Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The most frequent and important symptoms in primary biliary cirrhosis (PBC) are pruritus and jaundice. In the majority, jaundice appears within one half to two years of onset of pruritus. Xanthoma, bone change and sicca syndrome are often observed. The recent increase in number of asymptomatic PBC is the result of progagation of medical examination. In the biochemical tests, elevation of the biliary enzymes, such as ALP and gamma-GTP, is characteristic, as well as, a high level of serum IgM. Serum bilirubin values are gradually raised along with the clinical course of the disease. Positive antimitochondrial antibody (AMA) is the most valuable immunological finding for the diagnosis of PBC. Anti-pyruvate dehydrogenase complex E2 component E2 has the same significance as AMA. Diagnostic criteria si shown in this paper.
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PMID:[Primary biliary cirrhosis: symptoms, laboratory data and diagnosis]. 811 1

S-adenosyl-L-methionine (ademethionine) has been recently proposed as a therapeutic agent for the treatment of intrahepatic cholestasis (IHC), a syndrome that overlaps with many different types of liver diseases. To obtain a global assessment of the results of the therapeutic efficacy of this compound, a meta-analysis of 6 controlled clinical trials with ademethionine in the symptomatic treatment of IHC of liver diseases and pregnancy was carried out. The therapeutic response to ademethionine treatment, for 15 to 30 days, proved to be superior to placebo, as assessed by resolution of pruritus, normalisation or 50% improvement in serum total bilirubin, serum conjugated bilirubin, alanine aminotransferase, gamma-glutamyl transpeptidase and alkaline phosphatase. At present, the therapeutic effect of ademethionine should be regarded as symptomatic, but long term studies on the effect of drug administration on the course of the disease and survival are being performed.
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PMID:[A meta-analysis of therapeutic trials with ademetionine in the treatment of intrahepatic cholestasis]. 811 21

Primary sclerosing cholangitis is a cholestatic disease of the liver characterized by progressive fibrotic inflammation and obliteration of the extra- and/or intrahepatic bile ducts. There is no effective therapy. We, therefore, studied the safety and efficacy of ursodeoxycholic acid in patients with primary sclerosing cholangitis with or without additional ulcerative colitis. In a 1-year ursodeoxycholic acid treatment period, which preceded the controlled study period, ursodeoxycholic acid was well tolerated in 22 of 24 patients with ulcerative colitis and in all three patients without ulcerative colitis. In two patients with ulcerative colitis the dose of 750 mg ursodeoxycholic acid/day led to diarrhea, but following reduction of the dose to 500 and 250 mg/day ursodeoxycholic acid was well tolerated. After 1 year of ursodeoxycholic acid treatment, 20 patients were randomly assigned to receive either ursodeoxycholic acid 750 mg/day or placebo. All of them finished a double-blind, placebo-controlled study period. During ursodeoxycholic acid treatment, the liver enzymes improved markedly. The difference in alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase and gamma-glutamyltransferase between the placebo and ursodeoxycholic acid group was significant (p < 0.05). Following ursodeoxycholic acid treatment, pruritus and fatigue improved in half of the patients but the difference between the placebo and ursodeoxycholic acid group was not significant. According to the ethical guidelines, after 3 months of placebo treatment, the controlled study had to be discontinued because of a more than twofold increase of serum transaminases in 8/10 patients on placebo. After the end of the controlled study, all patients were continuously treated with ursodeoxycholic acid for up to 4 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Effect of ursodeoxycholic acid on liver and bile duct disease in primary sclerosing cholangitis. A 3-year pilot study with a placebo-controlled study period. 820 Dec 24

To evaluate the effect of ursodeoxycholic acid (UDCA) treatment according to the severity of primary biliary cirrhosis, a long-term prospective open trial in 54 consecutive PBC patients, 19 with histological stage I-II, 24 stage III, and 11 stage IV was carried out. UDCA was administered at a dosage of 250 mg twice a day. Clinical and biochemical assessment (AST, ALT, alkaline phosphatase, GGT, bilirubin) were done initially and every six months. Serum hyaluronate (HY) and type III procollagen amino propeptide (PIIIP) were also evaluated, as they are considered markers of fibrosis and prognosis. All patients were followed-up for at least two years (24-36 months); results were analyzed at 24 months after treatment. The composite pruritus score failed to show significant changes during UDCA treatment, while intensity score demonstrated a significant reduction from the 6th month. Patients with histological stage I-II disease had a significant decrease of liver enzymes (AST, ALT, alkaline phosphatase, GGT) after six months and maintained the levels up to 24 months. The patients with histological stage III disease showed a significant decrease of AST, ALT, alkaline phosphatase (but not GGT) up to month 18; subsequently AST and ALT serum levels increased, reaching values comparable to baseline by 24 months. In patients with histological stage IV disease no significant change in liver enzymes was observed during the follow-up. HY and PIIIP serum levels failed to show significant changes during UDCA treatment in the three groups of patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Different response to ursodeoxycholic acid (UDCA) in primary biliary cirrhosis according to severity of disease. 828 73

