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)

Between 80 and 115 million people worldwide are chronically infected with hepatitis C virus, with 60%-90% of these being undiagnosed. Untreated chronic hepatitis C (CHC) is associated with progressive liver disease, cirrhosis, hepatocellular carcinoma and liver-related mortality. A number of extrahepatic manifestations are also reported in CHC patients, further adding to the burden of the disease. CHC also impacts patients in terms of lower health-related quality of life, higher levels of fatigue and reduced productivity. Furthermore, the later stages of disease are costly for both healthcare systems and society. Pegylated-interferon (PEG-IFN)+ribavirin (RBV), for many years the mainstay of treatment, leads to sustained virological response (SVR) in 40%-70% of patients. However, a substantial number of patients are ineligible for treatment, and many patients fail to achieve SVR with this regimen. Furthermore, PEG-IFN+RBV leads to impairment of patient-reported outcomes during treatment, and most patients suffer from adverse events, associated with poor adherence, treatment discontinuation and treatment failure. The approval of second-generation direct-acting antivirals (DAAs) has revolutionized the treatment of CHC patients. All-oral, PEG-IFN and RBV-free regimens have higher efficacy rates, shorter treatment durations, fewer adverse events, higher adherence rates and improvement in PROs from as early as Week 4, compared to PEG-IFN+RBV regimens. The aim of this article is to review the evidence for HCV infection as a systemic disease, summarizing the impact of hepatitis C and its treatments on clinical, patient and economic outcomes, with a focus on data from Asia and Japan specifically.
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PMID:The impact of hepatitis C virus outside the liver: Evidence from Asia. 2774 64

Chronic hepatitis C virus (HCV) infection is a systemic disease that leads to increased risks of cirrhosis and its complications, as well as extrahepatic disturbances, including immune-related disorders and metabolic alterations such as insulin resistance and steatosis. Recent accumulating evidence suggests that HCV infection can increase cardiovascular risk, and that viral eradication can improve cardiovascular outcomes in the clinical setting. These data are strengthened by evidence identifying potential mechanisms (in)directly linking HCV infection to vascular damage. However, the high prevalence of both HCV infection and cardiovascular alterations, as well as the presence of contrasting results not identifying any association between HCV infection and cardiovascular dysfunction, provides uncertainty about a direct association of HCV infection with cardiovascular risk. Further studies are needed to clarify definitively the role of HCV infection in cardiovascular alterations, as well as the impact of viral eradication on cardiovascular outcomes. These features are now more attractive, considering the availability of new, safe, and very effective interferon-free antiviral agents for the treatment of HCV infection. This review aims to discuss carefully available data on the relationship between HCV infection and cardiovascular risk.
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PMID:Hepatitis C virus and cardiovascular: A review. 2814 51

This review summarizes our current understanding of nonalcoholic fatty liver disease (NAFLD), a multi-factorial systemic disease resulting from a complex interaction between a specific genetic background and multiple environmental/metabolic "hits". The role of gut microbiota, lipotoxicity, inflammation and their molecular pathways is reviewed in-depth. We also discuss the epidemiology and natural history of NAFLD by pinpointing the remarkably high prevalence of NAFLD worldwide and its inherent systemic complications: hepatic (steatohepatitis, advanced fibrosis and cirrhosis), cardio-metabolic (cardiovascular disease, cardiomyopathy, arrhythmias and type 2 diabetes) and neoplastic (primary liver cancers and extra-hepatic cancers). Moreover, we critically report on the diagnostic role of non-invasive biomarkers, imaging techniques and liver biopsy, which remains the reference standard for diagnosing the disease, but cannot be proposed to all patients with suspected NAFLD. Finally, the management of NAFLD is also reviewed, by highlighting the lifestyle changes and the pharmacological options, with a focus on the innovative drugs. We conclude that the results of ongoing studies are eagerly expected to lead to introduce into the clinical arena new diagnostic and prognostic biomarkers, prevention and surveillance strategies as well as to new drugs for a tailored approach to the management of NAFLD in the individual patient.
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PMID:AISF position paper on nonalcoholic fatty liver disease (NAFLD): Updates and future directions. 2821 16

