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 this article, as part of an evaluation of the future of medical education in California, we characterize the distribution of disease and injury in California; identify major factors that affect the epidemiology of disease and injury in California, and project the burden of disease and injury for California's population to the year 2007. Our goal is to elucidate the major causes of illness and disability at present and in the near future in order to focus state resources on the interventions likely to have the greatest impact. Data from various governmental agencies were utilized; the base year, 1993, is the most recent year with sufficient information available when this report was prepared. Several major risk factors have decreased, including smoking (30% decline from 1984 to 1993) and drinking and driving. However, hypertension prevalence has not changed, and overweight has increased dramatically. Poverty continues to burden about 15% of Californians, with poverty highest among children. During 1993, 220,271 Californians died, with 3 major causes accounting for 61% of these deaths: coronary heart disease (31%), cancer (23%), and stroke (7%). In terms of potential years of life lost (years lost before age 65), the most important causes of death in 1993 were unintentional injury (756 years lost/100,000 population), cancer (632 years), and the acquired immunodeficiency syndrome (AIDS; 491 years). Mortality rates were highest among blacks and lowest among Asians. Overall mortality in California has been declining for decades; in just 1 decade, from 1980 to 1991, mortality declined from 780 to 680 deaths per 100,000 population. Several major causes of death have declined, including coronary heart disease, stroke, unintentional injury, cirrhosis, and suicide, while others have increased, for example, chronic obstructive lung disease and diabetes mellitus. Death from AIDS increased dramatically in the past decade, but is leveling off, and death from cancer is beginning to decline. Rates for overall mortality and morbidity, and for most specific conditions, should continue to decline. A projected 28% population increase by 2007 will yield a corresponding increase in the absolute level of disease cases and death; a disproportionate increase in younger and older groups will yield increased conditions affecting young (unintentional injury, AIDS) and older (heart disease, cancer, stroke, diabetes mellitus) people. Californians should experience overall improved health in coming years, reaping benefits of reduced environmental and behavioral risk factors as well as improved medical treatment and rehabilitation. Coordinated strategies for health promotion, disease prevention, delivery of medical treatment, and rehabilitation are needed to maintain and improve present levels of health across the life span.
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PMID:Disease and injury in California with projections to the year 2007. Implications for medical education. 961 96

Cancer incidence and mortality risks were evaluated in a combined cohort of patients who were hospitalized for porphyria in Denmark (1977-1989) and Sweden (1965-1983). Patients were identified by using population-based hospitalization registries. The unique individual identification numbers of 530 patients with porphyria cutanea tarda (PCT) and 296 with acute intermittent porphyria (AIP) were linked to the nationwide cancer and death registries. Among patients with both types of porphyria, the authors found small but significantly elevated risks of all cancers combined (PCT: standardized incidence ratio (SIR) = 1.7, 95% confidence interval (CI) 1.3-2.2; AIP: SIR = 1.8, 95% CI 1.1-2.8) due to pronounced excesses of primary liver cancer (PCT: SIR = 21.2, 95% CI 8.5-43.7; AIP: SIR = 70.4, 95% CI 22.7-164.3) and moderate increases in lung cancer (PCT: SIR = 2.9, 95% CI 1.5-5.2; AIP: SIR = 2.8, 95% CI 0.3-10.2). PCT patients had a significantly increased risk of mortality from liver cirrhosis (standardized mortality ratio (SMR) = 8.4, 95% CI 3.1-18.4) or chronic obstructive pulmonary disease (SMR = 3.1, 95% CI 1.1-6.7). The increased risk of primary liver cancer and the increased risk of mortality from cirrhosis of the liver are consistent with findings from previous clinical surveys, but the new observations of excess lung cancer and chronic obstructive pulmonary disease require confirmation.
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PMID:Primary liver cancer, other malignancies, and mortality risks following porphyria: a cohort study in Denmark and Sweden. 1035 76

