Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a case series of 56 patients with essential cryoglobulinemia, 35 were followed-up for 4-13 years (mean 7 years). A membranous proliferative glomerulonephritis, which in about half the cases showed a progression to renal insufficiency, was the commonest complication, observed in more than one third of the patients. In 2 patients hepatic cirrhosis became manifest after a completely asymptomatic period and in 2 others a lymphoproliferative disease appeared 2 and 8 years after the onset of purpura. In 51% of patients the intial clinical pattern did not change. In searching for a correlation between the development of nephropathy and cryoglobulin characteristics, none was demonstrated studying the cryoglobulin level, the presence of autoantibody and the complement components.
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PMID:A long-term follow-up study in essential cryoglobulinemia. 10 49

Hepatic porphoria and histologically verified cirrhosis, teleangiectasia, purpose, papular skin lesions existing for eight years, as well as cryomacroglobulin were found in a 49 year old man. Pathogenetically the liver disease is considered to be the primary event, which was accompanied by secondary cryoglobulinemia. IgG and IgM type of thrombus in the vessels and of small granular deposits corresponding to the type of cryoprotein in the blood, were detected in the vessel walls of the upper part in the dermis and along these vessels of both purpuri and papular skin lesions. Immunoprecipitates could be revealed by FTC-labelled antihuman complement. In the skin lesions porphyrin could not be demonstrated. The investigation of cryoglobulin and of porphyrin disorders is reasonable in every patient with purpura accompanying pathological liver processes.
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PMID:[Cryomacroglobulinemic purpura with liver cirrhosis and hepatic porphyria]. 32 52

Mixed-typed cryoproteins, consisting of IgG and IgM, were demonstrated in the sera of four sisters. While the IgG component was polyclonal in every instance, in two of them the IgM component was found to be monoclonal with type chi light chains. Clinical diagnoses included the purpura-weakness-arthralgias syndrome, posthepatitis cirrhosis, congestive heart failure and mitral stenosis. The cryocrit differed in the four sisters, ranging from 3 to 16%; in addition, rheumatoid factor activity was consistently associated with both washed cryoprecipitates and their isolated IgM components. Endomembraneous deposits of IgG and IgM were revealed by immunofluorescent studies of the renal biopsy specimen from one patient. A genetic abnormality, possibly of the autosomal recessive type, is suggested in this instance of familial cryoglobulinemia.
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PMID:Cryoimmunoglobulinemia in four sisters. 41 43

Twenty-one of 30 patients with essential mixed cryoglobulinemia (EMC) had evidence of liver involvement. The liver disease was characterized by the absence of clinical symptoms, hepatosplenomegaly, mild elevation of enzymes, abnormal BSP retention and low albumin levels. Histology, available in 12 patients, showed either chronic persistent or chronic active hepatitis or liver cirrhosis; 44% of the patients had HBsAg or HBsAb in sera and/or cryoglobulins, confirming the high frequency of exposure to hepatitis B virus (HBV) infection in EMC. However, liver lesions were similar in all patients, regardless of HBV exposure. Since other factors usually associated with chronic liver diseases were absent or apparently irrelevant, it is temptative to speculate that a 'cryoglobulinemic hepatitis' may exist as a distinct syndrome. The characteristic complement profile of the patients with EMC (low CH50 and C4, normal C3PA), not related to albumin levels, can help to differentiate this disease from chronic liver disease without cryoglobulins.
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PMID:Liver involvement in essential mixed cryoglobulinemia. 54 44

Essential cryoglobulinaemia was detected in 44 out of 150 patients (29%) screened on the basis of histological confirmation of chronic inflammatory liver disease (chronic persistent or aggressive hepatitis, or cirrhosis). Cryoglobulinemia prevailed in the patients whose hepatic tissue showed more features of active inflammation; also, a female prevalence was observed. There were no correlations between cryoglobulinaemia and either HBsAg positivity or alcoholic liver disease. Mixed cryoglobulins made of heterogeneous immunoglobulins without monotypic components were mostly associated with established cirrhosis, whereas monotypic cryoglobulins were exclusively found in patients with either persistent or aggressive chronic hepatitis. Mixed cryoglobulins with a monotypic component were associated with all histological grades of liver damage. This study affords an objective evaluation of both the frequency and immunochemical features of cryoglobulins associated with chronic inflammatory liver disease.
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PMID:Incidence and immunochemical features of serum cryoglobulin in chronic liver disease. 85 82

A study of liver abnormalities in 36 patients with mixed cryoglobulinemia in the absence of underlying infectious, connective tissue, or lymphoproliferative disorders revealed clinical or biochemical evidence of liver dysfunction in 84%. Hepatomegaly was detected in 77%, splenomegaly in 54%, and abnormalities in bilirubin, alkaline phosphatase, or serum glutamic oxalacetic transaminase in 77%. Only four of the patients had overt liver disease. Of 15 biopsies from 12 patients, there was normal tissue structure in two, minimal nonspecific changes in one, portal fibrosis in three, chronic persistent hepatitis in one, chronic active hepatitis in two, chronic active hepatitis with cirrhosis in four, and postnecrotic cirrhosis in two. These findings, together with the previously reported high incidence of serologic evidence of hepatitis B virus (HBV) infection, support the view that the syndrome of purpura, arthritis, and nephritis is often a consequence of immune-complex vasculitis secondary to HBV infection.
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PMID:Liver involvement in the syndrome of mixed cryoglobulinemia. 90 Jun 72

