunosuppressive treatments (corticoids, cyclosporine, tacrolimus, azathioprine or mycophenolate mofetil) may require dose adaptation during pregnancy. Immunosuppressive drugs are not teratogenic, but breast feeding is not allowed.
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PMID:[Liver transplantation and pregnancy]. 2096 16


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