A 52-year-old male had fever, pleuritic chest pain, cough with purulent sputum and hemoptysis for 4 days. The patient had underlying alcoholic cardiomyopathy, cirrhosis of the liver, chronic obstructive lung disease and underwent corticosteroids therapy. Chest radiograph showed round opacities bilaterally. Legionella pneumophila serogroup 5 was identified by direct fluorescent antibody staining and culture from the sputum. Despite intravenous erythromycin and rifampin therapy, he died on the 7th hospital day. The autopsy showed bilateral pulmonary consolidation with abscess formation. Legionnaires' disease should be included in the differential diagnosis if an immunosuppressed patient presents with multilobar opacities on chest radiograph. Specific tests for Legionnaires' disease should be performed.
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PMID:Multilobar consolidation with abscess formation caused by Legionella pneumophila: an unusual chest radiographic presentation. 1049 59

The serpins are a family of proteinase inhibitors that play a central role in the control of proteolytic cascades. Their inhibitory mechanism depends on the intramolecular insertion of the reactive loop into beta-sheet A after cleavage by the target proteinase. Point mutations within the protein can allow aberrant conformational transitions characterized by beta-strand exchange between the reactive loop of one molecule and beta-sheet A of another. These loop-sheet polymers result in diseases as varied as cirrhosis, emphysema, angio-oedema, and thrombosis, and we recently have shown that they underlie an early-onset dementia. We report here the biochemical characteristics and crystal structure of a naturally occurring variant (Leu-55-Pro) of the plasma serpin alpha(1)-antichymotrypsin trapped as an inactive intermediate. The structure demonstrates a serpin configuration with partial insertion of the reactive loop into beta-sheet A. The lower part of the sheet is filled by the last turn of F-helix and the loop that links it to s3A. This conformation matches that of proposed intermediates on the pathway to complex and polymer formation in the serpins. In particular, this intermediate, along with the latent and polymerized conformations, explains the loss of activity of plasma alpha(1)-antichymotrypsin associated with chronic obstructive pulmonary disease in patients with the Leu-55-Pro mutation.
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PMID:Inactive conformation of the serpin alpha(1)-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational disease. 1061 72

Alpha-1-antitrypsin deficiency results from point mutations that distort the structure of the protein to allow a unique protein-protein interaction that we have termed loopsheet polymerisation. Polymers of Z alpha 1-antitrypsin accumulate within hepatocytes to form inclusion bodies that are associated with juvenile cirrhosis and hepatocellular carcinoma. The lack of circulating protein predisposes the Z alpha 1-antitrypsin homozygote to emphysema. This process also occurs in other members of the serine proteinase inhibitor (serpin) superfamily, antithrombin, C1-inhibitor and alpha 1-antichymotrypsin, in association with thrombosis, angioedema and chronic obstructive pulmonary disease, respectively, and we have recently shown that it underlies a novel inclusion body dementia. The interaction provides a useful paradigm for other 'conformational diseases' such as Huntington's disease, Creutzfeldt-Jakob disease and the amyloidoses.
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PMID:Alpha-1-antitrypsin deficiency, the serpinopathies and conformational disease. 1090 27

Alpha1-antitrypsin deficiency results from point mutations that distort the structure of the protein to allow a unique protein-protein interaction that we have termed loop-sheet polymerization. Polymers of Z alpha1-antitrypsin accumulate within hepatocytes to form inclusion bodies that are associated with juvenile cirrhosis and hepatocellular carcinoma. The lack of circulating protein predisposes the Z alpha1-antitrypsin homozygote to emphysema. This polymerization process also occurs in variants of other members of the serine proteinase inhibitor (serpin) superfamily, antithrombin, C1-inhibitor and alpha1-antichymotrypsin in association with thrombosis, angiooedema and chronic obstructive pulmonary disease respectively, and we have recently shown that it underlies a novel inclusion body dementia. Understanding this mechanism of polymerization allows rational drug design to block the protein-protein linkage and so ameliorate the associated disease.
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PMID:Loop-sheet polymerization: the mechanism of alpha1-antitrypsin deficiency. 1095 47

