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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Recurrent familial intrahepatic cholestasis is an autosomal recessive disorder characterized by episodes of severe pruritus and jaundice lasting for weeks to months without extrahepatic bile duct obstruction. Symptom-free intervals may last for months to years, and chronic liver damage does not develop. We recently studied four of the five patients from the Faeroe Islands described by us 30 years ago (one had recently died) and an additional five patients that were identified after the initial report. The episodes of cholestasis were more frequent and severe in patients with early onset, but tended to reduce in frequency with age. The youngest patient, aged 25 years, who had had 16 episodes each lasting about 6 months, had a liver transplant after which no further episodes were recorded (1 year after surgery). Signs of chronic liver disease were absent in all patients. The FIC1 gene was investigated for mutations in the surviving patients. A single mutation (I661T) was found on both chromosomes in all nine patients, indicating that they are genetically identical for the disease-causing defect. Nevertheless, considerable differences among patients were observed clinically.
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PMID:Recurrent familial intrahepatic cholestasis in the Faeroe Islands. Phenotypic heterogeneity but genetic homogeneity. 991 28

Progressive familial intrahepatic cholestasis (PFIC), also known as Byler disease, is an inherited cholestasis of hepatocellular origin which is characterized by cholestasis presenting often in the neonatal period leading to death due to liver failure at ages ranging from infancy to adolescence. The pattern of appearance of affected children within families is consistent with autosomal recessive inheritance. The etiology is poorly understood but several studies have recently provided support for an heterogeneity with at least three subcategories among the spectrum of PFIC. The first subtype is characterized by an early onset, often during the neonatal period, a severe pruritus, normal serum gamma-glutamyltransferase (GGT) activity and cholesterol level, high concentration of serum primary bile acids, absence or very low levels of primary bile acids, absence or very low levels of primary bile acids in bile, and absence of ductular proliferation on standard optical liver histology. Its leads to death due to liver failure within a few years, rarely after adolescence. It is possibly due to an inborn error in primary bile acid secretion and recently, a locus for this subtype has been mapped in the original Byler pedigree to 18q21-q22, the benign recurrent intrahepatic cholestasis region. In the second subtype, affected children exhibit also normal serum GGT activity and cholesterol level and absence of ductular proliferation, but have no pruritus and only traces of primary bile acids in serum. An inborn error in primary bile acid synthesis has been demonstrated in this subtype. The third subtype presents later in life, carries a higher risk of portal hypertension and gastrointestinal bleeding and ends in liver failure at a later age. It is characterized by a mild and unconstant pruritus, high GGT serum activity, moderately raised concentrations of serum primary bile acids, normal concentration of biliary primary bile acids, and ductular proliferation and inflammatory infiltrate with patency of intra and extrahepatic bile ducts. An abnormal expression of the MDR3 gene is involved. A fair proportion of children affected with all subtypes of PFIC may benefit from oral bile acid therapy. In some cases partial external biliary diversion or liver transplantation should be proposed.
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PMID:[Progressive familial intrahepatic cholestasis and hereditary anomalies lf hepatocellular metabolism of bile acids]. 1022 12

We report a case of benign recurrent intrahepatic cholestasis (BRIC) in an 11-year-old Saudi girl who developed three episodes of pruritus and jaundice at the ages of 4, 8, and 9 years. These episodes were almost stereotypic and lasted 5-8 weeks. Although she had elevated liver enzymes and serum bile acids in her blood during the attacks, they returned to normal between attacks. Thorough investigation excluded other causes of liver disease and her recurrent attacks were shortened by cholestyramine therapy. A diagnosis of BRIC should be kept in mind in patients with cholestasis.
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PMID:Benign recurrent intrahepatic cholestasis in a Saudi child. 1069 Feb 64

Recurrent familial intrahepatic cholestasis is an autosomal recessive disorder characterized by episodes of severe pruritus and jaundice lasting for weeks to months without extrahepatic bile duct obstruction. Symptom-free intervals may last for months to years, and chronic liver damage does not develop. We recently studied four of the five patients from the Faeroe Islands described by us 30 years ago (one had recently died), and a further five patients who were identified after the initial report. The episodes of cholestasis were more frequent and severe in patients with early onset, but tended to reduce in frequency with age. The youngest patient, aged 25 years, who had had 16 episodes, each lasting about six months, had a liver transplant after which no further episodes were recorded (one year after surgery). Signs of chronic liver disease were absent in all patients. The FIC1 gene was investigated for mutations in the surviving patients. A single mutation (I661T) was found on both chromosomes in all 9 patients, indicating that they are genetically identical for the disease causing defect. Nevertheless, considerable differences between patients were observed clinically.
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PMID:[Recurrent familial intrahepatic cholestasis in the Faroe Islands]. 1077 15

Greenland familial cholestasis is a severe form of intrahepatic cholestasis described among indigenous Inuit families in Greenland. Patients present with jaundice, pruritus, bleeding episodes, and steatorrhea, and die in childhood due to end-stage liver disease. We investigated the possibility that Greenland familial cholestasis is caused by a mutation in FIC1, the gene defective in patients with progressive familial intrahepatic cholestasis type 1 and many cases of benign recurrent intrahepatic cholestasis. Using single-strand conformation polymorphism analysis and sequencing of the FIC1 exons, a missense mutation, 1660 G-->A (D554N), was detected and was shown to segregate with the disease in Inuit patients from Greenland and Canada. Examination of liver specimens from 3 Inuit patients homozygous for this mutation revealed bland canalicular cholestasis and, on transmission electron microscopy, coarsely granular Byler bile, as previously described in patients with progressive familial intrahepatic cholestasis type 1. These data establish Greenland familial cholestasis as a form of progressive familial intrahepatic cholestasis type 1 and further underscore the importance of unimpeded FIC1 activity for normal bile formation.
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PMID:A missense mutation in FIC1 is associated with greenland familial cholestasis. 1109 41

