Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant disorder caused by mutation in the transthyretin gene. The most common mutation is substitution of valine for methionine at position 30 (MET30). Liver transplantation (LT) is the preferred treatment. After LT, although many patients show stabilization or improvement in the disease, adverse outcomes have been reported in those who have malnutrition, long-standing disease, and non-MET (NMET) mutations at position 30. Our aim is to compare survival and outcome of symptoms associated with FAP after LT in patients with MET30 and NMET30 mutations. Medical records of all patients who underwent LT for amyloidosis at our institution were reviewed to obtain demographic information and clinical features, such as severity of neuropathy, diarrhea, orthostatic hypotension, and posterior wall or ventricle septal thickness before and after LT. Fifteen patients underwent LT for amyloidosis at our institution between 1990 and 2000 (MET30, n = 5; NMET30, n = 7; hereditary amyloidosis, n = 2; primary amyloidosis, AL type, n = 1). Patients with hereditary and primary amyloidosis were excluded from analysis. One- and 3-year survival rates after LT in MET30 patients were 100%. Before LT, five of five patients had sensorimotor neuropathy; five of five patients had diarrhea, and four of five patients had orthostatic hypotension. After LT, improvement or stabilization of neuropathy was seen in two of five patients; of diarrheal symptoms, in three of five patients; and of orthostatic hypotension, in three of four patients. One- and 3-year survival rates after LT in NMET30 patients were 100% and 85.7%, respectively. Before LT, six of seven patients had sensorimotor neuropathy, six of seven patients had diarrhea, and five of seven patients had orthostatic hypotension. After LT in this group, improvement or stabilization of neuropathy was seen in two of six patients; of diarrhea, in six of six patients; and of orthostatic hypotension, in five of five patients. Before LT, posterior wall and/or ventricle septal thickness was increased in two of five MET patients and seven of seven NMET patients. Five of seven NMET30 patients (71.4%) who received a combined liver and heart transplant had stabilization, and two patients in the NMET group and one patient in the MET group had progression of heart disease. Outcomes for LT for patients with FAP with MET or NMET mutations were similar. Earlier LT for patients with FAP with MET30 or NMET30 mutation would improve outcomes after LT.
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PMID:Outcome of liver transplantation for familial amyloidotic polyneuropathy. 1462 27

(1) Drug interactions potentiate or oppose the therapeutic or adverse effects of one or more co-administered drugs. (2) There are two mechanisms: pharmacodynamic interactions occur between drugs that have similar actions or adverse effects. The shared effect can potentiate or antagonise the actions of a physiological system such as clotting, or the parasympathetic nervous system. Pharmacokinetic interactions alter the metabolism of the relevant drug(s). (3) The clinical consequences of drug interactions are very variable. Most interactions with serious consequences involve either high risk drugs or high risk patients (such as older people). (4) The risk of interactions increases with the number of drugs taken, and with advancing age. (5) Some patients are at an increased risk of drug interactions because they have pathology that alters drug metabolism, such as renal failure, liver failure or malnutrition. Other patients are at risk because of the type and number of drugs they need (this is particularly true for patients with heart disease, HIV infection, epilepsy, tuberculosis and migraine). (6) In practice, patients must be questioned thoroughly about all the drugs they are taking, whether or not they have been prescribed.
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PMID:Avoiding drug interactions. 1523 47

Rheumatic heart disease poses a major challenge to public health and is the most prevalent heart disease in children. The major determinants of rheumatic fever and rheumatic heart disease are poverty, malnutrition, overcrowding, poor housing, and a shortage of health-care resources. Although cost-effective strategies for the prevention and control of these diseases are available, they remain underutilized in most developing countries. A World Health Organization Expert Consultation reviewed the current scientific knowledge of rheumatic fever and rheumatic heart disease, as well as medical and public-health practices, and revised the Jones diagnostic criteria for rheumatic fever and rheumatic heart disease. This revision facilitates the diagnosis of: primary episodes of rheumatic fever, recurrent attacks of rheumatic fever in patients with or without rheumatic heart disease, rheumatic chorea, insidious onset rheumatic carditis and chronic rheumatic heart disease. The present report provides practical recommendations for an evidence-based approach to the prevention and management of rheumatic fever and rheumatic heart disease. It also provides practical guidance for implementing cost-effective programmes for controlling these diseases. The report will be of interest to clinicians, policy-makers and public-health professionals.
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PMID:Rheumatic fever and rheumatic heart disease. 1538 6