The overall improvement in the health of Americans over the 20th century is best exemplified by dramatic changes in 2 trends: 1) the age-adjusted death rate declined by about 74%, while 2) life expectancy increased 56%. Leading causes of death shifted from infectious to chronic diseases. In 1900, infectious respiratory diseases accounted for nearly a quarter of all deaths. In 1998, the 10 leading causes of death in the United States were, respectively, heart disease and cancer followed by stroke, chronic obstructive pulmonary disease, accidents (unintentional injuries), pneumonia and influenza, diabetes, suicide, kidney diseases, and chronic liver disease and cirrhosis. Together these leading causes accounted for 84% of all deaths. The size and composition of the American population is fundamentally affected by the fertility rate and the number of births. From the beginning of the century there was a steady decline in the fertility rate to a low point in 1936. The postwar baby boom peaked in 1957, when 123 of every 1000 women aged 15 to 44 years gave birth. Thereafter, fertility rates began a steady decline. Trends in the number of births parallel the trends in the fertility rate. Beginning in 1936 and continuing to 1956, there was precipitous decline in maternal mortality from 582 deaths per 100 000 live births in 1935 to 40 in 1956. Since 1950 the maternal mortality ratio dropped by 90% to 7.1 in 1998. The infant mortality rate has shown an exponential decline during the 20th century. In 1915, approximately 100 white infants per 1000 live births died in the first year of life; the rate for black infants was almost twice as high. In 1998, the infant mortality rate was 7.2 overall, 6.0 for white infants, and 14.3 for black infants. For children older than 1 year of age, the overall decline in mortality during the 20th century has been spectacular. In 1900, >3 in 100 children died between their first and 20th birthday; today, <2 in 1000 die. At the beginning of the 20th century, the leading causes of child mortality were infectious diseases, including diarrheal diseases, diphtheria, measles, pneumonia and influenza, scarlet fever, tuberculosis, typhoid and paratyphoid fevers, and whooping cough. Between 1900 and 1998, the percentage of child deaths attributable to infectious diseases declined from 61.6% to 2%. Accidents accounted for 6.3% of child deaths in 1900, but 43.9% in 1998. Between 1900 and 1998, the death rate from accidents, now usually called unintentional injuries, declined two-thirds, from 47. 5 to 15.9 deaths per 100 000. The child dependency ratio far exceeded the elderly dependency ratio during most of the 20th century, particularly during the first 70 years. The elderly ratio has gained incrementally since then and the large increase expected beginning in 2010 indicates that the difference in the 2 ratios will become considerably less by 2030. The challenge for the 21st century is how to balance the needs of children with the growing demands for a large aging population of elderly persons.
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PMID:Annual summary of vital statistics: trends in the health of Americans during the 20th century. 1109 82

Early erroneous diagnosis of rheumatic disease is common in subjects with arthropathy due to hereditary hemochromatosis. A 71-year-old male with chronic obstructive pulmonary disease and monoclonal gammopathy underwent hip replacement and was referred to our Department because of altered liver function tests. Test results were negative for hepatitis B surface antigen and hepatitis C virus, and positive for rheumatoid factor. A diagnosis of rheumatoid arthritis had been made on the basis of compatible joint involvement and laboratory data and steroid treatment prescribed. Since his serum ferritin was 3249 ng/mL, genetic testing for hereditary hemochromatosis was carried out and revealed homozygosity for Cys282Tyr mutation in the HFE gene. Liver biopsy disclosed cirrhosis compatible with hemochromatosis. Following a review of the patients' radiographs, the diagnosis of hemochromatosis arthropathy was made. Phlebotomies and family screening for hereditary hemochromatosis were done. The most logical explanation for the positive rheumatoid factor result in this subject are his age and the presence of two chronic diseases involving long-standing antigenic stimulation and monoclonal gammopathy of uncertain significance. It is important to distinguish rheumatoid arthritis from hemochromatosis arthropathy for several reasons: patients with hereditary hemochromatosis do not require corticosteroid treatment; in case of erroneous diagnosis of rheumatoid arthritis, phlebotomy is not started early, and familial genetic counseling is not considered. In male subjects with positive rheumatoid factor and joint and liver disease, hereditary hemochromatosis should be considered. More liberal use of genetic testing is justified in such cases.
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PMID:Hereditary hemochromatosis masquerading as rheumatoid arthritis. 1168 50

