Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cardiomyopathy is a heart muscle disease with impaired stretch response that can result in severe heart failure and sudden death. A small proportion of hepatitis C virus (HCV)-infected patients may be predisposed to develop dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM). The molecular mechanisms involved in the predisposition remain unknown due in part to the lack of information on their genetic background. Because the human leukocyte antigen (HLA) region has a pivotal role in controlling the susceptibility to HCV-induced liver disease, we hypothesized that particular HLA alleles and/or non-HLA gene alleles within the human major histocompatibility complex (MHC) genomic region might control the predisposition to HCV-associated DCM (HCV-DCM) and/or HCV-associated HCM (HCV-HCM). Here, we present mapping results of the MHC-related susceptibility gene locus for HCV-associated cardiomyopathy by analyzing microsatellite and single nucleotide polymorphism markers. To delineate the susceptibility locus, we genotyped 44 polymorphic markers scattered across the entire MHC region in a total of 59 patients (21 HCV-DCM and 38 HCV-HCM) and 120 controls. We mapped HCV-DCM susceptibility to a non-HLA gene locus spanning from NFKBIL1 to MICA gene loci within the MHC class III-class I boundary region. Our results showed that HCV-DCM was more strongly associated with alleles of the non-HLA genes rather than the HLA genes themselves. In addition, no significant association was found between the MHC markers and HCV-HCM. This marked difference in the MHC-related disease susceptibility for HCV- associated cardiomyopathy strongly suggests that the development of HCV- DCM and HCV-HCM is under the control of different pathogenic mechanisms.
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PMID:The haplotype block, NFKBIL1-ATP6V1G2-BAT1-MICB-MICA, within the class III-class I boundary region of the human major histocompatibility complex may control susceptibility to hepatitis C virus-associated dilated cardiomyopathy. 1610 31

Body fluid volume regulation by the kidney relies upon the complex interaction of numerous factors. However, in edematous disorders, extrarenal factors can override the 'innate wisdom' of the kidney. For example, in patients with cardiac failure or liver disease and in pregnant women, the normal kidney continues to retain sodium and water despite expanded blood, plasma and extracellular fluid volumes. Such fluid retention can ultimately lead to pulmonary congestion, ascites or peripheral edema. Understanding the kidney's modulation of total body sodium and water in these patients has been perplexing. Cardiac output cannot provide the sole afferent signal for the kidney to regulate sodium and water balance, as the normal kidney can retain excessive amounts of sodium and water when cardiac output is low (e.g. in low output cardiac failure) or high (e.g. cirrhosis or pregnancy). Therefore the integrity of the arterial circulation, which can be impaired either by a low cardiac output or arterial vasodilation, is an important factor in body fluid composition and volume regulation in health and disease.
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PMID:Pathophysiology of water and sodium retention: edematous states with normal kidney function. 1648 46

An acute cardiac tamponade from any cause may result in rapid deterioration of hepatic function in a previously healthy patient. We describe a case of an acute ascending aortic dissection that presented as acute hepatic failure, due to an acute cardiac tamponade and severe right heart failure. The differential diagnosis of the aetiology of acute liver failure is extensive and includes poisonings, vascular obstruction and sepsis, particularly on the background of decompensated liver disease. Many of these conditions are associated with hypotension. The acute presentation in our patient, combined with the lack of a characteristic history delayed the diagnosis of a proximal (type A) dissection with tamponade and subsequent hepatic failure. Severe right-sided heart failure as a result of conditions such as cardiac tamponade should be excluded in patients presenting with acute hepatic failure of unknown aetiology.
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PMID:Acute hepatic failure caused by an acute aortic dissection with cardiac tamponade: a case report. 1656 89

The safest place to store lipids is the white adipose tissue, but its storage capacity may become saturated resulting in excess of fat "overspilled" to non-adipose tissues. This overspill of fat occurs in apparently opposite pathological states such as lipodistrophy or obesity. When the excess of energy is redirected towards peripheral organs, their initial response is to facilitate the storage of the surplus in the form of triacylglycerol, but the limited triacylglycerol buffer capacity becomes saturated soon. Under these conditions excess of lipids enter alternative non-oxidative pathways that result in production of toxic reactive lipid species that induce organ-specific toxic responses leading to apoptosis. Reactive lipids can accumulate in non-adipose tissues of metabolically relevant organs such as pancreatic beta-cells, liver, heart and skeletal muscle leading to lipotoxicity, a process that contributes substantially to the pathophysiology of insulin resistance, type 2 diabetes, steatotic liver disease and heart failure. The effects of this lipotoxic insult can be minimised by several strategies: (a) decreased incorporation of energy, (b) a less orthodox approach such as increased adipose tissue expandability and/or (c) increased oxidation of fat in peripheral organs. Aging should be considered as physiological degenerative process potentially accelerated by concomitant lipotoxic insults. Conversely, the process of aging can sensitise cells to effects of lipid toxicity.
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PMID:Lipotoxicity, overnutrition and energy metabolism in aging. 1663 Jul 50