Terry's nails are a type of apparent leukonychia, characterized by ground glass opacification of nearly the entire nail, obliteration of the lunula, and a narrow band of normal, pink nail bed at the distal border. The aim of this study is to guide clinical practice by reviewing all of the data concerning Terry's nail that have become available since the original description by Terry in 1954, with particular reference to all clinical features, associated medical conditions, pathogenesis, and necessary workup. PubMed was searched using the keywords "leukonychia" and "Terry nails." Although the abnormality can occur with normal aging, Terry's nails can also be an indication of an underlying medical condition, most notably, cirrhosis, chronic renal failure, and congestive heart failure. A change in nail bed vascularity, secondary to overgrowth of connective tissue, is thought to be responsible, with nail bed biopsy revealing telangiectasias in the distal band. The differential diagnosis for Terry's nails includes half-and-half nails (Lindsay's nails), Muehrcke's nails, and true leukonychia totalis/partialis. Having the ability to delineate these nail findings can be a valuable tool in clinical practice as each entity is associated with a different set of systemic conditions. Terry's nails highlight the intimate connection between nail changes and systemic disease as well as the importance of thorough nail inspection with every physical examination.
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PMID:Terry's Nails: A Sign of Systemic Disease. 2858 75

Non-alcoholic fatty liver disease (NAFLD) includes a wide spectrum of diseases that range from simple steatosis to non-alcoholic steatohepatitis (NASH) and cirrhosis. In addition, the burden of NAFLD is rapidly growing. Previously, NAFLD was regarded as a hepatic manifestation of metabolic syndrome, which is a traditional cardiovascular disease (CVD) risk factor. However, there has been an increasing evidence that suggest NAFLD to be an independent risk factor of CVD. Therefore, currently, NAFLD should be reconsidered as not only a simple manifestation of metabolic syndrome, but also a systemic disease that contribute to CVD. There are some reasonable hypotheses about the relationship between NAFLD and CVD. Moreover, many studies have been performed to better understand this relationship. Nonetheless, the underlying mechanisms and pathogenesis of NAFLD that contribute to CVD have not yet been fully elucidated to date. This review focuses on the underlying mechanisms and relationship between NAFLD and CVD.
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PMID:[Cardiovascular Risk in Patients with Non-alcoholic Fatty Liver Disease]. 2863 1

Non-alcoholic fatty liver disease (NAFLD) is the most common form of liver disease and leading cause of cirrhosis in the United States and developed countries. NAFLD is closely associated with obesity, insulin resistance and metabolic syndrome, significantly contributing to the exacerbation of the latter. Although NAFLD represents the hepatic component of metabolic syndrome, it can also be found in patients prior to their presentation with other manifestations of the syndrome. The pathogenesis of NAFLD is complex and closely intertwined with insulin resistance and obesity. Several mechanisms are undoubtedly involved in its pathogenesis and progression. In this review, we bring together the current understanding of the pathogenesis that makes NAFLD a systemic disease.
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PMID:Non-alcoholic fatty liver disease: A sign of systemic disease. 2864 88

Death was the most important side effect of anaesthesia in dentistry. In this article we reviewed more than 20 studies with adequate data focusing on death associated with dental procedures since 1955 and found 218 deaths out of 71,435,282 patients (3 deaths per 1,000,000 persons) with the mortality rate of 1:327,684. In addition, mortality rate per million has dropped to half (6.2 per 1,000,000 vs. 3 per 1,000,000) since 1955 till the last report in 2012 without any sex predilection. In children, most cases died in the age of two to five years. Hypoxia was the most common cause of death, and cardiovascular, respiratory, and endocrine disorders, hepatic cirrhosis, septicaemia, and bacterial endocarditis were the most frequent underlying systemic disease in deceased patients. Although rare death following general anaesthesia in dentistry, is a critical side effect mostly seen in patients with compromised health condition. Therefore, appropriate case selection in regard with patients' general health status as well as standard technical and equipment conditions are mandatory to diminish the risk of death during dental anaesthesia.
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PMID:Death Rate of Dental Anaesthesia. 2876 9

