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)

Many cases of cardiovascular diseases have been examined in reference to the distribution of ABO blood-groups, in order to calculate the relative risk of disease and the hemogroupal distributive significance in our samples as related to those of other authors, using combined calculation. The analysis concerned the following cases: 746 with arterial hypertension, 3258 with congenital heart disease, 4503 with articular rheumatism, 1047 with acquired valvulopathia, and respective controls. It was found that blood-group phenotypes represent an important biophysiopathological action in regard to articular rheumatism and its cardiac consequences, in myocardial infarction and in hypertension, males only. On the contrary, no action in regard to congenital heart disease was found, with the exception of some single anomalies which have yet to be confirmed. This hemogroupal action greatly exceeds the one limited to the immunitary analogy and is a noticeable part of family heredity. It shows itself in: -- a significant negative association with group O and positive association with group A in the myocardial infarction; -- a significant negative association with group O and positive for the others in the valvulopathic (rheumatic) diseases; -- a positive association with A phenotype and negative with B in arterial hypertension, males only; -- no association with ABO blood-groups and congenital heart disease.
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PMID:[ABO blood-group phenotypes and pathogenesis of cardiovascular diseases. Congenital, rheumatic and coronaric heart disease and arterial hypertension (author's transl)]. 120 47

A large pedigree with high prevalence of heart disease is investigated to analyse the association between polymorphic blood markers and quantitative risk factors for cardiovascular disease. The analysis incorporates a familial correlation structure among the individuals in the pedigree and a generalized power transformation to induce approximate residual normality of the risk factors. A total of 380 marker/risk factor combinations are analysed, and at the normal 1% significance level, positive associations are found between the A antigen of the ABO locus and both serum total cholesterol and low-density lipoprotein cholesterol, and negative associations are found between the B antigen of the ABO locus and serum total cholesterol, and between the B allele of acid phosphatase (AP) locus and systolic blood pressure.
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PMID:Association between polymorphic blood markers and risk factors for cardiovascular disease in a large pedigree. 347 81

The correlation analysis of ratios between six polymorphic genetic systems (ABO, MNSs, Rh, Hp, Gm, HLA) and mortality from ishemic heart disease, brain vascular lesions, and hypertensive disease in 17 European populations has been made. A statistically significant correlation has been established between the populational frequency of most of the 50 phenotypes and genes under study, and mortality. The qualitative structure of correlations and their quantitative expression depend on the cause of death, age and sex. The possible mechanisms of relationship between the genetic populational differences and mortality from cardiovascular diseases are discussed.
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PMID:[Balanced hereditary polymorphism and the mortality from cardiovascular diseases in the populations of 17 countries of Europe. I. A correlation analysis]. 653 62

Five patients with thrombotic thrombocytopenic purpura (TTP) were treated with corticosteroids, plasma exchange (PE), and antiplatelet agents. ABO- and Rh-compatible fresh-frozen plasma (FFP) was used as the replacement fluid. None of the patients received FFP infusion without PE. The patients were followed for 14-32 months after initial treatment. Four of the five patients are in complete clinical remission. One responded to plasma exchange but died of heart disease. This report shows an 80% survival rate which is markedly better than that without the use of PE and FFP infusion. We found dramatic clinical and hematologic improvement beginning shortly after initiating treatment. Two patients showed reversal of their initial clinical and hematologic improvement when PE was discontinued, despite continuing treatment with antiplatelet drugs and in one with corticosteroids. Both patients showed hematologic and clinical remission after reinstitution of PE. None of the survivors had any neurologic or renal deficit. They were maintained on aspirin and dipyridamole for 1 year after discharge.
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PMID:Treatment of thrombotic thrombocytopenic purpura with plasma exchange: five cases. 654 86

Six cases of combined heart and kidney transplantation with organs from the same donor are reported. All six patients suffered from primary end-stage kidney disease, two chronic glomerulonephritis, two glomerulosclerosis, one chronic pyelonephritis and one with unknown etiology. Four patients were undergoing hemodialysis. Three patients had the diagnosis of ischemic heart disease, one dilated cardiomyopathy secondary to congenital heart disease, two idiopathic dilated cardiomyopathy. Five were males and one female. Ages ranged from 38 to 54 years. On-site or short-distance young donors with normal renal function and good cardiac function necessitating low inotropic support were selected. ABO compatibility was used exclusively. Orthotopic heart transplantation was performed first. During cardiopulmonary bypass, hemofiltration was used in four cases. Kidney transplantation was performed immediately after the closure of the chest. Diuresis was immediate in all cases. No cardiac rejection was documented at EMB. Renal function normalized within few days with no signs of kidney rejection. All six patients are alive and well with normal cardiac and renal function at a mean follow-up of 43 months. Patients and donors selection associated with a proper surgical strategy and prompt immunosuppressive therapy administration make the combined heart and kidney transplantation an effective therapeutic option.
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PMID:Combined heart and kidney transplantation: an effective therapeutic option--report of six cases. 937 Apr 13

