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)

The case is described of an infant who suffered from progressive, severe dystrophy, hemolytic and megaloblastic anemia, hematuria, proteinuria and slight uremia. He died at 4 months of age following two acute episodes of heart failure. Abnormally increased excretion of methylmalonate and homocystine was detected by our screening program for metabolic disorders. Amino acid analyses showed that the plasma and urine levels of methionine were very low whereas those of cystathionine were raised. Vitamin B12 deficiency, malabsorption or abnormal cobalamin transport were excluded by a normal serum total cobalamin and normal transcobalamins. These findings suggested a congenital error of cobalamin metabolism. Treatment with vitamin B12 resulted in a biochemical though not a clinical response. Postmortem examination revealed severe vascular lesions with changes in the kidney characteristic of thrombotic microangiopathy supporting a diagnosis of hemolytic-uremic syndrome. It is assumed that the elevated plasma homocysteine induced the vascular lesions by causing detachment of endothelium.
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PMID:Congenital defect in intracellular cobalamin metabolism resulting in homocysteinuria and methylmalonic aciduria. I. Case report and histopathology. 52 29

The pathology of primary familia amyloidosis with polyneuropathy is described on the basis of post-mortem examination of six cases from Northern Sweden. Clinically the disease is characterized by progressive sensory and motor disturbances with loss of sensation, muscular wasting and flaccid paralysis. Impotence, urinary bladder dysfunction, motility disturbances of the gastro-intestinal tract and postural hypotension indicate affection of the autonomic nervous system as well. Malabsorption, cardiac insufficiency and vitreous opacites also occur. As regards the distribution of amyloid, the following findings seemed to be characteristic. Usually there were no gross lesions indicating the amyloid disease. Histopathologically, amyloid deposits were observed in great extent in the peripheral nervous system and in various parts of the peripheral autonomic nervous system as well. It occurred extensively in the walls of blood vessels of various calibres, in the perivascular collagenous connective tissue and adjacent to the smooth musculature. Amyloid deposition was also found more or less abundantly in various other organs and tissues. No deposits, however, or only insignificant amounts, were found in the central nervous system, either in the parenchyma of the liver, in the islets of Langerhans, or in the bone marrow. Clinical manifestations seemed to be related to the local deposition of the amyloid substance. Our clinical and pathological findings in this particular type of familial amyloidosis conformed mostly to those previously described.
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PMID:Postmortem findings in primary familial amyloidosis with polyneuropathy. 115 99

Twenty-two patients with general variable immunodeficiency (GVI) and malabsorption syndrome (MS) were followed up for 2-12 years. III degree MS was found in 17 cases. Serum immunoglobulins concentration and T-lymphocyte count were reduced, the latter at the expense of theophylline-resistant and active E-RFC. With casein and milk albumin as the antigens, lymphokine-producing capacity of the mononuclear cells appeared elevated. MS treatment with adjuvant gamma-globulin produced a positive trend in clinical manifestations of the disease, content of T lymphocytes and relevant subpopulations. Long-term results were less favourable: partial compensation with recurrences persisted in 15 patients only. Seven patients died: two of pneumonia, five of cardiac failure and visceral dystrophy. All MS patients are recommended to undergo serum immunoglobulins diagnosis of GVI and in case of its verification to receive life-time gamma-globulin replacement therapy.
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PMID:[General variable immunologic deficiency with malabsorption syndrome]. 239 30

Cs pharmacokinetic profiles using HPLC have aided in predicting necessary dosage alterations for specific groups of transplant patients. Additional information has been gained by HPLC profiles in nontransplant subjects who are healthy or have a stable disease state. The clinician now knows that liver disease not only impairs Cs elimination but may also have a pronounced effect upon drug absorption. While the cardiac failure patient may have reversible inhibition of Cs clearance, other factors may affect the distribution of the drug to lower dosage requirements. Impaired renal function is not an impediment to Cs elimination, but malabsorption similar to that observed in liver and bone marrow transplant patients may still occasionally complicate therapy. Pharmacokinetic information on Cs must be integrated into the complex care plan of a transplant patient to optimally utilize and monitor this pharmacologic agent.
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PMID:Cyclosporine pharmacokinetic profiles in liver, heart, and kidney transplant patients as determined by high-performance liquid chromatography. 353 65

