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)

Mortality and morbidity on dialysis remains high regardless of age in spite of technological improvements. While some of this is explainable and acceptable and related to co-morbid problems such as heart disease, malignancy, diabetes, etc. much is also preventable. Data from the literature would indicate that the provision of adequate dialysis as determined by a Kt/V (urea) of > 1.2 or a urea reduction rate (URR) > 65% will improve outcome. Attention to the nutritional status of the patient should also have impact. Low serum levels of urea, creatinine, albumin, anion gap, ideal body weight, and a low dietary protein intake as suggested by a reduced protein catabolic rate, are bad prognostic features. The elderly are more likely to have these features. While many factors may contribute to or cause protein malnutrition, underdialysis should be one easily recognized and reversible cause. Underdialysis will inevitably lead to poor nutrition and have an adverse effect on outcome.
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PMID:Adequacy of haemodialysis in the elderly. 949 36

Radiocontrast nephrotoxicity, which has increased in incidence with widespread use of radiological methods in medicine, is a serious complication of radiocontrast materials. In this study, we have prospectively investigated whether children with cyanotic congenital heart disease are at risk for radiocontrast nephrotoxicity with the use of a nonionic low osmolar contrast agent. Thirty-five children (17 cyanotic and 18 acyanotic patients) who underwent diagnostic cardiac catheterization were subjects of the study. The age range was from five days to 13 years. The volume of contrast material was 3.11 +/- 1.37 ml/kg in cyanotic patients and 2.67 +/- 0.86 ml/kg in acyanotic patients. Blood samples and timed urine samples were taken from all patients 24 hours before and 48 hours after cardiac catheterization. Blood urea nitrogen, creatinine, sodium, and phosphorus in serum, and creatinine and N-acetyl-beta-D-glucosamine in urine were analyzed. There was not a statistically significant difference between the values before and after angiography. As a result, we could find no evidence of radiocontrast nephrotoxicity with the use of a nonionic contrast agent in cyanotic and acyanotic patients who underwent cardiac angiography.
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PMID:Evaluation of renal functions in children with congenital heart disease before and after cardiac angiography. 967 35

One of the consequences of genetic impairments in early childhood is their long-term effect on children's developmental skills in communication, learning, and adaptive behaviors. Functional assessment provides families and clinicians with a common language for describing a child's strengths and limitations in self-care (feeding, dressing, grooming, bathing, continence), mobility, and communication/social cognition. The National Center for Medical Rehabilitation Research described a model of disablement that includes five dimensions: pathophysiology, impairment, functional limitations, disability, and societal limitations. Using this framework, along with the Functional Independence Measure for children, the WeeFIM(R), we describe functional strengths and challenges in children with Down syndrome, spina bifida, congenital limb anomalies, congenital heart disease, urea cycle disorders, severe multiple developmental disabilities, and DiGeorge malformation sequence. We also briefly describe several pediatric functional/adaptive assessment instruments used by developmental professionals (Battelle Developmental Inventory, Vineland Adaptive Behavior Scales, Amount of Assistance Questionnaire). By tracking functional status, health professionals can prioritize secondary and tertiary prevention strategies that optimize self-care, mobility, communication, and learning. When functional limitations interfere with the acquisition of these essential skills, family and community support programs can be maximized.
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PMID:Measuring functional status in children with genetic impairments. 1055 60

The P0 protein is part of the ribosomal eukaryotic stalk, which is an elongated lateral protuberance of the large ribosomal subunit involved in the translocation step of protein synthesis. P0 is the minimal portion of the stalk that is able to support accurate protein synthesis. The P0 C-terminal peptide is highly antigenic and a major target of the antibody response in patients with systemic lupus erythematosus and patients suffering chronic heart disease produced by the Trypanosoma cruzi parasite. The T. cruzi P0 (TcP0) protein was cloned into the pRSET A vector and expressed in Escherichia coli fused to a His-tag. The identity of the protein was confirmed by immunoblotting. Due to the formation of inclusion bodies the protein was purified using the following steps: (i) differential centrifugation to separate the inclusion bodies from soluble proteins and (ii) affinity chromatography under denaturing conditions. TcP0 showed high tendency to aggregation during refolding assays. However, TcP0 could be efficiently folded in the presence of a low concentration of SDS. The folding of the protein was confirmed using urea gradient electrophoresis, limited proteolysis, circular dichroism, and tryptophan fluorescence. Native electrophoresis showed that the folded TcP0 (and not a folding intermediate) was the cause of aggregation in the absence of SDS. The protocol described here permitted us to obtain large amounts (up to 30 mg per culture liter) of pure and folded TcP0, a very hydrophobic protein with a high tendency to aggregation.
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PMID:Overexpression and refolding of the hydrophobic ribosomal P0 protein from Trypanosoma cruzi: a component of the P1/P2/P0 complex. 1143 98