Portopulmonary hypertension (POPH) is regarded as a subtype of pulmonary arterial hypertension (PAH); however, established PAH therapies have not been evaluated for this condition. The current authors treated 14 patients (four male, 10 female; mean (range) age 55 (39-75) yrs) with moderate (n = 1) or severe (n = 13) POPH caused by alcoholic liver disease (n = 7), chronic viral hepatitis (n = 3), autoimmune hepatitis (n = 3), and hepatic manifestation of hereditary haemorrhagic teleangiectasia (n = 1) with oral sildenafil. Eight patients were newly started on pulmonary vasoactive treatment, while six patients were already on treatment with inhaled prostanoids (iloprost, n = 5; treprostinil, n = 1). During treatment with sildenafil, mean +/- sd 6-min walk distance increased from 312 +/- 111 m to 397 +/- 99 m after 3 months, and 407 +/- 97 m after 12 months. Mean +/- sd pro-brain natriuretic peptide levels decreased from 582 +/- 315 ng x mL(-1) to 230 +/- 278 ng x mL(-1), and to 189 +/- 274 ng x mL(-1) after 3 and 12 months, respectively. Two patients died after 1 and 2 months from liver failure and cardiac failure, respectively. There was a similar response to sildenafil treatment after 3 and 12 months in patients on monotherapy and those on combination therapy. In conclusion, sildenafil might be effective in monotherapy and in combination therapy with inhaled prostanoids in portopulmonary hypertension, leading to significant improvement by 3 months and sustained response over 12 months.
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PMID:Sildenafil treatment for portopulmonary hypertension. 1719 88

Acute renal failure (ARF) incidence varies depending on whether the intensive care unit only or also general and specialist medicine departments are considered. In some cases, however, such as after major cardiosurgical operations, ARF can occur in up to 30% of patients. Most of ARFs in intensive care units are secondary to acute tubular necrosis occurring because of a multi-organ dysfunction syndrome. Factors most often associated with acute renal damage are: advanced age, volume depletion, arterial hypotension, massive bleeding, and sepsis. ARF often leads to complications for the following pathologies: serious liver disease, pancreatitis, pre-existing renal dysfunction, great burns, and cardiosurgical and vascular operations on large vessels. Among the so-called 'iatrogenic factors', contrast media and aminoglycosides are definitely the main cause of a rapid deterioration of renal function. Mortality is low for the isolated forms of ARF,whereas it peaks to 0-80% in multi-organ failures where co-existing pathologies often dominate. The mortality rate over the past 20 years has not changed, although pharmacological supports and especially dialysis instruments have improved. Patients are now older and older, affected by multiple pathologies and with poor recovery capacity. Mortality is higher among elderly patients, while toxic forms (from contrast media or from myoglobinuria) result generally in better outcomes. Patients with acute renal damage and oliguria have a worse prognosis than non-oliguric patients. Finally, some unfavorable prognostic factors include the prolonged use of high dose inotropic drugs, mechanical ventilation, cardiac failure and a septic state.
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PMID:[Epidemiology of acute renal failure]. 1706 24

A small but significant percentage of patients with acute pancreatitis die within 2 weeks of hospitalization, usually with multiorgan system failure. To determine the effect of chronic medical comorbidities on early death, we conducted a retrospective analysis of all patients who were hospitalized in California with first-time pancreatitis between 1992 and 2002. Among 84,713 patients, 1514 (1.8%) died within 2 weeks. In a risk-adjusted multivariate model, the strongest predictors of early death were age 65 to 75 years (OR = 2.6, 95% CI: 2.2-3.1 versus <55 years), age over 75 years (OR = 5.2, 95% CI: 4.4-6.1), and the presence of either two chronic comorbid conditions (OR = 3.5, CI: 2.7-4.6) or three or more comorbidities (OR = 7.4, 95% CI: 5.7-9.5). Among the 14,280 patients younger than 55 years who had no chronic comorbid conditions, only 14 (0.1%) died in the first 14 days compared to 701 (5.9%) of 24,852 patients 64 years or older who had three or more comorbidities (RR = 29, 95% CI: 17-50). Comorbid conditions associated with early death included recent cancer, heart failure, renal disease, and liver disease. We conclude that advancing age and the number of chronic comorbid conditions are very strong predictors of early death among patients with acute pancreatitis.
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PMID:Co-morbidity is a strong predictor of early death and multi-organ system failure among patients with acute pancreatitis. 1741 10