Heart failure is a severe pathology, which has displayed a dramatic increase in the occurrence of patients with chronic heart disease in developed countries, as a result of increases in the population's average age and in survival time. This pathology is associated with severe malnutrition, which worsens the prognosis. Although the cachexia associated with chronic heart failure is a well-known complication, there is no reference animal model of malnutrition related to heart failure. This study was designed to evaluate the nutritional status of rats in a model of loss of cardiac function obtained by ascending aortic banding. Cardiac overload led to the development of cardiac hypertrophy, which decompensates to heart failure, with increased brain natriuretic peptide levels. The rats displayed hepatic dysfunction and an associated renal hypotrophy and renal failure, evidenced by the alteration in renal function markers such as citrullinemia, creatininemia, and uremia. Malnutrition has been evidenced by the alteration of protein and amino acid metabolism. A muscular atrophy with decreased protein content and increased amino acid concentrations in both plasma and muscle was observed. These rats with heart failure displayed a multiorgan failure and malnutrition, which reflected the clinical situation of human chronic heart failure.
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PMID:Aortic banding in rat as a model to investigate malnutrition associated with heart failure. 1563 66

1. In developed countries, the major burden of disease is due to chronic diseases, such as heart disease and stroke. In contrast, the major burden of disease among people in developing countries has been due largely to diseases caused by malnutrition, poor sanitation and infection. In recent years, with increasing economic and demographic development, there has been a shift in developing countries from diseases caused by poverty towards chronic, non-communicable, lifestyle-related diseases. The rapid emergence of these chronic diseases has not occurred with a similarly rapid decline in infectious diseases. Therefore, these developing countries are experiencing high rates of both infectious and chronic diseases. 2. The increase in chronic diseases in developing countries has been brought about by the increasing prevalence of risk factors, such as increased alcohol consumption, smoking, obesity, physical inactivity and low fruit and vegetable intake. In parallel with this, there is also increased evidence of high blood pressure and high cholesterol levels. 3. The preventive strategies required to reverse this trend for these emerging diseases include the education of public health professionals, the introduction of surveillance activities to monitor changes in risk factors, the introduction of health promotion, the development of prevention research and improved advocacy for disease prevention programmes. Experience from other countries provides evidence that prevention programmes can work. The global challenge is to ensure that implementation of such programmes in the world's developing nations does not come too late.
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PMID:Hypertension 2020: confronting tomorrow's problem today. 1585 45

Human existence requires that agriculture provide at least 50 nutrients (e.g., vitamins, minerals, trace elements, amino acids, essential fatty acids) in amounts needed to meet metabolic demands during all seasons. If national food systems do not meet these demands, mortality and morbidity rates increase, worker productivity declines, livelihoods are diminished and societies suffer. Today, many food systems within the developing world cannot meet the nutritional needs of the societies they support mostly due to farming systems that cannot produce enough micronutrients to meet human needs throughout the year. Nutrition transitions are also occurring in many rapidly developing countries that are causing chronic disease (e.g., cancer, heart disease, stroke, diabetes, and osteoporosis) rates to increase substantially. These global developments point to the need to explicitly link agricultural technologies to human health. This paper reviews some ways in which agriculture can contribute significantly to reducing micronutrient malnutrition globally. It concludes that it is imperative that close linkages be forged between the agriculture, nutrition and health arenas in order to find sustainable solutions to micronutrient malnutrition with agriculture becoming the primary intervention tool to use in this fight.
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PMID:Agriculture: the real nexus for enhancing bioavailable micronutrients in food crops. 1602 91