The objective of our study was to investigate the safety and efficacy of high-dose methyl prednisolone (MP) in modifying the systemic inflammatory response (SIR) to cardiopulmonary bypass (CPB) and to compare its efficacy with low-dose MP in children undergoing cardiac surgery for congenital heart disease. Thirty children with congenital heart disease undergoing CPB were randomly assigned to two groups: group 1 (n = 15) received 30 mg/kg MP by an intravenous infusion for 30 minutes and group 2 (n = 15) received 2 mg/kg intravenously, before the onset of CPB. Postoperative clinical parameters were recorded, and serum interleukin (IL)-6 and 8 levels, acute phase reactants, and blood biochemistry were determined serially for both groups. In both groups plasma IL-6 and 8 levels were elevated above the preoperative levels at 2 and 24 hours after declamping. The peak levels were obtained at 2-hour samples. The difference between the two groups in terms of postoperative IL-6 and 8 levels was not statistically significant. C-reactive protein (CRP) levels and polymorphonuclear leukocyte counts, postoperative core temperature, duration of mechanical ventilation, period of stay in intensive care unit, oxygenation indices, and biochemical parameters of patients did not significantly differ in the two groups. Only 1 patient in group 1 had elevated liver enzymes, blood urea nitrogen, and creatinine in the postoperative period. No significant complications were observed due to treatment with high-dose MP. Although postoperative IL and CRP levels indicated a SIR in our patients, the clinical picture was apparently affected in only 1 patient and she was in the high-dose MP group. CPB initiates a SIR that is associated with an increase in neutrophil count, CRP, and IL-6 and 8 levels. High-dose (30 mg/kg) MP was not superior to low-dose (2 mg/kg) in blunting the SIR to CPB in pediatric patients undergoing open-heart surgery.
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PMID:Systemic inflammatory response related to cardiopulmonary bypass and its modification by methyl prednisolone: high dose versus low dose. 1217 Mar 62

OBJECTIVE: The objective of this study was to analyze the incidence and significance of hepatic dysfunction after cardiac surgery in children. DESIGN: Prospective, observational study. SETTING: Pediatric intensive care unit of a university hospital. PATIENTS: The study consisted of 232 children ranging in age from newborn to 17 years with no history of liver disease. MEASUREMENTS AND MAIN RESULTS: Aspartate aminotransferase (AST), alanine aminotransferase (ALT), gammaglutamyltranspeptidase (GGT), alkaline phosphatase, total and conjugated bilirubin, blood glucose, urea, creatinine, and coagulation studies were determined at admission, at 24 and 48 hrs, and at 7 days. Hepatic dysfunction was taken as an ALT of > 100 IU/L or a moderate or high hepatic score. The statistical study included bivariate analysis and multivariate logistic regression to study the risk factors for hepatic dysfunction. Twenty-one patients (9%) showed an ALT > 100 IU/L, and 29.3% had a moderate or high hepatic score. A relationship was found between hepatic dysfunction and the type of cardiopathy (D-transposition of the great arteries and coarctation of the aorta), shock, the administration of dopamine or epinephrine, renal insufficiency, the presence of pulmonary changes (pulmonary edema, atelectasis, pulmonary hypertension, hypoxemia), hematologic disturbances (prothrombin time, kaolin-cephalin time, fibrinogen, and platelets), and the need for a greater number of transfusions of packed cells, plasma, and platelets. Compared with 7.6% of the rest of the patients (p <.001), 38% of patients with an ALT > 100 IU/L died. The hepatic score of those patients who died was 4.2 (2.3)-higher than that of the survivors at 1.5 (1.8), (p <.001). Shock and renal insufficiency were the factors most significantly related to the development of hepatic dysfunction. CONCLUSIONS: Hepatic dysfunction is an uncommon complication in children after cardiac surgery. This complication is related mainly to hemodynamic disturbances and renal insufficiency and is an indicator of poor prognosis.
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PMID:Hepatic dysfunction after cardiac surgery in children. 1279 88