The administration of ursodeoxycholic acid, a hydrophilic bile acid not hepatotoxic to humans, has been suggested for treatment of primary biliary cirrhosis to improve cholestasis and reduce hepatocellular damage. Efficacy of treatment has been studied mainly in patients with asymptomatic or early-stage disease. In January 1988, to establish the efficacy and safety of ursodeoxycholic acid in a population with more severe disease, we started a multicenter, double-blind, placebo-controlled trial in patients with symptomatic disease, that is, with pruritus or serum bilirubin exceeding 2 mg/dl. Forty-four patients were assigned to ursodeoxycholic acid, 500 mg daily (corresponding to about 8.7 mg/kg body weight in these patients), and 44 to a placebo. As planned at the beginning of the study, a preliminary analysis was performed when all patients had been followed for at least 6 months (33 patients up to 12 months). Pruritus, self-evaluated by the patients, and cholestyramine consumption, as recorded in a diary, decreased significantly (p < 0.01) in both groups. In patients who initially had abnormal levels, serum bilirubin decreased significantly (p < 0.05) in the ursodeoxycholic acid group compared to placebo. After 6 months the following were also significantly better in the ursodeoxycholic acid than in the placebo group: a composite weighted biochemical index taking into account the changes in serum bilirubin, alkaline phosphatase, gamma-GT and AST (p < 0.001); serum prealbumin (p < 0.05); IgG (p < 0.01) and IgM (p < 0.01) levels.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Ursodeoxycholic acid for symptomatic primary biliary cirrhosis. Preliminary analysis of a double-blind multicenter trial. Italian Multicenter Group for the Study of UDCA in PBC. 831 62

Pregnancy is very uncommon in patients with primary biliary cirrhosis (PBC) and only few reports exist about pregnancy and PBC. However, no data are available on therapy and potential risks of treatment with ursodeoxycholic acid (UDCA) in PBC, especially in the first trimester of pregnancy. Furthermore, it is not known, whether UDCA is secreted into the breast milk during lactation. We report a 41 year old patient with the diagnosis of PBC stage III, who had been treated with UDCA (750 mg/day) for three years. At the time of diagnosis of pregnancy (5th gestational week), UDCA was withdrawn. Within nine days, severe pruritus developed, alkaline phosphatase and gamma-glutamyltransferase increased. UDCA was administered again (750 mg/day). The pruritus disappeared completely within one week. Liver enzymes decreased to baseline values and remained stable throughout the remainder of the pregnancy. No drug-related side effects were observed. Caesarean section for placental insufficiency unrelated to PBC was performed at the 34th week of pregnancy. The newborn thrived normally during a follow-up period of six months. When the patient's breast milk was analyzed by high pressure liquid chromatography, cholic acid, deoxycholic acid and lithocholic acid, but not UDCA were detected in trace amounts. It is concluded that UDCA therapy in PBC may be continued in the early pregnancy and during the breast feeding period. UDCA may be effective for the prevention of cholestasis in PBC during pregnancy.
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PMID:-Therapy with ursodeoxycholic acid in primary biliary cirrhosis in pregnancy-. 865 Sep 73

A case of primary biliary cirrhosis (PBC) associated with idiopathic thrombocytopenic purpura (ITP) is reported. The patient is a 59-year-old man. When he was 49 years old, he was diagnosed with ITP and received steroid therapy that successfully increased platelet numbers. However, the steroid therapy failed to normalize the elevated gamma-glutamyl transpeptidase. Ten years after this episode, he suffered from general itching and malaise and exhibited a gradual increase of serum biliary enzyme levels. Immunologically, IgM was increased and anti-mitochondrial antibody was positive. Histological findings of liver needle biopsy showed chronic non-suppurative destructive cholangitis, confirming the diagnosis of PBC. To date, very few PBC cases associated with ITP have been reported. Our case is the second one in Japan. PBC and ITP in our patient seemed to develop simultaneously, but the effect of steroid therapy on the two conditions was different. This result suggests that the autoimmune process may have been different in PBC and ITP in the present patient.
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PMID:Primary biliary cirrhosis associated with idiopathic thrombocytopenic purpura. 868 May 53