Jejunoileal bypass reportedly inhibits the absorption of cyclosporine. In patients with malabsorption syndromes, bile acid replacement has been given to enhance the absorption of cyclosporine. A candidate for heart transplantation with New York Heart Association class IV heart failure and a jejunoileal bypass received cyclosporine alone and with concomitant ursodiol administration to determine whether therapeutic cyclosporine plasma concentrations would be attainable after heart transplantation. There were no differences in peak concentration, time to peak, area under the serum concentration versus time curve, or bioavailability of oral cyclosporine when administered alone or with ursodiol. Plasma cyclosporine concentrations were consistent with those in the general population.
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PMID:Influence of bile acid replacement on cyclosporine absorption in a patient with jejunoileal bypass. 766 69

The sulphonylureas and the biguanides are widely used as adjuncts to dietary measures in the treatment of non-insulin-dependent (type 2) diabetes mellitus (NIDDM). Adverse effect profiles differ markedly between the sulphonylureas and biguanides, reflecting differences in chemical structure and mode of action. Sulphonylureas are generally well tolerated, although pharmacokinetic differences between these agents have important clinical implications. The main adverse effect associated with sulphonylureas is hypoglycaemia. This effect is a predictable consequence of the principal pharmacological effect of these drugs, i.e. sensitisation of the islet beta-cell to glucose, resulting in enhanced endogenous insulin secretion. Sulphonylurea-induced suppression of hepatic glucose production may cause profound and protracted hypoglycaemia, especially in elderly patients, in individuals with intercurrent illnesses and reduced caloric intake, or when taken in combination with other compounds with hypoglycaemic potential, e.g. alcohol (ethanol). Sulphonylureas with a longer duration of action, notably chlorpropamide and glibenclamide (glyburide), are more liable to induce serious hypoglycaemia, particularly when drug elimination is reduced by renal impairment. Other drugs such as salicylates may potentiate the actions of sulphonylureas, thereby increasing the risk of hypoglycaemia. Biguanide therapy is associated with alterations in lactate homeostasis which under certain clinical circumstances may result in fatal lactic acidosis. Phenformin is associated with a markedly greater risk of lactic acidosis than metformin. Phenformin has been withdrawn in many countries for this reason. All biguanides must be avoided in patients with renal impairment, hepatic dysfunction and cardiac failure--conditions where drug accumulation or disordered lactate metabolism may predispose to lactic acidosis. Phenformin should not be given to individuals who exhibit a severe, genetically conferred hepatic defect of hydroxylation which impedes metabolism of this drug. Less seriously, the biguanides are associated with a relatively high incidence of gastrointestinal adverse effects which limit compliance. Acarbose, a competitive inhibitor of intestinal alpha-glucosidases, has recently been introduced. In contrast to the sulphonylureas and biguanides, acarbose has not been associated with life-threatening adverse effects. This reflects the low systemic absorption of the drug and, predictably, its principal unwanted effects are gastrointestinal disturbances resulting from iatrogenic carbohydrate malabsorption.
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PMID:Comparative tolerability profiles of oral antidiabetic agents. 784 43

Malnutrition resulting from chronic congestive heart failure (cardiac cachexia, CC) is not uncommon and contributes to mortality and morbidity especially of elderly people. The aetiology of cardiac cachexia is probably multifactorial. We have assessed whether malabsorption of fat is associated with CC and if so whether it is due to small-bowel bacterial overgrowth. Three groups of subjects were studied: 29 (20 women) patients (mean age 76.1 years) with controlled congestive heart failure and weight loss (CC); 14 (seven women) patients (mean age 74.0 years) with controlled congestive heart failure and no weight loss (non-cachexia, NON-CC); and 29 (20 women) healthy controls (mean age 74.9 years). Fast absorption was quantified using the cumulative 6 h 14CO2 exhalation in the 14C-triolein breath test and small-bowel bacterial overgrowth was quantified using the cumulative 8 h 14CO2 exhalation in the 14C-glycocholic acid breath test. The cumulative 6 h 14CO2 exhalation in the triolein breath test was reduced in the CC group (p = 0.001) implying impaired fat absorption. There was no evidence of small-bowel bacterial overgrowth in any group. Impaired absorption of fat was related to the clinical severity of heart failure and its duration. Impaired fat absorption is associated with cardiac cachexia. It is not due to small-bowel bacterial overgrowth. The aetiology of fat malabsorption in heart failure requires further studies.
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PMID:Fat malabsorption in elderly patients with cardiac cachexia. 867 May 44

