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)

Four women aged 35 to 57 years (m: 49.2) with primary biliary cirrhosis and intractable pruritus had an estimated median survival time according to Christensen between 6 and 50 months (m: 27). They were enrolled in a therapeutic trial associating plasma exchange (PE) and immunosuppressive drugs. During the first 2 months, they received prednisone 15 mg/day, cyclophosphamide 2 mg/kg BW/day and 28 PE (60 ml/kg BW). Pruritus disappeared rapidly. After 2 months, mean levels of bilirubin, alkaline phosphatases, IgM and anti-mitochondrial antibodies dropped by 27 p. 100, 47 p. 100, 50 p. 100 and 85 p. 100 respectively, whereas amino-transferase and gamma-glutamyl-transpeptidase activities were unaltered. Two patients then received supportive therapy only: one was lost to follow-up after one year, the other died 50 months later from liver failure. The third patient received PE only when intractable pruritus reappeared: anti-mitochondrial antibodies, IgM and alkaline phosphatases remained below initial values for 38 months, until successful liver transplantation was performed. The fourth patient was treated on a long term basis by PE twice a month, prednisone 10 mg/day for 3 years and cyclophosphamide 1 mg/kgBW/day for one year only. Her initial estimated survival time was 6 months, but 3 years later she developed portal hypertension; anti-mitochondrial antibody titer was between 0 and 1/80, alkaline phosphatase levels reduced by 80 p. 100 to 70 p. 100; bilirubin level up by 50 p. 100. The mean survival time for the 4 patients exceeded 34 months, results better than those obtained with other kinds of therapy.
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PMID:[Primary biliary cirrhosis: therapeutic trial using plasma exchange and immunosuppression. Preliminary results]. 324 87

A cholestatic syndrome has been reported as one of the main side effects of CyA therapy. The aim of the present study was to evaluate frequency and degree of severity of the cholestatic syndrome in a group of patients with renal transplant treated with CyA. In 55 patients we evaluated both clinical: jaundice, pruritus, presence of biliary lithiasis and biochemical parameters: total serum biliary salts (TBS), total bilirubin (TB), alkaline phosphatase (AP), gammaglutamyl transpeptidase (GGT), transaminase (AST, ALT), cholesterol (CT), triglycerides (TG), HDL-cholesterol (HDL-C) and compared them with a control group matched for sex and age. In the transplant patients significantly higher values of TBS, TB, AP (p < 0.05) were found; 55% of the patients had above mean values of at least one of the classical parameters of liver function and an higher frequency of biliary lithiasis was also found, in the absence of the classical risk factors. However, none of the patients presented severe signs of hepatic disease and to date it has never been necessary to stop treatment. In conclusion, our study shows that the dosage of CyA used at present is quite safe; however, it is necessary to monitor in these patients some parameters of liver function to prevent the minor side effects we observed from progressing into more serious damage.
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PMID:[Effects of cyclosporin A on various indices of cholestasis in kidney transplant recipients]. 856 Mar 51

The clinical, biochemical and histological characteristics in six Arab children with progressive familial intrahepatic cholestasis (PFIC) (Byler's disease) are described. The autosomal recessive mode of inheritance is established. Jaundice and pruritus were early symptoms, with onset in the 1st 3 months in all patients. Other features included growth failure, developmental delay, ataxia, areflexia, gall-stones and epistaxis. Gamma-glutamyl-transpeptidase and cholesterol were normal, but total bile acid levels were uniformly elevated in all patients. Histology showed features of hepato-canalicular cholestasis, lack of bile duct proliferation and fibrosis or cirrhosis in all patients. Five patients who were followed up were alive at a mean age of 75.8 months.
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PMID:Progressive familial intrahepatic cholestasis (Byler's disease) in Arab children. 868 10