Severe pain is highly prevalent, with rates of 40% to 70% in patients with advanced cancer, liver disease, heart failure, human immunodeficiency virus, and renal failure. Wide variations in pain assessment and reporting methods and the measurement of multiple symptoms should be addressed in future studies. Regarding psychological approaches, determining whether hypnotherapy or other individual psychotherapeutic interventions reduce pain and/or psychological distress in a palliative care population is difficult. Interest is increasing in the concept of demoralization syndromes and the role of posttraumatic stress disorder in modulating responses to pain at the end of life. We review evidence from multiple studies that the use of rehabilitative therapy improves functional status and pain control among patients with advanced cancer, and we raise the possibility that rehabilitation therapy will be helpful in patients with other advanced diseases. We summarize ongoing clinical trials of electronic order sets, clinical care pathways, and care management pathways to improve pain management in palliative care. Wagner's Chronic Illness Model provides a way of analyzing how healthcare systems can be changed to provide adequate and continuing pain management in palliative care. Much work remains to ensure that pain is recognized, treated, and monitored effectively.
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PMID:Pain and palliative medicine. 1755 79

We report here four cases of pediatric patients in whom the diagnosis of cystic fibrosis was made only after the histological examination of a liver specimen obtained by biopsy (three cases) or at autopsy (one case). There were two boys and two girls, aged 13 months to 7.5 years. None had a personal or familial history suggestive of cystic fibrosis. One patient, presenting with myocardial lesion and hepatomegaly, died of heart failure; at autopsy, the liver showed a typical aspect of focal biliary cirrhosis. In the three other cases, liver disease was the only manifestation of cystic fibrosis at the time of diagnosis. Liver biopsy examination showed focal biliary cirrhosis in one case and massive steatosis in two. In all four cases, the diagnosis was confirmed by the existence of known pathogenic mutations in the CFTR gene. The evolution was variable; one patient had progressive liver disease with severe portal hypertension after 7 years; another one had lung complications after 1 year. In conclusion, our experience recalls that the diagnosis of cystic fibrosis must be considered in children presenting with unexplained liver disease; its confirmation by molecular techniques makes it possible to set up an appropriate follow-up.
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PMID:Unexpected diagnosis of cystic fibrosis at liver biopsy: a report of four pediatric cases. 1755 56

The importance of differences in clinical characteristics between ethnic groups on operative mortality of coronary artery bypass grafting (CABG) has not been clarified. Data reporting to the California CABG outcomes reporting program is mandated under state law in California. Data from 121 hospitals in 2003 were analyzed, including clinical characteristics and predicted and observed operative mortalities in patients who underwent isolated CABG. In total 21,272 isolated CABGs were reported in 2003. Compared with Caucasians (n = 15,069), Hispanics (n = 2,561), Asians (n = 1,772), and African-Americans (n = 785) were younger, more likely to be women, and had more hypertension, diabetes, renal failure, and severe liver disease (all p values <0.05). Hispanics had more heart failure and Asians had a lower body mass index compared with Caucasians, whereas African-Americans had a higher body mass index and more peripheral artery disease and heart failure (all p values <0.05). Based on a multivariate model, the predicted operative mortality for Hispanics (3.2%), Asians (3.3%), and African-Americans (3.6%) was higher (all p values <0.001) than that of Caucasians (2.8%). However, observed operative mortality was similar across ethnic groups, although there was a trend toward higher mortality in Asians compared with Caucasians (3.5% vs 2.8%, p = 0.077). In conclusion, significant differences in risk profile for CABG exist across ethnicities. Although the predicted operative mortality for Hispanics, Asians, and African-Americans was significantly higher than that for Caucasians, there was no significant difference in the observed operative mortality.
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PMID:Clinical characteristics and 30-day mortality among Caucasians, Hispanics, Asians, And African-Americans in the 2003 California coronary artery bypass graft surgery outcomes reporting program. 1759 41


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