The diagnosis and management of strongyloidiasis present a continuous challenge in developing countries including Taiwan. In this study, the clinical characteristics and microbiological findings of 27 patients with Strongyloides stercoralis infection were retrospectively analyzed. Intestinal infection was identified in 17 patients and hyperinfection syndrome or disseminated disease in 10 (including 2 autopsy cases). The most frequent clinical findings were diarrhea (74%), fever (70%), abdominal pain (59%), cough (37%), dyspnea (33%), and constipation (26%). The common initial laboratory abnormalities were leukocytosis (81%), anemia (67%), liver function impairment (52%), and eosinophilia (44%). Most of the 27 patients had comorbid conditions, including malnutrition in 20 (74%), corticosteroid dependence in 15 (55%), chronic obstructive pulmonary disease in 9 (33%), chronic liver disease or cirrhosis in 8 (30%), and peptic ulcer disease in 7 (26%). There was no difference in the time interval from symptom onset to diagnosis between the intestinal infection group and the hyperinfection/disseminated group (22 +/- 15 vs 17 +/- 9 days). Larvae of S. stercoralis were identified in the stool of 24 patients, in the sputum smear of 5, in the gastric biopsy of one, and on histology of autopsy specimens in 2. Twenty-six patients received antiparasitic drug therapy of variable duration (mebendazole in 24, albendazole in 2, combined therapy in one). The overall cure rate was 52% (14/27). Relapse occurred in 4 patients. The overall mortality was 26% (7/27). There was a high mortality (up to 50%) in the hyperinfection/disseminated disease group. In conclusion, diagnosis of strongyloidiasis is often delayed and overlooked because of nonspecific symptoms. Physicians in endemic regions should include strongyloidiasis in the differential diagnosis when patients present with gastrointestinal and/or pulmonary symptoms with peripheral eosinophilia.
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PMID:Clinical manifestations of strongyloidiasis in southern Taiwan. 1195 Jan 17

The purpose of this investigation was to explore the psychiatric complications of liver cirrhosis in a Nigerian general hospital setting. The mental state of 31 consecutive patients with liver cirrhosis seen in a gastroenterology unit, from July 1996 to August 1998, was assessed using the 30-item General Health Questionnaire (GHQ-30) and Present State Examination (PSE) and compared with those of 20 hypertensive patients and 20 apparently healthy controls. The socio-demographic characteristics of patients with liver cirrhosis who developed psychiatric complications were compared with those without such complications. The mental status of the cirrhosis patients in the present study was also compared with those of Chronic Obstructive Pulmonary Disease (COPD) and hemodialysis patients in earlier studies. About fifty-five percent of all the liver cirrhosis patients developed diagnosable psychiatric morbidity. This was significantly higher than that of the hypertensive patients (15%) or healthy controls (5%) (P=0.0001). The psychiatric conditions were depressive episode, generalized anxiety disorder, delirium and adjustment disorder. No socio-demographic parameter had a significant effect on the mental state of the patients with liver cirrhosis. The same proportion (55%) of hemodialysis and a lower proportion (30%) of COPD patients compared with cirrhosis patients had psychiatric morbidity. We found a high rate of psychiatric morbidity in the patients with liver cirrhosis compared with those of hypertension and normal subjects. None of the socio-demographic variables considered was found to be associated with psychiatric complications in the liver cirrhosis patients. Poor medical conditions seem to be associated with high levels of psychiatric morbidity.
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PMID:Specific psychiatric morbidity in liver cirrhosis in a Nigerian general hospital setting. 1249 Mar 47


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