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)

The hepatitis C virus (HCV) is a major cause of chronic liver disease worldwide, with approximately 170-200 million people infected. The HCV virus is transmitted by blood and blood products and such transmission occurs primarily through drug use by injection, sex with an infected partner and occupational exposure. The severity of the disease varies widely from mild chronic hepatitis to cirrhosis and hepatocellular carcinoma (HCC). Nowadays, the reference treatment is combination therapy of pegylated interferon and ribavirin, which is an inosine monophosphate dehydrogenase inhibitor and immunomodulator. Efficacy of treatment in our clinical trials is 87% in patients infected by HCV genotypes 2 or 3, whereas in patients infected by HCV genotype 1 response to treatment is 66%. The current combination treatment has significant side-effects and sometimes is poorly tolerated. HCV genotypes 2 or 3 can be treated with a lower dose of ribavirin and a shorter course of therapy, 24 weeks vs 48 weeks for patients with genotype 1. There is a growing consensus that acute control of HCV infection is associated with a vigorous intrahepatic antiviral CD4+ and CD8+ T-cell response, enhanced Th1 and natural killer activity. Pretreatment genotype and response to therapy measured at weeks 12 and 24 of treatment have been identified as key determinants in decisions about continuing treatment. Elevated serum ferritin levels and hepatic iron deposition as well as hepatic steatosis and high ALT levels with chronic hepatitis C are risk factors for HCC development. Heterozygosityfor the C282Y mutation in HFE contributes to iron accumulation and fibrosis progression in chronic hepatitis C. Ribavirin could cause dose-dependent reversible haemolytic anaemia, which can be managed with dose reductions or with administration of epoetin alpha at 40,000 IU once weekly without sacrificing the optimal dosing of ribavarin. Among patients who received ribavirin alone, serum ALT levels and necroinflammatory features of liver histology were improved, whereas symptoms, HCV RNA levels and hepatic fibrosis scores were not changed significantly from baseline. For HCV-HIV co-infected patients, treatment is given when blood CD4 counts are above 350/ml and before antiretroviral (ART) treatment is needed.
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PMID:Ribavirin in the treatment of hepatitis C. 1586 84

Chronic infection with hepatitis C virus (HCV) can progress to cirrhosis, hepatocellular carcinoma, and end-stage liver disease. The current best treatment for HCV infection is combination therapy with pegylated interferon and ribavirin. Although this regimen produces sustained virologic responses (SVRs) in approximately 50% of patients, it can be associated with a potentially dose-limiting hemolytic anemia. Hemoglobin concentrations decrease mainly as a result of ribavirin-induced hemolysis, and this anemia can be problematic in patients with HCV infection, especially those who have comorbid renal or cardiovascular disorders. In general, anemia can increase the risk of morbidity and mortality, and may have negative effects on cerebral function and quality of life. Although ribavirin-associated anemia can be reversed by dose reduction or discontinuation, this approach compromises outcomes by significantly decreasing SVR rates. Recombinant human erythropoietin has been used to manage ribavirin-associated anemia but has other potential disadvantages. Viramidine, a liver-targeting prodrug of ribavirin, has the potential to maintain the virologic efficacy of ribavirin while decreasing the risk of hemolytic anemia in patients with chronic hepatitis C.
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PMID:Definition and management of anemia in patients infected with hepatitis C virus. 1662 41

Transaldolase (TALDO) deficiency is a newly recognized metabolic disease, which has been reported so far in 2 patients presenting with liver failure and cirrhosis. We report a new sibship of 4 infants born to the same consanguineous parents; all presented at birth or in the antenatal period with dysmorphic features, cutis laxa and hypertrichosis, hepatomegaly, splenomegaly, liver failure, hemolytic anemia, thrombocytopenia, and genitourinary malformations. The clinical courses were variable: the first child died of liver failure at 4 months of age; the second pregnancy was medically terminated at 28 weeks gestation because of hydrops fetalis with oligohydramnios. The third child is doing well at age 7 with liver fibrosis and mild kidney failure. The fourth child is now 21 months old and has hepatosplenomegaly, mild anemia, and thrombocytopenia. Urine assessment of polyols showed elevations of erythritol, arabitol, and ribitol consistent with TALDO deficiency. TALDO activity was undetectable in the patients' tissues, and mutation in the TALDO1 gene was found in the 4 patients.
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PMID:Transaldolase deficiency: a new cause of hydrops fetalis and neonatal multi-organ disease. 1709 51