Progressive familial intrahepatic cholestasis (PFIC) is a lethal inherited childhood cholestasis of hepatocellular origin. Different subtypes of PFIC have been described according to serum gamma-glutamyl transpeptidase (GGT) activity. There is currently no effective medical therapy available for children with PFIC. We report on 39 patients with PFIC who received ursodeoxycholic acid (UDCA) orally (20-30 mg/kg b.w./day) for a period of 2 to 4 years. Group 1 (n = 26) consisted of children with normal GGT activity, and group 2 (n = 13) of children with high GGT activity. Within group 1, liver tests normalized in 11 children, improved in 5, and stabilized or worsened in 10. Within group 2, liver tests normalized in six children, improved in four, and stabilized or worsened in three. Improvement of parameters was associated with an enrichment of the circulating pool of bile acids with UDCA. Hepatosplenomegaly and pruritus disappeared or diminished in children in whom liver tests normalized. In nine of these children, liver tests worsened and normalized again after stopping and restarting UDCA. Liver histology assessed in four children after normalization of liver tests and 2 years of treatment showed a decrease in fibrosis. We conclude that UDCA should be considered in the initial therapeutic management of children with PFIC, because it appears effective in resolving or improving the liver function and the clinical status of a fair proportion of children. Chronic UDCA therapy might thus avoid the need for liver transplantation in some children with PFIC.
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PMID:Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis. 904 90

Malignant diseases may cause cholestatic jaundice through either main bile duct obstruction or widespread hepatic metastasis. Renal cell carcinoma (hypernephroma, RCC) can cause a variety of paraneoplastic manifestations which can be the main presenting symptoms. Cholestasis, as a paraneoplastic syndrome, has been well described in patients with malignant lymphohyperplastic diseases. Non-metastatic nephrogenic hepatic dysfunction syndrome without jaundice has often been described in patients with hypernephroma (Stauffer's syndrome). Paraneoplastic cholestatic jaundice has not yet been described. We report, for the first time, two patients who presented with pruritus and cholestatic jaundice. During the diagnostic work-up, RCC was diagnosed. The renal tumour was an unexpected finding during computed tomographic (CT) scan. No clinical manifestations of hypernephroma, short of microscopic haematuria, were detected. Conjugated bilirubin, alkaline phosphatase and gamma-glutamyltranspeptidase were markedly increased. No hepatic metastasis or main bile duct obstruction were detected by appropriate investigations. After radical nephrectomy, liver abnormalities disappeared rapidly. We conclude that RCC should be included among neoplasms causing not only anicteric intrahepatic cholestasis but also frank jaundice as part of a paraneoplastic syndrome. The differential diagnosis from hepatic metastasis, main bile duct obstruction or other causes of jaundice is of clinical importance and of prognostic value. Patients with unexplained cholestasis should be investigated for malignant diseases including hypernephroma.
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PMID:Cholestatic jaundice as a paraneoplastic manifestation of renal cell carcinoma. 909 37

Intrahepatic cholestasis of pregnancy (ICP) is a syndrome usually manifesting during the third trimester of pregnancy and disappearing after delivery. Multiple factors seem to be involved in pathogenesis of the syndrome; however, ICP appears to take place in women congenitally hypersensitive to estrogens. Typical is pruritus, which may be followed by jaundice and associated with other less common symptoms. The biochemical parameters are characteristically altered: an increase in the levels of aminotransferases (AST, ALT), total bile acids and alkaline phosphatase is observed; while serum GGT are normal. Maternal prognosis is benign. By contrast, a higher risk of acute fetal distress and prematurity has been reported. Various drugs are used in the treatment of ICP. We present the case of a patient treated with S-adenosyl-L-methionine (SaMe). SaMe therapy has proved to be effective in improving the altered biochemical parameters, whose normalization was obtained before delivery.
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PMID:[Intrahepatic cholestasis in pregnancy]. 911 20


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