Chronic hepatitis C infection is a systemic disease that leads to a high risk of cirrhosis and hepatic carcinoma, as well as extrahepatic related disorders, immune-related and metabolic alterations such as glucose metabolism impairment and steatosis, thus being a new cardio-metabolic risk factor. It has been shown that, due to chronic inflammation, HCV infection has a direct effect on the arterial wall, initiating endothelial dysfunction which is the first step in atherosclerotic processes with proatherogenic effects and numerous cardiovascular events. The recent data emphasize that HCV infection can induce insulin resistance in the liver and peripheral tissues through multiple mechanisms which interfere with insulin signaling, inducing the production of several proinflammatory cytokines, and modify the lipid metabolism with the result of hepatic steatosis, which is more pronounced in patients with HCV. The emergence of new direct acting, interferon-free antiviral treatment, leading to HCV cure in most cases with a satisfactory safety profile is, according to numerous studies, improving the glucose metabolism disorders and lowering the number of cardiovascular events in patients who obtained sustained viral response, thiugh further studies are needed to clarify definitively the role of HCV infection in cardiovascular and metabolic alterations, as well as the impact of viral eradication on cardiovascular outcomes.
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PMID:Chronic hepatitis C virus infection: a new modifiable cardio-metabolic risk factor? 2878 19

Lysosomal acid lipase deficiency (LAL-D) results in progressive microvesicular hepatosteatosis, fibrosis, cirrhosis, dyslipidemia, and vascular disease. Interventions available prior to enzyme replacement therapy development, including lipid lowering medications, splenectomy, hematopoietic stem cell and liver transplantation were unsuccessful at preventing multi-systemic disease progression, and were associated with significant morbidity and mortality. We report two sisters, diagnosed in infancy, who succumbed to LAL-D with accelerated disease progression following splenectomy and liver transplantation. The index patient died one year after hematopoietic stem cell transplant and liver transplantation. Her younger sister survived five years post liver-transplantation, complicated by intermittent, acute rejection. Typical LAL-D hepatopathology, including progressive, microvesicular steatosis, foamy macrophage aggregates, vacuolated Kupffer cells, advanced fibrosis and micronodular cirrhosis recurred in the liver allograft. She died before a second liver transplant could occur for decompensated liver failure. Neither patient received sebelipase alfa enzyme replacement therapy, human, recombinant, lysosomal acid lipase enzyme, FDA approved in 2015. Here are reviewed 18 LAL-D post-liver transplantation cases described in the literature. Multi-systemic LAL-D progression occurred in 11 patients (61%) and death in six (33%). These reports demonstrate that liver transplantation may be necessary for LAL-D-associated liver failure, but is not sufficient to prevent disease progression, or liver disease recurrence, since the pathophysiology is predominantly mediated by deficient enzyme activity in bone marrow-derived monocyte-macrophages. Enzyme replacement therapy addresses systemic disease and hepatopathology, potentially improving liver-transplantation outcomes. This is the first systematic review of liver transplantation for LAL-D, and the first account of liver allograft LAL-D-associated hepatopathology recurrence.
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PMID:Lysosomal acid lipase deficiency allograft recurrence and liver failure- clinical outcomes of 18 liver transplantation patients. 2965 41

BACKGROUND Sarcoidosis is a systemic disease that can affect any organ, including the liver. It is manifested by the presence of non-caseating granulomas within involved organs, most commonly the pulmonary, lymphatic, and hepatic system. Unlike pulmonary or lymphatic involvement, hepatic involvement is usually asymptomatic and it is underdiagnosed. Here, we report a case of a patient with a history of pulmonary sarcoidosis who developed hepatic sarcoidosis. CASE REPORT 68-year-old female with pulmonary sarcoidosis with a 2-week history of severe abdominal pain and epigastric tenderness presented to our center. Abdominal magnetic resonance imaging (MRI) demonstrated mild hepatic fibrosis and cirrhosis. A thorough workup was performed including a liver biopsy which showed chronic non-necrotizing granulomas consistent with sarcoidosis. She was started on prednisone and subsequently improved. The patient was symptom-free on follow-up 1 month later. CONCLUSIONS The majority of patients with hepatic sarcoidosis are usually asymptomatic, with only 5-30% presenting with abdominal pain, jaundice, nausea, vomiting, and hepatosplenomegaly. In rare cases, hepatic sarcoidosis can lead to cholestasis, portal hypertension, cirrhosis, or Budd-Chiari syndrome. Treatment with steroids is the mainstay of therapy; however, in severe cases, patients may require liver transplantation. This case report demonstrates that hepatic sarcoidosis is a serious condition, and if not treated, can lead to portal hypertension and cirrhosis. In patients with sarcoidosis, early detection and longitudinal follow-up is important in preventing overt liver failure.
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PMID:Hepatic Involvement in Systemic Sarcoidosis. 3030 3


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