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)

Knowledge of the anatomy and function of the nail apparatus is essential when performing the physical examination. Inspection may reveal localized nail abnormalities that should be treated, or may provide clues to an underlying systemic disease that requires further workup. Excessive keratinaceous material under the nail bed in a distal and lateral distribution should prompt an evaluation for onychomycosis. Onychomycosis may be diagnosed through potassium hydroxide examination of scrapings. If potassium hydroxide testing is negative for the condition, a nail culture or nail plate biopsy should be performed. A proliferating, erythematous, disruptive mass in the nail bed should be carefully evaluated for underlying squamous cell carcinoma. Longitudinal melanonychia (vertical nail bands) must be differentiated from subungual melanomas, which account for 50 percent of melanomas in persons with dark skin. Dystrophic longitudinal ridges and subungual hematomas are local conditions caused by trauma. Edema and erythema of the proximal and lateral nail folds are hallmark features of acute and chronic paronychia. Clubbing may suggest an underlying disease such as cirrhosis, chronic obstructive pulmonary disease, or celiac sprue. Koilonychia (spoon nail) is commonly associated with iron deficiency anemia. Splinter hemorrhages may herald endocarditis, although other causes should be considered. Beau lines can mark the onset of a severe underlying illness, whereas Muehrcke lines are associated with hypoalbuminemia. A pincer nail deformity is inherited or acquired and can be associated with beta-blocker use, psoriasis, onychomycosis, tumors of the nail apparatus, systemic lupus erythematosus, Kawasaki disease, and malignancy.
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PMID:Evaluation of nail abnormalities. 2253 87

Systemic sarcoidosis is a disease of unknown etiology, with the liver being the third most commonly affected organ. Most cases of hepatic sarcoidosis are not clinically apparent, but a few can progress to liver cirrhosis, portal hypertension and ultimately liver failure. The diagnosis of hepatic sarcoidosis is difficult, considering that no single laboratory test or radiographic finding can definitively diagnose this systemic disease. Diagnosis of hepatic sarcoidosis relies heavily on histopathologic evaluation of two or more organs, a diagnostic modality that is invasive and may not be applicable to all patients. The treatment of hepatic sarcoidosis is challenging, with no large randomized controlled trials done to date. Physicians must be aware of the complications of hepatic sarcoidosis, and must include the same in the differential diagnosis of liver cirrhosis. We present a case of hepatic sarcoidosis complicated by portal hypertension and liver cirrhosis.
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PMID:Hepatic sarcoidosis presenting as portal hypertension and liver cirrhosis: case report and review of the literature. 2267 8

End-stage liver cirrhosis is a systemic disease carrying a short-term desperate prognosis without liver transplantation. Given the discrepancy between the growing number of candidates and the limited available liver grafts, the pre-transplantation screening process has become a challenging task. Cardiopulmonary exercise testing, by measuring maximal oxygen consumption at peak exercise, provides a global integrative approach of the health status of an individual. In the setting of liver cirrhosis, decreased oxygen consumption at peak exercise may result from a combination of multiple extra-hepatic complications, including deconditioning, malnutrition-associated muscle weakness, anaemia, cirrhotic cardiomyopathy, and hepato-pulmonary syndrome for instance. In addition, oxygen consumption at peak exercise not only correlated with the severity of the liver disease, but it is also independently associated with survival following liver transplantation. The present article aims to review the numerous determinants of impaired aerobic capacity in patients with severe liver disease, and to discuss how useful is cardiopulmonary exercise testing as a critical tool in the pre-transplantation assessment of these patients.
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PMID:Response to exercise in patients with liver cirrhosis: implications for liver transplantation. 2313 95