A total 1400 hepatitis B surface antigen-positive Alaska natives, 824 men and 576 women of all ages, were followed up prospectively over a period of 7815 carrier years for the development of sequelae related to chronic hepatitis B virus infection. During the observation period, 20 cases of hepatocellular carcinoma, 14 cases of chronic active hepatitis, 8 cases of cirrhosis, and 1 case of glomerulonephritis developed in this cohort. The annual incidence of hepatocellular carcinoma was 387 per 100,000 for men and 63 per 100,000 for women. The incidence of chronic active hepatitis and cirrhosis was 193 and 107 per 100,000 in men and 158 and 95 per 100,000 in women, respectively. No cases of either essential mixed cryoglobulinemia or necrotizing vasculitis were seen. Sixty of the hepatitis B surface antigen-positive carriers died, with 13 (21.7%) of the deaths due to hepatocellular carcinoma. The leading cause of death in this group was malignant neoplasms compared with accidents in the general Alaska native population.
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PMID:Hepatitis B-related sequelae. Prospective study in 1400 hepatitis B surface antigen-positive Alaska native carriers. 215 73

Essential mixed cryoglobulinemia (EMC) is a syndrome characterized by cryoglobulinemia and clinical features including purpura, arthralgia, asthenia (Meltzer-Franklin syndrome) without evidence of any systemic disease Liver involvement in the course of EMC is described in 50-84% of patients. It consists of mild silent hepatosplenomegaly and slightly rise of serum amino transferase. Eleven patients with clinical and laboratory findings suggestive for EMC (five type II and six type III) underwent percutaneous liver biopsy to evaluate the degree of liver involvement. Two liver cirrhosis, two chronic active hepatitis, one chronic persistent hepatitis and a case of hepatic steatosis were found. A type III cryoglobulinemia was present in four of the six patients with liver involvement. All the patients were Hbs Ag negative but three of them were Hbs Ab positive. The pathogenesis of liver involvement in the course of EMC is still now uncertain. The authors believe that a previous HBV infection plays no role in the pathogenesis of EMC syndrome. This syndrome must be considered different from mixed cryoglobulinemia secondary to chronic liver disease. They suggest that liver biopsy is mandatory during the course of EMC even when clinical and laboratory data are silent.
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PMID:[Essential mixed cryoglobulinemia with liver involvement: a still open problem]. 238 52

Twelve patients with clinical and laboratory findings typical of essential mixed cryoglobulinemia, type II (EMC II) underwent multiple liver and bone marrow biopsies. In 9 of 12 cases (all hepatitis B surface antibody-negative), routine histology revealed patent infiltration of liver portal tracts, lobules and sinusoids by small lymphocytes provided with cytological characteristics closely resembling those of the LP immunocytoma of the Kiel classification. At immunophenotyping on frozen sections, these elements expressed the CD22 antigen (marker of B cells) and bore the same type of immunoglobulin (IgM/k = 8, IgM/lambda = 1) as the monotypic component in the serum. Furthermore, in 7 of 9 patients repeated bone marrow needle biopsies showed multiple foci of infiltration by plasmacytoid cells, often with paratrabecular location. In the remaining 3 cases (all hepatitis B surface and core antigen-positive), liver biopsies were consistent with a diagnosis of cirrhosis (two) or chronic active hepatitis (one). In two of them, however, Jamshidi needle biopsy evidenced bone marrow infiltrates quite similar to those observed in the other group. On the basis of these findings, the authors discuss the hypothesis that most EMC II are substained by a low-grade malignant lymphoma.
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PMID:Essential mixed cryoglobulinemia, type II: a manifestation of a low-grade malignant lymphoma? Clinical-morphological study of 12 cases with special reference to immunohistochemical findings in liver frozen sections. 312 57

Twelve HBsAg-negative patients with histologically documented cirrhosis of the liver of either alcoholic (8 of 12) or cryptogenic (4 of 12) origin underwent renal biopsy to investigate proteinuria, hematuria and/or renal failure. Immunofluorescence was positive for IgA in 2 patients with mesangiocapillary glomerulonephritis (MCGN) and could not be performed in 2 additional patients with the same diagnosis. However, in the remaining 8 patients, immunofluorescence was negative for IgA and frequently positive for C3, IgG, IgM and/or fibrinogen. These 8 patients without IgA were classified as follows: MCGN with subendothelial electron-dense deposits (2 cases), IgM-IgG cryoglobulinemia with diffuse endocapillary glomerulonephritis (1 case), membranous nephropathy (1 case), diffuse endocapillary proliferative glomerulonephritis (1 case), vasculitis with focal segmental necrotizing glomerulitis and crescentic glomerulonephritis (2 cases). These results show that cirrhosis of the liver can be associated with a wide variety of glomerular disorders. Contrary to previous belief, IgA is absent in two thirds of patients with cirrhosis and glomerulopathy. Therefore, the pathogenetic importance of IgA in the development of glomerular disease in such patients is doubtful.
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PMID:Glomerular disease in cirrhosis of the liver: low frequency of IgA deposits. 352 93


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