The primary aim of this study was evaluation of the efficacy of telmisartan (angiotensin II receptor blocker- AT(1) blocker) on blood pressure in 10 patients with renal impairment in moderate or advanced stages of renal insufficiency and not dependent on haemodialysis. Its effect on proteinuria, renal function (represented by serum urea, creatinine, glomerular filtration), evaluation of overall therapy compliance in comparison with a previously prescribed angiotensin converting enzyme inhibitors (ACEI) were secondary aims. Considering the presence of left ventricle hypertrophy in all patients as a marker of hypertensive cardiopathy, the effect of telmisartan therapy on non-invasive cardiovascular parameters (ECG, echocardiography, and assessment of heart rate variability-HRV) was also evaluated. The study group involved 10 hypertensive patients (6 women, 4 men) with diabetic and non-diabetic renal impairment, proteinuria above 1 g/24 hours, hypertensive cardiopathy and intolerance of ACEI (cough). Telmisartan was added to their long-term antihypertensive combination therapy in a dose of 40 mg for the first 14 days, after which the dose increased to the maximal of 80 mg. The average initial daytime systolic blood pressure (SBP) was 149 +/- 19.7 mm Hg, average night-time SBP 145 +/- 23.0 mm Hg, average initial daytime diastolic BP (DBP) 90.6 +/- 2.5 mm Hg, night-time DBP 88.9 +/- 13.5 mm Hg. Average initial serum creatinine was 207.2 +/- 48.5 micromol/l, urea 15.1 +/- 4.4 mmol/l, GF 0.5 +/- 0.1 ml/s. Echocardiography revealed left ventricular (LV) hypertrophy with well preserved systolic and moderately impaired diastolic LV function. Also the HRV assessment revealed impaired neurovegetative (e.g. sympathovagal) balance. After 1 year of combination therapy with telmisartan, there was a clearly significant reduction in both SBP and DBP in both day and night-time (SBP daytime 149.6 vs.116.6 mm Hg, night-time 145.8 vs. 129.5 mm Hg; DBP daytime 90.6 vs. 83.5 mm Hg, night-time 88.9 vs. 79.3 mm Hg) and proteinuria (2.37 vs. 1.27 g/24 hour, p < 0.05). There were no significant changes in serum creatinine, urea values, and LV functions. On the other hand, further progression of the sympathovagal balance impairment was noted (continuing reduction of HRV in 9 from 10 patients), which can be described as the priority finding. The total compliance of telmisartan therapy was very good and without adverse clinical side effects. In conclusion - telmisartan reduces blood pressure and proteinuria safely and effectively in patients with various types of nephropathy in moderate or advanced stages of renal insufficiency.
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PMID:Telmisartan in the treatment of hypertension in patients with chronic renal insufficiency. 1552 50

Gadolinium chelates are widely used in magnetic resonance imaging as contrast medium in patients with nephropathy. However, only few studies have investigated the effect of gadolinium on serum creatinine concentration and estimated GFR as surrogate markers of renal function. This study was performed to evaluate the effect of gadopentetate dimeglumine in a dose sufficient for diagnostic and interventional purposes on renal function in a large sample of patients. We analyzed serum creatinine and serum-urea levels before and after the administration of gadopentetate dimeglumine in patients with normal and patients with pre-existing impaired renal function. Age, height, body mass, sex, medication and preexisting illnesses such as diabetes, renal artery stenosis and heart disease were monitored. In 181 patients with normal renal function, there was no statistically significant change in serum creatinine concentration after the administration of gadopentetate dimeglumine (at baseline: 0.72 +/- 0.18 mg/dl, after gadolinium: 0.73 +/- 0.22 mg/dl). In contrary, serum creatinine levels decreased significantly after the administration of gadolinium in 198 patients with pre-existing renal impairment (1.82 +/- 1.03 mg/dl before and 1.72 +/- 1.03 mg/dl after gadolinium) (p < 0.01). According to this surrogate marker of renal function, the change of estimated GFR in patients with normal baseline renal function was not significant, while in patients with impaired renal function, GFR increased after the administration of gadolinium (p < 0.001). The high diagnostic value of gadolinium contrast media is associated with a very small risk of adverse reactions. Our findings show that the administration of gadolinium even is associated with a decrease of serum creatinine in patients with pre-existing renal impairment. In conclusion, the use of gadolinium-based contrast media may be considered as a safe alternative in patients with impaired renal function for whom use of iodine-based contrast agents is prone to a high rate of radiocontrast-induced nephropathy.
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PMID:Renal effects of gadopentetate dimeglumine in patients with normal and impaired renal function. 1594 10