The health care systems of the U.S.-associated Pacific Island jurisdictions, especially the three freely associated states (Federated States of Micronesia, Republic of the Marshall Islands, and Republic of Belau), are faced with problems similar to developing countries such as malnutrition and infectious diseases, as well as diseases relating to westernization such as diabetes, heart disease, and cancer. Although cancer has emerged as an important cause of morbidity and mortality in the Pacific, little population-based data are currently available. This paper addresses some of the practical and methodological challenges to obtaining accurate and reliable cancer data in these jurisdictions. This paper discusses the use of annualized period prevalence to allow for some measurement of cancer burden when cancer incidence cannot be accurately calculated. This method, however, has its own limitations as cancer prevalence relates to both incidence and duration of illness, and numerous factors impact survival potential (i.e., preexisting diseases, lifestyle practices, and access to treatment). In addition, under-ascertainment and data quality issues will impact any cancer morbidity or mortality measurements. Thus, improvement in the health care systems, including the creation and ongoing support of active cancer registries would be the optimal approach to better delineating cancer occurrence and risk for the populations of these Pacific Island jurisdictions.
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PMID:Cancer epidemiology in the freely associated U.S. Pacific Island jurisdictions: challenges and methodologic issues. 1628 83

Pompe disease, a disorder caused by a deficiency in the lysosomal enzyme acid alpha glucosidase, is frequently overlooked as a cause of floppy baby syndrome. The accurate diagnosis of floppy baby syndrome requires the sequential evaluation of medical causes (e.g., hypothyroidism, sepsis, malnutrition, malabsorption, congenital heart disease), neurologic etiologies (central [cerebral] and peripheral [lower motor unit]) and anatomic characteristics of the abnormality. Cardiomegaly on chest x-ray in a patient with floppy baby syndrome should alert the pediatrician to suspect Pompe disease. Based on this finding, further work-up or referral to a specialist can be considered. Pompe disease requires immediate attention. Symptomatic intervention of this disorder should be initiated at the earliest time possible to maximize the potential benefit from therapy and to prevent irreversible organ damage. Moreover, early diagnosis is important for providing parents with realistic information about their child's prognosis, and where appropriate, professional genetic counseling. Enzyme replacement therapy (ERT) with recombinant human GAA is currently being evaluated in clinical trials; the future availability of this option makes early identification of this condition even more critical. This article presents a unified view on the optimal approach to the accurate diagnosis of Pompe disease and to its recognition as one of the possible and treatable causes of floppy baby syndrome.
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PMID:Diagnostic challenges for Pompe disease: an under-recognized cause of floppy baby syndrome. 1670 78

Anemia is prevalent in renal transplant recipients (RTRs), as it is in all chronic kidney disease (CKD) populations. Mild anemia occurs in up to 40% of RTRs, and more severe anemia (110 g/L) occurs in about 9% to 22% of patients. As in CKD, impaired graft (renal) function is a major predictor of anemia identified in nearly all studies, suggesting a major role for erythropoietin deficiency. Chronic inflammation, malnutrition, iron deficiency, and medications (angiotensin converting enzyme inhibitors, angiotensin receptor blockers, mycophenolate, azathioprine, and sirolimus) are contributory factors seen in some, but not all, studies. Although pathophysiologic and observational data strongly support a causal association between low hemoglobin levels and cardiovascular outcomes in RTRs, no randomized controlled trial to date has been able to show a clear benefit of anemia treatment on cardiovascular outcomes or mortality in either RTR or other CKD populations. This important paradox has led some investigators to question the causal nature of the association between anemia and heart disease. Resolution of this paradox, at least for patients with stage 2/3 CKD, will depend on the outcome of randomized controlled trials currently in progress. Similar trials sorely are needed in renal transplant populations. In the interim, current opinion favors treating persistent anemia in RTRs to achieve targets similar to those recommended for dialysis and CKD patients.
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PMID:Anemia, renal transplantation, and the anemia paradox. 1694 69

Bone disorders clearly related to nutrition are osteomalacia and osteoporosis. Osteomalacia is caused by a deficiency of vitamin D or a disturbance of its metabolism. Dietary deficiency of phosphate or excess of aluminum or cadmium will also cause osteomalacia. Osteoporosis is associated with low intake of calcium and other nutrients. Dietary copper deficiency might stimulate bone metabolism and increase in hip fractures. Excess vitamin A intake was also associated with lower bone mineral density and higher risk of hip fractures. Excess vitamin D sometimes causes mental simplicity, congenital heart disease and calcification of soft tissue. Therefore not only diet but also drugs and supplements of nutrients should be carefully observed in older women.
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PMID:[Nutrition-related bone disease]. 1697 84


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