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

One hundred seventy children, 16 months to 14 years of age, were intoxicated by the ingestion of rape oil denatured with aniline. The most frequent findings were fever, itch, adenopathy, hepatomegaly, and varied exanthems. A few weeks after the onset of the disease, more than a third of the patients experienced a syndrome similar to scleroderma. After more than nine months, three patients were found to be seriously impaired. Two girls died of measles contracted during the course of the intoxication. Prednisone caused dramatic and rapid symptomatic relief.
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PMID:Intoxication caused by ingestion of rape oil denatured with aniline. 661 45

A prospective study of the clinical pattern and course of primary biliary cirrhosis based on the data of 236 patients (211 females, 25 males) in an international randomized trial has been performed mainly using life-table technique. The median follow-up period has been 18 mo. After the entry into the study, at which time the median duration of symptoms was 2.1 yr, the estimated 5-yr increase in the cumulative percentage of patients was for pruritus from 75% to 95%, jaundice 59% to 82%, hepatomegaly 54% to 86%, pigmentation 54% to 85%, xanthomas 27% to 50%, GI bleeding 17% to 46%, ascites 7% to 49%, and vertebral collapse 3% to 20%. The frequency of cirrhosis increased from 30% to 82% in 4 yr. The mitochondrial antibody titer showed a high rate of progression with time. In analysis of subgroups, age, histologic stage, and particularly the serum bilirubin level were important prognostic factors. Sex, duration of symptoms, and character of first symptom or sign had no independent prognostic influence. The most important indication of seriously progressive course was rapid development of severe cholestasis independent of the histologic stage.
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PMID:Clinical pattern and course of disease in primary biliary cirrhosis based on an analysis of 236 patients. 735 46

A patient with Sulindac-induced hepatitis is presented. Sulindac was confirmed as the cause of the patient's liver disease when hepatic dysfunction abruptly recurred after an inadvertent reexposure to Sulindac and quickly resolved when the drug was withdrawn. The prompt reappearance of fever, chills, pruritus, rash, tender hepatomegaly, and abnormal liver tests after only two doses of Sulindac suggested a hypersensitivity reaction.
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PMID:Sulindac (Clinoril) hepatitis. 745 Apr 49

Whether prolonged cholestasis is followed by hepatic cirrhosis is still controversial. We have studied two unrelated children who have had cholestasis for 15 years, but neither of whom have developed cirrhosis. Both have severe growth retardation, peculiar facies, pulmonic stenosis, transitory renal tubular acidosis, and vitamin D-resistant rickets. The patients presented in infancy with hepatomegaly and direct hyperbilirubinemia; liver biopsy at that time revealed cholestasis and paucity of bile ducts. Subsequent serial liver biopsies have continued to demonstrate cholestasis, but there has been no evidence of cirrhosis. Electron microscopy has revealed swollen and blunted microvilli of the canalicular membrane of the hepatocyte. The patients have had elevated bile acids in the serum as well as a reversed ration of tri- to dihydroxy bile acids. Treatment with cholestyramine and phenobarbital has brought about symptomatic relief from severe pruritus and excoriation and has lowered the level of serum bile acids, although they are still above the normal range. These findings suggest that cholestasis accompanied by an elevated and reversed bile acid ratio does not universally cause hepatic cirrhosis.
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PMID:Intrahepatic cholestasis for 15 years without cirrhosis. 745 22

The cholangiocarcinoma is a low-prevalence neoplasia, but represents a great medical challenge in its diagnosis and treatment because of its insidious clinical manifestations. Its cellular origin has been associated to other hepatic tumors, and to multiple congenital or acquired factors (infections, drugs and other diseases). The most frequent histological type is the adenocarcinoma and the most common localization is the choledochal duct; local metastases are frequently found in autopsies. The usual clinical findings are pruritus, weight loss, anorexia, jaundice and hepatomegaly. The immunohistochemical advances related to oncogenes (ras & C-erb B-1) and tumor markers (Epidermal Growth Factor and Integrin among others) allow to make differential diagnosis with other hepatic cancers and will provide valuable information about its cell biology. The initial approach to patients with this disease is made by ultrasound (US), which can be associate to Doppler; Computed Axial Tomography and Nuclear Magnetic Resonance have similar sensitivity and specificity, so they are preferred to US in the elderly with history of weight loss. It's better to perform Percutaneous Transhepatic Cholangiography than Endoscopic Retrogrado Pancreatocholangiography when its possible because the first permits a comprehensive visualization of the biliary tree. The treatment includes resection and endoprosthesis placement. The combination of surgery, Chemotherapy and Radiotherapy allows to achieve the best survival rates, and opens a door to new strategies related to this malignancy.
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PMID:[Cholangiocarcinoma]. 856 79