A continuously increasing production level in poultry breeding has resulted in changes in metabolism. Selection procedures in breeding programmes are focused on an increase in growth rate and on a decrease in feed conversion ratio (less feed intake per unit of deposited tissue). These procedures do not pay attention to the maintenance requirements of birds. Imbalances between production (protein and fat deposition) and supply of energy for maintenance requirements lead to homeostatic dysregulation and to diseases of organs which supply the energy for production and maintenance. The alarming increase in metabolic diseases, such as heart failure syndrome, ascites, and oedema in the lungs and heart, can be directly related to an insufficient oxygen supply. A low oxygen consumption and heat production is one of the mechanisms by which a low feed conversion ratio can be achieved, as is induced hypothyroidism by which physical activity and thus heat production is reduced. Other diseases, such as liver cirrhosis, malabsorption syndrome, sudden death syndrome in broilers, and fatty liver-hemorrhage syndrome, which is nowadays the most important disease in laying hens in the Netherlands, can be related to an imbalance between the production rate and maintenance requirements. A continued selection on the basis of retained energy (in protein and fat) without paying attention to the maintenance requirements of birds will be detrimental for the health and welfare of poultry. These undesirable developments in poultry husbandry should be a challenge for sciences focused on welfare and stress in animals. Such a scientific approach to animals suffering from dysgenic changes in metabolism is needed to solve serious problems in poultry breeding.
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PMID:Pathological changes in metabolism of poultry related to increasing production levels. 932 54

We reviewed clinical presentation, investigations, therapy, prognosis and outcome of 232 patients with primary (AL) cardiac amyloidosis. There were 142 men and 90 women. Median age at presentation was 59 years (range 29-85). AL heart disease was unusual both in patients under the age of 40 (3.0%) and in non-Caucasians (6.5%). Fatigue and weakness were the commonest presenting symptoms. Hallmark features of periorbital ecchymoses and macroglossia were present in 12.5% and 27.2%, respectively. AL cardiac amyloidosis was unusual in isolation (3.9%), and most frequently patients had features of multiorgan dysfunction; heavy proteinuria and features of malabsorption predominating in this respect. Heart involvement represents the worst prognostic indicator, with a median survival from diagnosis of 1.08 years, falling to 0.75 years with the onset of heart failure. Current therapeutic procedures appear to prolong survival, with left ventricular wall thickness, mass and ejection fraction on echocardiography and late potentials on signal averaged electrocardiography of use in prognostic stratification. Cardiac involvement from AL amyloidosis is rapidly fatal. It should be suspected in all patients with heart failure who have wall thickening on echo, normal chamber sizes, low EKG voltages and evidence suggesting a multisystem disease.
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PMID:The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. 957 96

Cardiac cachexia is divided into two types, i.e., the classic type, which occurs in patients with severe heart failure, and the nosocomial type, which develops in the postoperative state. Cardiac cachexia is due both to a decrease in nutrient intake (anorexia, malabsorption) and to specific metabolic alterations (hypercatabolism with increased energy expenditure, response to hypoxia, inflammatory status, etc). Among the various mechanisms involved in the pathogenesis of cachexia, cellular hypoxia has long been recognized. The chronic activation of the endogenous neurohormonal system is another specific feature of such patients; a striking relationship was found between cardiac cachexia and hormonal levels which correlate better than the classical parameters of cardiac failure severity. Finally, inflammatory syndrome has been known to occur frequently in patients with cardiac cachexia. Several studies have shown that tumor necrosis factor-alpha was significantly increased in cachectic patients and that chronic activation of the systemic immune response might be a common and unifying factor.
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PMID:Metabolic and nutritional disorders in cardiac cachexia. 1152 73


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