Twenty male alcoholics (age: 40-60 years) abstinent for 2 weeks and affected by liver steatosis with positive laboratory indexes of biliary stasis (increased levels of total and free bilirubin, gamma glutamil transpeptidase and hepatic alkaline phosphatase) were treated for 3 weeks with 500 ml/day p.o. of placebo (aqua fontis) (Group I:N. 10) or sulfureous sulfate calcic water of the "Pergoli" spring-Tabiano (Parma, Italy) (Group II: N. 10). The abovementioned laboratory indexes of biliary stasis were measured in groups I and II before and after water treatments and were compared to values obtained in 10 age-matched normal male subjects. In addition, both before and after water treatments, alcoholic patients underwent a double blind test, where individual sensations of itch were scored. Finally, in order to establish the effect of water treatments on gallbladder contraction, the bidimensional areas at maximal longitudinal and transverse diameters were echographically evaluated in alcoholics before (time 0) and 20, 40 and 60 minutes after drinking in 10 minutes a 500 ml load of placebo (Group I) or Pergoli water (Group II). The results showed a significant reduction in the measured gallbladder area in group II alcoholics, but not in patients of group I. Furthermore, three weeks of treatment with Pergoli water significantly reduced to normal total and direct bilirubin levels, the enzyme markers of biliary stasis and the subjective sensation of itch. These parameters did not change in alcoholics of group I after placebo treatment. These data demonstrate that the oral administration of sulfureous sulfate calcic water Pergoli exerts a cholagogue activity in humans. The cholagogue activity together with the wellknown choleretic effect of this water argue in favor of the possibility for its use as support treatment to the therapy of biliary stasis in alcoholism.
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PMID:[Choleretic and cholagogic effect of sulphuric sulfate water from the springs of Tobiano in cholestasis in alcohol related liver diseases]. 937 33

Pruritus is a common symptom in cholestatic liver disease but is rare in chronic hepatitis C. Eight patients with chronic hepatitis C and severe pruritus were compared with regard to biochemical, serological, and histological features to eight disease controls with primary biliary cirrhosis and seven with cirrhosis due to hepatitis C. Among those with severe pruritus associated with chronic hepatitis C, serum aminotransferases were raised in all, alkaline phosphatase in four, and gamma-glutamyl-transpeptidase levels in all except one. Serum cholylglycine levels were elevated in seven of eight patients. Liver biopsies showed moderate to severe fibrosis in all patients and cirrhosis in five. Compared to control subjects with cirrhosis due to hepatitis C but no pruritus, ductopenia, and cholestatic changes were prominent, although less so than in controls with primary biliary cirrhosis. Chronic hepatitis C with moderate to severe fibrosis may result in low-grade cholestasis with pruritus, possibly in association with bile duct disappearance.
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PMID:Pruritus as a presenting symptom of chronic hepatitis C. 979 Apr 51

Progressive intrahepatic familial cholestasis (PFIC), previously called Byler's disease, is a syndrome in which children develop severe cholestasis progressing to biliary cirrhosis and chronic liver failure, usually during the first decade of life. Clinical features include jaundice, hepatomegaly, splenomegaly, growth retardation and severe pruritus. Laboratory tests demonstrate elevated bilirubin and bile acids, without an increase in serum gamma-glutamyl-transpeptidase or cholesterol. This study was performed to evaluate our experience with medical therapy as well as two types of surgical treatment used in children with PFIC, particularly partial external biliary diversion (PEBD) as an alternative method of therapy to liver transplantation (OLTx). Between 1979 and 1998 we have treated 46 children with PFIC (27 boys and 19 girls), aged 10 months to 19 yr (at the time of this study). Medical treatment with ursodeoxycholic (UDCA) was used in 39 patients for the period between 6 and 82 months. PEBD (cholecysto-jejuno-cutaneostomy) was performed in 16 patients, OLTx in eight children (including one after unsuccessful PEBD). Retrospective analysis of the clinical course and selected laboratory tests (bilirubin, ASPAT, ALAT, bile acids), and histopathological examinations were performed. Results of treatment were assessed by means of influence of the type of treatment on clinical symptoms, laboratory tests, progress of liver cirrhosis and hepatic failure, as well as physical development and survival. Medical therapy was effective in the long term in four (10%) of the patients resulting in clinical and biochemical normalization. Both surgical methods of therapy of PFIC, PEBD and OLTx, resulted in an 80% success rate and therefore should be used as complementary therapies. In patients before established liver cirrhosis, PEBD should be the first choice of treatment. Patients presenting with cirrhosis or after ineffective PEBD should qualify for OLTx. With this strategy most children with PIFC can be cured.
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PMID:Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion. 1048 83

Cholestasis, a frequent occurrence in clinical practice, can be suspected when confronted to clinical signs (pruritus and jaundice) and/or increased blood level of alkaline phosphatases, gammaglutamyl transpeptidase and/or conjugated bilirubin. This short review will deal with the management of clinical manifestations and extrahepatic complications, whatever the cause of cholestasis. The management according to etiology will be dealt with in a separated chapter.
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PMID:[Cholestasis in adults. 2. Clinical signs and symptomatic treatment]. 1700 52