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)

Sera from 88 patients with progressive systemic sclerosis were examined for precipitating mitochondrial antibodies using sonicated rat liver mitochondrial fraction as an antigen source in immunodiffusion. Precipitin lines indicating the presence of anti-mitochondrial antibodies (AMA) in 22 patients were detected. Only six of 22 sera had, additionally, precipitating antibodies to nuclear antigens. Standardized reference sera containing antibodies to mitochondrial antigens (M-A, M-B and M-C systems) were used to further characterize the type of mitochondrial antibodies. M-B antibody was most commonly detected (72.7%) either alone (eight patients) or in combination (eight patients) with M-A and M-C antibodies. M-A antibody was found in 12 patients (54.5%) and M-C antibody was present in three. The antigen related to M-B is DNAase and trypsin sensitive, in contrast to the resistant M-A antigen. AMA were detected in 21 of 22 patients by indirect immunofluorescence. When solid phase ELISA was used to detect AMA using mitochondrial fraction as antigen, a significant difference (P less than 0.005) was noted between sera with and without precipitating mitochondrial antibody. The antibody was frequently present in patients with progressive systemic sclerosis detected 2 or more years earlier (P less than 0.01). Three patients were found to have primary biliary cirrhosis and others had pruritus, hepatomegaly or abnormal liver function tests. The implication of the findings is discussed.
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PMID:Precipitating autoantibodies to mitochondrial proteins in progressive systemic sclerosis. 643 13

Two female patients with grade IV primary biliary cirrhosis, untractable pruritus, arthralgias and xanthomatous neuropathy in one case, underwent long term plasmapheresis for 30 months (on a continuous centrifugation cell separator) and 18 months (on capillary plasma filters). Plasma exchange were performed monthly and substitution achieved by diluted human serum albumin. No major side effect occurred. Pruritus, arthralgias and xanthomatous neuropathy greatly improved or even disappeared. The plasma levels of transaminases, albumin, globulin and clotting factors were stable, alkaline phosphatases slightly diminished. Bilirubin, after an initial fall at 6 months, increased again. Cholesterol stabilized at near normal values. Bile acids blood levels decreased in both cases.
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PMID:Treatment of intra-heptic cholestasis in primary biliary cirrhosis by long term plasmapheresis. 644 35

A survey of vitamin D status in 152 patients with chronic gastrointestinal conditions and 104 patients with chronic liver diseases is presented. Mild deficiency was common and severe deficiency, as judged by plasma 25-OHD levels less than 8 nmol/l, was encountered in every disease category tested. In the gastrointestinal disease patients, deficiency was significantly more common in patients following gastroenterostomy than other gastric surgery, in patients with active Crohn's disease than in those with inactive disease and in patients with chronic pancreatitis or pancreatic carcinoma with cholestatic features than in those without cholestatic features. Deficiency was as common in patients with Crohn's disease who had not been treated surgically as in those who had. There was no significant correlation between plasma 25-OHD levels and any laboratory index of malabsorption or malnutrition except for serum albumin in the gastric surgery patients, haemoglobin and ESR in the Crohn's disease patients and albumin and vitamin E in the group of patients with gastrointestinal disorders taken as a whole. In the chronic liver disease patients, those with late primary biliary cirrhosis had lower plasma 25-OHD levels than those with histological Stage I and II disease who all had normal levels, and those with pruritus and jaundice were more commonly severely deficient. Whatever the underlying disease process, patients with other coincidental medical conditions were much more likely to be deficient as were patients with cholestasis. Evidence of secondary hyperparathyroidism and osteomalacia on bone histology indicated the clinical relevance of the vitamin D deficiency. This study showed no relationship between abnormal plasma vitamin D binding protein levels and vitamin deficiency.
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PMID:A survey of vitamin D deficiency in gastrointestinal and liver disorders. 654

The investigators report an ultrastructural study of a case of primary biliary cirrhosis histologically in stage II (Scheuer), a rare disease which has not been investigated extensively at the ultrastructural level. Biliary canaliculi are dilated and microvilli are scarce. Large granular formations are observed in the perisinusoidal cytoplasm. The plasma membrane between adjacent hepatocytes appears convoluted. The ultrastructural alterations of the biliary canaliculi which we observed have been described in the literature in cholestatic patients and ascribed to cholestasis. Our patients, however, has never suffered episodes of jaundice or itching and has no histological signs indicating cholestasis. We suggest that these are precocious alterations which precede the appearance of clinical and histological signs of cholestasis. It is necessary to study other cases in order to evaluate the significance of the large granular formations and the changes observed in the plasma membrane.
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PMID:[Unusual ultrastructural findings in a case of asymptomatic primary biliary cirrhosis]. 671 83

This paper presents a case of a patient who developed a severe illness with marked jaundice after 1 cycle of oral contraceptives (OCs). Although effects on liver physiology are a frequent but clinically insiginificant consequence of OC use, major hepatic dysfunction with clinically apparent jaundice occurs rarely. This patient's illness was manifest initially by pruritus and later was characterized by marked jaundice with mild to moderate transaminase and alkaline phosphatase elevations. Serologic studies for hepatitis and primary biliary cirrhosis were negative, and extrahepatic biliary obstruction was excluded by cholangiography. Liver biopsy revealed cholestasis without features of hepatitis. The patient, 22 years of age, has shown gradual resolution of jaundice and pruritus and normalization of liver tests over a 12-month period. OC-induced cholestasis appears to be associated primarily with the estrogen component of OCs, although the progeston component may enhance the cholestatic effects of the estrogen. This case demonstrates that, although typically benign, OC jaundice may occasionally be manifest by a severe and lengthy clinical illness. Since patients with prior jaundice of pregnancy often experience a recurrence of cholestatic symptoms if OC use is resumed, OC use should be avoided in such cases.
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PMID:Severe and prolonged oral contraceptive jaundice. 671 53