Nonalcoholic fatty liver disease (NAFLD) affects about 20%-30% of the general population, and its clinical relevance arises from the fact that 20%-30% of these subjects develop non-alcoholic steatohepatitis (NASH), a condition at risk of cirrhosis progression. In addition NAFLD, and in particular NASH patients, are also at high risk of cardiovascular alterations, suffering overall from an increased liver and no liver-related events of risk and death. At the moment liver biopsy is the gold standard for a correct evaluation of NASH and fibrosis among NAFLD patients. However, the high and increasing prevalence of NAFLD has triggered an intensive search for alternative and non-invasive methods for evaluating disease severity. Specifically we can distinguish two main groups of non-invasive methodologies, namely 'serum markers' that use clinical and/or biochemical variables, and methodologies derived from elaboration of parameters arising from liver imaging techniques. All these tools showed encouraging results, even though their utility in clinical practice in the individual patients is still under debate. Therefore further efforts are needed in order to generate non-invasive algorithms that correctly assess liver damage in NAFLD patients. In particular, it should be interesting to perform gender-specific analysis, by combining old and new tools, with the aim to generate more accurate scores. Finally we think that non-invasive scores should not only be able to correctly classify the severity of liver disease in NAFLD patients, but also predict liver and non-liver related morbidity and mortality, further acting as time-dependent markers of liver and systemic disease activity. This review summarizes the present knowledge on noninvasive diagnosis in NAFLD patients, and suggest future directions for this complex research area.
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PMID:Non invasive indexes for the assessment of patients with non-alcoholic fatty liver disease. 2339 90

Sarcoidosis is a multi-systemic disease of unknown etiology that results in the development of non-caseating epithelioid granulomas. The liver is the third most frequently involved organ after the lymph nodes and the lungs. Most cases of liver sarcoidosis do not present with symptoms and involve minimal liver dysfunction, but some cases display progression to portal hypertension and liver cirrhosis, and finally to liver failure. The mechanism and the risk of progression in liver sarcoidosis are still unknown because of the diagnostic difficulty associated with this condition, and because follow-up examinations can only be done in an invasive manner. Here, we present an informative case of liver sarcoidosis with rapid progression of esophagogastric varices. Four months prior to the definitive diagnosis, no signs of varices were observed on endoscopy, and developmentof esophagogastric varices, rapid progression, and eventual rupture occurred in a short period of time. A liver biopsy, carried out after endoscopic sclerotherapy, revealed that granulomas primarily affected the portal area without fibrotic and cirrhotic changes, which is considered a primary cause of portal hypertension and esophagogastric varices. Following the liver biopsy, the patient was given systemic steroids and is currently receiving outpatient care. Thus, we should consider the possibility that liver sarcoidosis, even in the absence of cirrhotic changes, can cause serious events such as esophagogastric variceal rupture following rapid progression as a result of portal hypertension.
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PMID:Endoscopically proven case of rapid esophagogastric variceal progression and rupture as a result of portal hypertension with liver sarcoidosis. 2435 53

Hepatitis C virus (HCV) infected patients are known to be at risk of developing liver complications i.e. cirrhosis and liver cancer. However, the risks of morbidity and mortality are underestimated because they do not take into account non-liver consequences of chronic hepatitis C virus infection. Numerous extrahepatic manifestations have been reported in up to 74% of patients, from perceived to disabling conditions. The majority of data concern hepatitis C virus-related autoimmune and/or lymphoproliferative disorders, from mixed cryoglobulinaemia vasculitis to frank lymphomas. More recently, other hepatitis C virus-associated disorders have been reported including cardiovascular, renal, metabolic, and central nervous system diseases. This review aims to outline most of the extrahepatic manifestations that are currently being investigated, including some of autoimmune and/or lymphoproliferative nature, and others in which the role of immune mechanisms appears less clear. Beyond the liver, hepatitis C virus chronic infection should be analyzed as a multifaceted systemic disease leading to heavy direct and indirect costs. The accurate consideration of extrahepatic consequences of such a systemic infection significantly increases the weight of its pathological burden. The need for effective viral eradication measures is underlined.
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PMID:Extrahepatic manifestations of chronic hepatitis C virus infection. 2545 76

A disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS13) specifically cleaves unusually-large von Willebrand factor (VWF) multimers under high shear stress, and down-regulates VWF function to form platelet thrombi. Deficiency of plasma ADAMTS13 activity induces a life-threatening systemic disease, termed thrombotic microangiopathy (TMA) including thrombotic thrombocytopenic purpura (TTP). Children with advanced biliary cirrhosis due to congenital biliary atresia sometimes showed pathological features of TMA, with a concomitant decrease of plasma ADAMTS13 activity. Disappearance of their clinical findings of TTP after successful liver transplantation suggested that the liver is a major organ producing plasma ADAMTS13. In situ hybridization analysis showed that ADAMTS13 was produced by hepatic stellate cells. Subsequently, it was found that ADADTS13 was not merely responsible to development of TMA and TTP, but also related to some kinds of liver dysfunction after liver transplantation. Ischemia-reperfusion injury and acute rejection in liver transplant recipients were often associated with marked decrease of ADAMTS13 and concomitant formation of unusually large VWF multimers without findings of TMA/TTP. The similar phenomenon was observed also in patients who underwent hepatectomy for liver tumors. Imbalance between ADAMTS13 and VWF in the hepatic sinusoid might cause liver damage due to microcirculatory disturbance. It can be called as "local TTP like mechanism" which plays a crucial role in liver dysfunction after liver transplantation and surgery.
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PMID:Relevance of ADAMTS13 to liver transplantation and surgery. 2616 50