BACKGROUND: Amino terminal pro-brain natriuretic peptide (NT-proBNP) measurement can detect and assess heart failure. However, compared with traditional clinical parameters, its value in predicting the in-hospital mortality of patients with suspected heart disease has not been reported. METHODS: We examined the ability of 11 continuous and 21 categorical variables, including NT-proBNP levels measured at the time of admission, to predict in-hospital mortality. The setting was a small Irish rural hospital where 342 consecutive patients with suspected heart disease were admitted as acute medical emergencies. RESULTS: The 31 patients who died while in hospital had significantly higher NT-proBNP levels on admission than patients discharged alive (11,548+/-13,531 vs. 3805+/-6914 pg/mL, p<0.0001). Patients who died in-hospital were older, had significantly higher white cell counts, blood urea and modified early warning (MEW) scores, and lower temperatures, blood pressures and oxygen saturation. Four variables were found to be independent predictors of in-hospital mortality: a systolic blood pressure equal to or below 100 mm Hg, a urea level above 13 mmol/L, a white cell count greater than 13*10(9)/L and a NT-proBNP level greater than or equal to 11,500 pg/mL. The presence of three of these variables was associated with an in-hospital mortality rate of 54%. CONCLUSIONS: Four variables (i.e. hypotension, elevated urea, leukocytosis and elevated NT-proBNP levels) are comparable independent predictors of in-hospital mortality.
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PMID:The prediction of in-hospital mortality by amino terminal pro-brain natriuretic peptide (NT-proBNP) levels and other independent variables in acutely ill patients with suspected heart disease. 1596 36

To study the variation and significance of plasma coagulation factor VII (FVII) in different kinds of ischemia heart disease (IHD) and examine its relation with plasma lipid and gene polymorphism. FVIIa was determined with one stage clotting assay by using a recombinant soluble tissue factor (rsTF). FVIIc was measured with one stage clotting assay. FVIIag was quantified with an enzyme-linked immunosorbent assay (ELISA). Polymorphism was analyzed with PCR-urea-polyacrylamide gel electrophoresis. Our results showed that FVIIa in stable angina (SA), unstable angina (UA), obsolete and acute myocardial infraction (OMI, AMI) patients was higher than those of normal group with the differences being significant within any two groups. FVIIag in UA, OMI and AMI was higher than those in SA and normal groups. There were positive correlations between FVIIa and serum triglycerides, FVIIa and FVIIc, FVIIc and FVIIag. FVII-323 0/10 bp polymorphism analysis was performed in 60 patients and 0/10 bp polymorphism was found in 5 cases. FVIIc and FVIIag were much lower in cases of 0/10 bp groups than those in cases of 0/0 bp groups. It is concluded that there was activation of extrinsic coagulation pathway in every kind of IHD to different extent. FVIIa was the risk factor in the development of IHD, and more sensitive in reflecting the severity of cardiovascular disease than FVIIc or FVIIag. FVIIa was higher in OMI, which may be one of the risk factors of re-infraction. Serum triglyceride may indirectly lead to the development of IHD by increasing the level of FVIIa. FVII-323 0/10 bp polymorphism was present in Chinese patients with IHD and it was correlated with the level of FVIIc, FVIIag in plasma. 10 bp allelomorphic gene was a protective factor against thrombogenesis.
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PMID:Role of coagulation factor VII in pathogenesis of ischemic heart disease. 1735 81


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