Primary biliary cirrhosis is a disease where small intra-hepatic bile ducts are destroyed as a result of an immunological reaction, presumably by cytotoxic T-lymphocytes on biliary epithelium. Analogies can be made to the graft-versus-host reaction. Hepatic copper retention is secondary to cholestasis. A circulating mitochondrial antibody is virtually constant. A mitochondrial antibody, specific for primary biliary cirrhosis, has been identified. Liver biopsy appearances have been staged but there are difficulties because of the varying rte of evolution in different parts of the liver, the focal nature of the changes and the lack of correlation with the clinical picture. Other diagnostic criteria include female sex, raised serum alkaline phosphatase values and various associated diseases. Many patients are now diagnosed when presymptomatic and such patients, particularly with granulomas in the liver, may have a normal life expectancy. General therapeutic measures include control of pruritus and prevention of fat soluble vitamin and calcium deficiencies. There is no specific treatment but D-penicillamine is useful in the later stages and prolongs survival. The prognosis in those with progressive jaundice is very poor.
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PMID:Primary biliary cirrhosis: critical evaluation and treatment policies. 675 Aug 30

Three patients who had undergone orthrotopic liver transplantation for primary biliary cirrhosis and were being maintained on immunosuppressive therapy were investigated 31/2 to 41/2 years later because of the redevelopment of pruritus and mild jaundice. In one patient pigmentation was again evident, and all three had a rise in the titer of serum mitochondrial antibody after an initial fall. Liver histology showed features of primary biliary cirrhosis with non-suppurative destructive cholangitis, lymphoid aggregates, and increased deposition of copper-binding protein in the absence of cholestasis. None of these features was found in patients who had received grafts for other conditions and had lived for comparable periods, nor were they found in patients who had had rejection with bile-duct abnormalities. The overall findings indicate a recurrence of primary biliary cirrhosis in the donor organ.
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PMID:Recurrence of primary biliary cirrhosis after liver transplantation. 703 71

Hemodialysis with a polyacrylonitril membrane is evaluated as a possible method to alleviate pruritus accompanying severe prolonged cholestasis. To that extent results of a series of 30 dialysis sessions of a patient with advanced primary biliary cirrhosis are reported. Efficacy is measured with a pruritus score, kept by the patient herself, as well as with pre- and postdialysis plasma bile acid concentrations and bile acid concentrations in dialysates. Bile acid removal can then be calculated in three different ways: (a) From the decrease of the plasma bile acid concentration by dialysis a figure of 300 mumol bile acid removed is estimated. (b) Measurements of bile acid concentration in the dialysates results in a figure of 666 +/- (SD) 87 mumol bile acid removed. (c) From the mean pre- and postdialysis plasma bile acid concentrations together with an effective clearance rate of 8 ml/min for the bile acid mixture, calculated from ultrafiltration experiments, a removal of 589 +/- 100 mumol bile acid is calculated. These figures compare favorably with figures reported in the literature for the alternative techniques used, plasmapheresis and plasma perfusion over activated charcoal.
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PMID:Removal of bile acids from the blood by hemodialysis with a polyacrylonitril membrane: treatment of pruritus of cholestatic disease. 709 14

The conjugated cholic acid (glycocholic acid) (GC) in the serum and the conjugated sulfolithocholic acid (sulfolithoglycocholic acid (SLGC) in 35 aggressive chronic hepatitis (HCA), 22 cirrhosis, 11 primary biliary cirrhosis (CBP), 11 pruritus of pregnancy and in 20 normal controls were determined. The control group revealed a mean value of GC of 21 micrograms % with a dispersion of +/- 14 micrograms % and for SLGC of 36 micrograms % with a dispersion of +/- 9 micrograms %. The frequency of the alteration of GC and SLGC in the cases of chronic hepatitis was low (14% and 9%) as well as in the cirrhosis (23% and 14%). In the pruritus of pregnancy the values reached 64% and 45%. On the other hand, the values were significantly elevated in CBP (100% and 90%) (p.001 and p.006). The absolute values in the various pathologies revealed mean values which were different but with a very wide dispersion that prevented us from seeing any relation between those absolute values and the different diseases. The alteration in the metabolism of biliary acids in the hepatic pathology was manifested by the postprandial determination of GC and SLGC in 20 HCA in whom the frequency of abnormal values rose from 14% and 9% on fasting to 60% and 45% prandially.
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PMID:[Determination of cholic and sulfolithocholic acids in various hepatopathies]. 715 42

Four cases of chronic active hepatitis with cholestasis resembling primary biliary cirrhosis are reported. Two patients were women and two were men; their age ranged from 18 to 52 years. They had recurrent jaundice with pruritus, and, in two cases, xanthelasma or xanthomas. All patients had hyperbilirubinemia, a moderate increase in serum aspartate aminotransferase activity, an increase in serum alkaline phosphatase activity and immunoglobulins G levels. Hepatitis B surface antigen was present in one patient. Histological examination of the liver revealed active chronic hepatitis with cholestasis. Moderate doses of prednisone had no effect on clinical or biochemical signs in any of the patients.
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PMID:[Ineffectiveness of corticosteroids in cholestatic forms of chronic active hepatitis]. 718 71


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