Progressive familial intrahepatic cholestasis (PFIC) presents in early childhood with pruritus, jaundice, hepatomegaly, and growth failure. Medical therapy is unsuccessful, with progression from cholestasis to hepatic fibrosis, cirrhosis, and ultimately death before the age of 10 years. Because of evidence that biliary diversion can arrest or reverse progression to hepatic fibrosis, we have used partial biliary diversion (PBD) as primary therapy in PFIC, reserving orthotopic liver transplantation (OLT) for children who have progressive disease or established cirrhosis. Seventeen children with PFIC (aged 2 months to 19 years) have been treated. PBD was performed in eight cases. In these procedures, a 10-cm properistaltic jejunal segment was anastomosed to the side of the gallbladder, terminating as an end stoma for the collection and discard of bile. Eleven patients with hepatic insufficiency (or end-stage cirrhosis) received OLT using standard techniques, at the average age of 4 years. Six of the eight children treated with PBD had complete resolution of clinical symptoms and remain well 1 to 13 years postoperatively. These six patients have conjugated bilirubin values of less than 0.3 mg/dL, normal transaminases, and a serum bile salt concentration of less than 10 nmol/mL. All have had either reversal or no progression of the hepatic fibrosis. Postoperative bleeding complications occurred in two (25%), which required reoperation. One patient had an adhesive intestinal obstruction that was managed surgically 9 months postoperatively. Two patients had no benefit from PBD, and all of them had severe bridging fibrosis (1) or cirrhosis (3). These and nine others with cirrhosis at the time of presentation received orthotopic liver transplantation; of these, eight are alive (1 to 5 years postoperatively). These results show the importance of establishing a correct diagnosis in children with cholestasis. Clinical symptoms often are severe in children with PFIC before the development of irreversible hepatic fibrosis. Because several patients who appear to have been cured with PBD initially were scheduled for OLT, it is important that transplant surgeons recognize the feasibility of this approach.
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PMID:Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease). 874 12

In order to determine the frequency and characteristics of patients with liver abnormalities as the presenting manifestation of Hodgkin's disease (HD), 421 consecutive HD patients were studied. Six patients in the series (1.4%) presented with liver abnormalities that led to of a liver biopsy and the subsequent diagnosis of HD. All had fever prior to HD diagnosis, four frank jaundice, and one hepatic failure. No patient had pruritus. Moderate hepatomegaly was present in four patients. Cholestasis was observed in all cases; in most patients a moderate increase in the transaminase activity was also seen. Two patients had a mild rise in the serum LDH, four had leukopenia, and one eosinophilia. At liver histologic study, Reed-Sternberg cells were demonstrated in four patients; in the remaining two, the presence of atypical histiocytes, lymphocytes, and eosinophils was highly suggestive of HD, the latter diagnosis being confirmed by subsequent study of bone marrow and/or retroperitoneal lymphadenopathies. In three of the six patients, HD was not demonstrated in sites other than the liver. Three patients older than 60 years died shortly after HD diagnosis. By contrast, three patients younger than 40 years showed a dramatic response to chemotherapy: two of them had a further relapse, and one is considered cured after 14 years of continuous remission. Liver disease constitutes an infrequent form of HD presentation which must be included in the differential diagnosis of any patient with fever of unknown origin.
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PMID:Hodgkin's disease presenting as a cholestatic febrile illness: incidence and main characteristics in a series of 421 patients. 876 4

The aminoterminal propeptide of type III procollagen (PIIINP) and hyaluronan have previously been studied in different liver diseases. The results of these studies are controversial. The aim of the present study was to examine the relationship between PIIINP and hyaluronan levels and the clinical, biochemical and histological features of primary biliary cirrhosis (PBC) and its prognosis. Fifty-five PBC patients were studied at the time of diagnosis of PBC and were followed up for a mean of 58 months. During the follow-up period 21 patients died. In addition, 30 healthy subjects were examined in the present study. Hyaluronan and PIIINP were measured by radioimmunoassay and the levels of both PIIINP and hyaluronan were higher in PBC patients than in healthy volunteers (P < 1.8 x 10(-6) and 1.6 x 10(-9), respectively). Hyaluronan and PIIINP levels were above normal values in 82 and 84% of PBC patients, respectively. There were correlations between PIIINP and hyaluronan and the histological stage of PBC (r = 0.44, P < 0.004 and r = 0.56, P < 0.00001, respectively). The correlation between PIIINP and hyaluronan was 0.46 (P < 0.0035). In symptomatic patients, both PIIINP and hyaluronan values were higher than in controls (P < 0.002 and P < 0.006, respectively). The levels of PIIINP correlated significantly with bilirubin (r = 0.43, P < 0.006), while hyaluronan was correlated with age (r = 0.33, P < 0.015), pruritus (r = 0.32, P < 0.02), fatigue (r = 0.41, P < 0.003), hepatomegaly (r = -0.46, P < 0.0008), the presence of oesophageal varices (r = 0.34, P < 0.002), weight loss (r = 0.29, P < 0.05), bilirubin (r = 0.54, P < 0.0001), albumin (r = -0.30, P < 0.04), extent of fat excretion (r = 0.53, P < 0.009) and length of symptomatic period before diagnosis of PBC (r = 0.43, P < 0.002). Using Cox's logistic regression analysis, survival was found to be influenced by bilirubin concentration but not by hyaluronan, PIIINP, age, albumin or histological stage. Therefore, hyaluronan is a more sensitive marker for predicting advanced PBC than is PIIINP. However, neither hyaluronan nor PIIINP gave any indication of prognostic outcome.
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PMID:Aminoterminal propeptide of type III procollagen and hyaluronan in patients with primary biliary cirrhosis: markers of fibrosis in primary biliary cirrhosis. 898 19

The incidence of liver metastasis is quite frequent in patients with advanced cancer. Some patients are asintomatic, but more often a correlation can be present between the clinical observation and the anatomic and functional alteration of the liver provoked by metastasis. Hepatomegaly may cause pain, dyspnea, hiccup. Biliary obstruction generates jaundice and itching. Portal hypertension may cause ascitis, encephalopathy, varices of the esophagus. Hepatic failure may produce symptoms like sopor, dysrasic oedema, coagulation problems, jaundice. The treatment of the symptoms listed above is crucial for the quality of life of these patients, and must be the mainstay of the therapeutic approach. This paper describes the palliative treatment of the clinical complications related to liver metastasis.
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PMID:[Clinical features and symptomatic treatment of liver metastasis in the terminally ill patient]. 921 73


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