Spur cell anemia is an acquired hemolytic anemia, which may occur in patients with severe liver dysfunction, especially in those with alcoholic cirrhosis. Recently, non-alcoholic steatohepatitis (NASH) has been highlighted as one of the causes of chronic liver diseases, which could progress to cirrhosis. Histological features of NASH are indistinguishable from those of alcoholic hepatitis. We report on a cirrhotic NASH patient who developed progressive indirect dominant jaundice and intractable hemolytic anemia during his follow-up. This report shows the first case of a cirrhotic NASH patient who died as a result of spur cell anemia.
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PMID:Spur cell anemia associated with a cirrhotic non-alcoholic steatohepatitis patient. 1753 20

A 17-year-old girl with haemolytic anaemia, parenchymal livel disease and gallbladder calculi, is reported. Kayser-Fleischer rings, transaminasaemia, deficiency of ceruloplasmin, increased cupriuria, and nodular cirrhosis of the liver, confirmed the diagnosis of Wilson's disease. Penicillamine therapy had to be interrupted a short time after it was started, because of penicillamine-induces acute psychotic episode. Zinc-sulphate has controlled Wilson's disease in the patient.
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PMID:[Wilson's disease]. 1797 25

Warm autoimmune hemolytic anemia (WAIHA), a rare disease (0.2-1 per 100,000 population), ranges from an indolent form with mild hemolysis to a life-threatening condition that necessitates transfusion of incompatible red cells. WAIHA can be either idiopathic or secondary to medications or to a lymphoproliferative disorder. We report a case of profound hemolytic anemia in a liver-transplant eligible patient who was diagnosed with cirrhosis secondary to non-alcoholic steatohepatitis (NASH). The patient initially was treated with red cell transfusion, iv immunoglobulin, and steroids. He developed acute renal failure that required dialysis. Subsequent management included plasmapheresis and rituximab therapy. The patient developed hepatorenal syndrome and died from progressive hepatic failure. To our knowledge, this is the first report of an association between NASH and WAIHA.
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PMID:Unusual warm autoimmune hemolytic anemia in non-alcoholic steatohepatitis. 1871 57

Chronic hepatitis C is associated with substantial morbidity and mortality and poses a considerable socioeconomic burden. Improved treatment regimens, including the standard of care pegylated interferon alfa and ribavirin, have increased sustained virologic response rates; however, treatment has a long duration and is often associated with adverse events that may affect adherence. The goal of therapy is viral eradication and reduced disease-related complications such as fibrosis, cirrhosis, and hepatocellular carcinoma. The clinical outcome of hepatitis C virus infection is altered with antiviral treatment, which can be influenced by host (e.g., weight, ethnicity, health) and viral (e.g., genotype, baseline viremia) factors. Overall, sustained virologic response was attained by 54-63% of patients in clinical trials treated with pegylated interferon alfa-2a or -2b and ribavirin. However, this benefit is not without risk. Interferon-induced adverse events include flu-like symptoms, bone marrow suppression, and emotional or cognitive effects, whereas hemolytic anemia accounts for most ribavirin dosage reductions. These adverse events may be ameliorated with dosage adjustments, symptom therapy, and judicious use of preventive strategies (e.g., antidepressants, hematopoietic growth factors). Appropriate management of adverse events can increase treatment adherence, thereby enhancing outcomes and improving quality of life. Pharmacists are in an ideal position to improve the treatment of patients with chronic hepatitis C by providing education about the disease and its treatments and associated adverse events and by emphasizing the importance of treatment adherence for successful outcomes.
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PMID:Treatment options for patients with hepatitis C: role of pharmacists in optimizing treatment response and managing adverse events. 1875 86