Benign recurrent intrahepatic cholestasis is a rare autosomal recessive disorder characterized by repeated episodes of intense pruritus and jaundice. Patients are completely asymptomatic for months to years between symptomatic periods. We report a case of a patient with a 7-year history of benign recurrent intrahepatic cholestasis. During the follow-up period the patient has suffered three attacks of cholestasis, confirmed by biochemical tests and histological exam. Liver enzymes were normal between the cholestasis episodes. Despite multiple attacks of cholestasis, no permanent liver damage has occurred. Although the diagnosis of benign recurrent intrahepatic cholestasis is rare, it should be included in the evaluation of a patient with cholestasis. The patients should be reassured of the benign course of this disorder.
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PMID:[Benign recurrent intrahepatic cholestasis: a seven-year follow-up report]. 1146 Jun 4

When cholestatic liver disease is present, liver ultrasound should be performed to ascertain if cholestasis is extrahepatic or intrahepatic. If bile ducts appear dilated and the probability of interventional treatment is high, endoscopic retrograde cholagio-pancreatography (ERCP) or trans-hepatic cholangiography (THC) should be the next step. If the probability of interventional therapeutics is low, cholangio-MRI should be performed. Once bile duct dilation and space occupying lesions are excluded, a work up for intrahepatic cholestasis should be started. Some specific clinical situations may be helpful in the diagnostic strategy. If cholestasis occurs in the elderly, drug-induced cholestatic disease should be suspected, whereas if it occurs in young people with risk factors, cholestatic viral hepatitis is the most likely diagnosis. During the first trimester of pregnancy cholestasis may occur in hyperemesis gravidorum, and in the third trimester of gestation cholestasis of pregnancy should be suspected. A familial history of recurrent cholestasis points to benign recurrent intrahepatic cholestasis. The occurrence of intrahepatic cholestasis in a middle-aged woman is a frequent presentation of primary biliary cirrhosis, whereas primary sclerosing cholangitis should be suspected in young males with inflammatory bowel disease. The presence of vascular spider nevi, ascites, and a history of alcohol abuse should point to alcoholic hepatitis. Neonatal cholestasis syndromes include CMV, toxoplasma and rubinfections or metabolic defects such as cystic fibrosis, alpha1-antitrypsin deficiency, bile acid synthesis defects, or biliary atresia. The treatment of cholestasis should include a management of complications such as pruritus, osteopenia and correction of fat soluble vitamin deficiencies. When hepatocellular failure or portal hypertension-related complications occur, liver transplantation should be considered.
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PMID:Diagnostic and therapeutic approach to cholestatic liver disease. 1497 98

HAES 130/04 Voluven is a new coloid plasma expander with better farmacokinetic properties as other well-known HAES solutions. It's reflected with quick volume 1 effect, plato takes about 4 and its clinical effect lasts for 6 hours. Voluven is metabolised by alfa-amilase and eliminated through kidney in 24 hours. Anafilactoid reactions are very rare, pruritus appears only with extensive long- term use. Voluven interferes with haemostasis less than other HAES solutions. With its use needs for homologic blood transfusion are diminished. Activated FVIII, PTT and time for tromb formation reach the preoperative values significantly earlier when Voluven is used. Voluven does not accumulate in plasma and tissue if used frequently. That's why greater daily amounts (50-70 ml/kg/day) are recommended. Voluven is not contraindicated in renal insufficiency until there is urine production. Recent studies show that, in severe head trauma, there're less complications with sudden ICP rise, less second haemorrhagic and mechanical ventilation is abbrevated with Voluven. Greater tissue pO2 values on the first postoperative day prove better microperfussion and less oedema of endothelial cells in shock with Voluven application. Owing to these distinguished properties Voluven has a role in treatment of traumatic and haemorrhagic shock.
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PMID:[Administration of Voluven, a plasma expander, in emergency medicine]. 1501 61

Benign recurrent intrahepatic cholestasis is a rare autosomal recessive disorder characterized by repeated episodes of intense pruritus, profound elevations in serum alkaline phosphatase and bilirubin, with normal or nearly normal values for serum gamma-glutamyl transferase. Attack lasts from several weeks to months and resolve spontaneously. Between attacks patients remain asymptomatic for months to years. The disorder does not lead to progressive liver injury and is not fatal. Genetic studies have demonstrated that the disorder is the result of a mutation in ATP8BI, a gene that codes for the FIC1 (familial intrahepatic cholestasis) protein, which is also affected in other forms of familial intrahepatic cholestasis. It is believed this protein plays a role in bile acid secretion, in aminophospholid transport, and in maintaining fluidity of the cell membrane. Therapy is supportive and aimed at relieving pruritus and other complications of severe cholestasis until the episode resolves spontaneously.
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PMID:Benign recurrent intrahepatic cholestasis. 1506 97


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