Hepatitis E is a systemic disease affecting the liver predominantly and caused by infection with the hepatitis E virus (HEV). HEV has marked genetic heterogeneity and is known to infect several animal species including pigs, boar, deer, mongoose, rabbit, camel, chicken, rats, ferret, bats and cutthroat trout. HEV is the sole member of the family Hepeviridae and has been divided into 2 genera: Orthohepevirus (mammalian and avian HEV) and Piscihepevirus (trout HEV). Human HEVs included within the genus Orthohepevirus are designated Orthohepevirus A (isolates from human, pig, wild boar, deer, mongoose, rabbit and camel). Hepatitis E is an important public health concern, and an estimated one-third of the world population has been infected with HEV. In recent years, autochthonous hepatitis E is recognized as a clinical problem in industrialized countries. Several animal species especially domestic swine, wild boar and wild deer are reservoirs of genotype HEV-3 and HEV-4 in these countries. Human infections occur through intake of uncooked or undercooked meat of the infected animals and pig livers or sausages made from these livers and sold in supermarkets. HEV can be transmitted through blood and blood component transfusions, and donor screening for HEV is under serious consideration. Chronic hepatitis E resulting in rapidly progressive liver cirrhosis and end-stage liver disease has been described in organ transplant patients. Ribavirin monotherapy attains sustained virological response in most patients. HEV 239 vaccine has been marketed in China and its long-term efficacy over four and a half years reported.
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PMID:Hepatitis E: an emerging global disease - from discovery towards control and cure. 2634 32

During hepatitis C virus (HCV) chronic infection, extrahepatic manifestations are frequent and polymorphous. This article reports on a large cohort of patients with HCV-related autoimmune or lymphoproliferative disorders, from mixed cryoglobulinemia vasculitis to frank lymphomas. The relationship between HCV infection and such immune-related diseases has been formally demonstrated by epidemiological, clinical, immunological and pathological data, and results of therapeutic trials. More recently, other nonliver-related HCV disorders have been reported, including cardiovascular (i.e. stroke, ischemic heart disease), renal, metabolic and central nervous system diseases. For these manifestations, most evidence comes from large epidemiological studies; there is a need for mechanistic studies and therapeutic trials for confirmation. Beyond the risk of developing liver complications, that is, cirrhosis and liver cancer, patients with HCV infection have an increased risk of morbidity and mortality related to nonliver diseases. HCV chronic infection should be analyzed as a systemic disease in which extrahepatic consequences increase the weight of its pathological burden. The need for effective viral eradication measures is underlined.
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PMID:Extrahepatic manifestations of chronic hepatitis C virus infection. 2686 98

Hepatitis C virus infection (HCV) affects approximately 170-200 million individuals globally. HCV is one of the primary causes of hepatocellular carcinoma (HCC) and cirrhosis and has been identified as the leading indication for liver transplantation in most Western countries. Because HCV is a systemic disease with hepatic, extrahepatic, economic and patient reported consequences, it is important for healthcare practitioners to understand the comprehensive and multi-faceted picture of this disease. In this context, it is important to fully appreciate the impact of HCV on the individual patient and the society. With the recent advent of the new generation of direct antiviral agents, the long standing goal of eradicating HCV in most infected patients has been accomplished. Therefore, now more than ever, it is critical to assess the total benefits of sustained virological response in a comprehensive manner. This should not be limited to the clinical benefits of HCV cure, but also to account for the improvement of patient reported health and economic outcomes of HCV cure. It is only through this comprehensive approach to HCV and its treatment that we will understand the full impact of this disease and the tremendous gains that have been achieved with the new antiviral regimens.
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PMID:Hepatitis C infection: A multi-faceted systemic disease with clinical, patient reported and economic consequences. 2764 81


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