Viral hepatitis continues to be a relevant topic for haemodialysis centres, although the number of infected dialysis patients is declining in most countries. Chronic hepatitis B and C lead to detrimental complications such as liver cirrhosis and hepatocellular carcinoma. These complications can be avoided by successful antiviral treatment. In individuals with normal renal function, drug therapy of chronic hepatitis B is evolving quickly. Today there are several options but no agreed standard therapy. In the absence of renal failure, chronic hepatitis C should be treated with a combination of pegylated interferon-alpha and ribavirin. For both infections, there is no general indication to treat all patients; several criteria can be used to predict benefits and downsides. Chronic renal failure severely alters immune function, particularly activation of T lymphocytes and cytokine production by mononuclear cells. Aging further influences the immune system with deviation of T-lymphocyte differentiation. Both effects seem to act additively, leaving the elderly haemodialysis patient with extensive immune dysfunction. While these effects do not put the patient at risk of opportunistic infection, they do have a relevant effect on the clinical course of viral hepatitis. Haemodialysis patients infected with hepatitis B manifest a subclinical, often anicteric disease, and at least 60% of the infections become chronic. These patients usually do not fulfil the criteria for successful antiviral treatment, since they have normal or slightly elevated liver enzyme levels and few histological signs of liver inflammation. In addition, the prognosis in terms of cirrhosis and hepatocellular carcinoma might be more favourable than in individuals with normal renal function. The former standard treatment of chronic hepatitis B with interferon-alpha or its derivate pegylated interferon was badly tolerated in dialysis patients and associated with low efficacy. Indeed, prior to the advent of nucleoside analogues there was a clear recommendation not to treat chronic hepatitis B infection in all except a few dialysis patients. However, the newer treatment options appear to work well. In particular, there is growing evidence for the effectiveness and tolerability of lamivudine in dialysis patients, including the elderly. Use of adefovir and entecavir has also been reported in a few cases. At present, while we still do not recommend treatment, therapy with nucleoside analogues might be an option in selected patients, for example, those planning renal transplantation. The major effort against hepatitis B should be directed at vaccination and hygienic precautions to prevent the infection.Treatment of hepatitis C in patients undergoing haemodialysis is also limited by the poor tolerability of interferons. Ribavirin is contraindicated because of severe haemolytic anaemia, although a few studies have attempted to manage this with administration of high doses of erythropoetin. Those patients who complete the full course of interferon therapy may expect sustained viral responses comparable with healthy individuals, but in most trials, 30-50% of patients were forced to interrupt treatment because of adverse effects. There is no general indication to treat chronic hepatitis C in haemodialysis patients. Arguments in favour of treatment include elevated liver enzymes, histological signs of relevant liver inflammation, younger age, a virus genotype other than 1 and planned renal transplantation.
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PMID:Viral hepatitis in elderly haemodialysis patients: current prevention and management strategies. 1880 7

Here, we report a case of a 17-year old female with Wilson's disease presenting with progressive Coombs' negative hemolytic anemia and hepatic cirrhosis who was treated with one session of therapeutic plasma exchange (TPE) and clinically improved. In clinical situations where multiple sessions of TPE may not be possible, the use of a single session of TPE in conjunction with conventional therapy may be of benefit in preventing further clinical deterioration.
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PMID:Clinical improvement of a patient with severe Wilson's disease after a single session of therapeutic plasma exchange. 1915 72

Here we report a 33-years-old woman with hereditary spherocytosis and hemochromatosis due to homozygosity for the C282Y mutation of the HFE gene. The coinheritance of both conditions led to severe iron overload and liver cirrhosis at young age. The patient was treated by repeated phlebotomy, and reversibility of cirrhosis was documented by transient elastography. This report discusses the pathophysiology of iron accumulation in patients with hemolytic anemia combined with HFE C282Y homozygosity. The case indicates that patients with hematological disorders characterized by increased erythropoetic activity should be screened for HFE mutations.
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PMID:Coinheritance of hereditary spherocytosis and reversibility of cirrhosis in a young female patient with hereditary hemochromatosis. 1938 Feb 92


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