We report on a female preterm infant with hepatic failure and neonatal tissue siderosis of hemochromatotic type diagnosed by using both histochemistry and atomic absorption spectroscopy. The infant presented with meconium ileus, signs of rapidly progressive hepatic failure, and hyperferritinemia (7132 ng/ml). Despite surgery and intensive care the infant died 32 days after birth. Postmortem examination showed a wrinkled liver with extensive collapse of the hepatic architecture and regenerating nodules as well as hepatic and extrahepatic iron accumulation of hemochromatotic type, sparing the reticuloendothelial system. Atomic absorption spectroscopy confirmed an increase in the iron content of various organs: liver, heart, pancreas, oral salivary gland, kidney, and adrenal gland. The increase in the iron content of various organs was determined by comparing the analysis of the propositus with those of 5 gestationally age-related preterm infants who had died in the intensive care unit: 2 died of meconium aspiration syndrome, the other 3 of hyaline membrane disease, bronchopulmonary dysplasia, and immaturity, respectively. We also compared the analysis of 15 fetuses having a a condition predisposing to iron accumulation (trisomy 21, trisomy 18, cytomegalovirus, amnion infection syndrome, Rhesus- and ABO-incompatibility, congenital hemolysis, anti-phospholipid syndrome, congenital heart disease). Delta F508, the most frequent mutation seen in cystic fibrosis patients, was excluded by gene sequencing. Different noxae causing iron accumulation in the neonatal period have led to the statement that neonatal hemochromatosis may collect different etiologies, such as metabolic disorders, infections, chromosomal aberrations, and immunological disorders. In this study, we report the singular evidence of neonatal iron accumulation of hemochromatotic type in an infant presenting with meconium ileus and propose a classification of the neonatal disorders associated with iron accumulation.
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PMID:Hepatic failure with neonatal tissue siderosis of hemochromatotic type in an infant presenting with meconium ileus. Case report and differential diagnosis of the perinatal iron storage disorders. 1170 Aug 92

Based on data reported to the United Network for Organ Sharing through December 2001: 1. The number of heart transplant procedures performed in the United States increased slightly (< 1%) during each of the last 2 complete years from 2,187 transplants during 1999 to 2,197 transplants during 2000, followed by an additional increase in 2001 to 2,202 transplants. A more substantial increase was seen in the number of lung transplants performed: from 890 transplants in 1999 to 956 in 2000 (+7%) and an additional increase to 1,053 transplants in 2001 (+10%). Fewer than 30 heart-lung transplants were performed in 2001. Living-donor lung transplants comprised 2-3% of lung transplants performed between 1995-2001. 2. Pediatric recipients were more frequently on life support in the ICU; more likely to have an ischemia time of at least 4 hours; more often gender mismatched; and more often ABO-compatible rather than ABO-identical with the donor than adult recipients for all thoracic organ types. 3. The most common indication for transplant since 1996 in adult heart recipients was coronary artery disease (50%), followed closely by cardiomyopathy (42%). Among pediatric heart recipients, the 2 most common indications: cardiomyopathy (46%) and congenital heart disease (43%) accounted for approximately 90% of the transplants. The indications for lung transplants were more disparate. In adult lung recipients, the 4 most common diagnoses (COPD - 42%, IPF - 17%, CF - 15% and A1A - 9%) encompassed more than 80% of the transplants. More than half of the pediatric lung transplants were performed in recipients with CF. The 3 most frequently cited indications for adult heart-lung transplant recipients (Eisenmenger's Syndrome, other congenital heart diagnoses and PPH) accounted for greater than 75% of the transplants. 4. Approximately 30-35% of adult heart transplants since 1999 have been performed in patients who were Status 1A. For pediatric transplant recipients, Status 1A comprised 60-70% of the transplants. 5. The one-year survival rate for transplants performed during the first three-quarters of 2001 was 85% for both adult and pediatric heart transplant recipients and 77% for both adult and pediatric recipients of lung transplants. For adult heart-lung transplants performed during 2000, the one-year survival rate was 69%. 6. The long-term patient survival rates were: 39% for adult heart recipients and 50% for pediatric heart recipients at 12 years; 18% at 11 years for adult lung recipients and 31% at 9 years for pediatric lung recipients; and 24% at 11 years for adult heart-lung recipients and 21% at 8 years for pediatric heart-lung recipients. 7. Drug-treated rejection and drug-treated infection were reported to occur before discharge in approximately 20-40% of transplant recipients, with the exception of pediatric lung and heart-lung recipients, with rates varying by organ and age group. Drug-treated infections were reported before discharge in more than 60% of pediatric lung recipients and approximately half of pediatric heart-lung recipients. 8. Approximately 60% of adult heart recipients and 70% of pediatric heart recipients were hospitalized at least once during the first 3 years following their transplant.
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PMID:Thoracic organ transplantation in the US. 1297 35

ABO blood groups were determined in 404 patients who had cardiac surgery for heart disease; 136 of these patients had rheumatic valvular heart disease and 268 had congenital heart disease. The incidence of each ABO blood group was compared to a control series of 2171 patients by means of the varkappa(2) test. There was no statistical difference in the incidence of ABO blood group when patients with congenital and rheumatic valvular heart disease were compared with the control group.
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PMID:ABO BLOOD GROUPS IN PATIENTS WITH CONGENITAL AND RHEUMATIC VALVULAR HEART DISEASE. 1412 69

Heart transplantation with ABO blood type-incompatible donors has historically been contraindicated because of the high risk of an immediate hyperacute humoral graft rejection. The immature neonatal immune system presents an immunologic window that allows for breaching the ABO barrier before the natural development of anti-ABO antibodies. Information from a small series of neonates has demonstrated similar survival rates and posttransplant outcomes compared to ABO-compatible transplantations. In the posttransplant period, particular attention is placed on the surveillance of graft-specific antibody production and monitoring for immunologic signs and symptoms of early graft vasculopathy. This article presents a case study of a neonate with congenital heart disease who underwent one of the first successful ABO-incompatible heart transplantations in the United States.
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PMID:Pediatric heart transplantation across ABO blood type barriers: a case study. 1601 65

In the pediatric age group shortage of donor hearts leads to mortality rates of 30-50% on the waiting list. Because of the immaturity of the immune system of infants, ABO-incompatible heart transplantation may be an option to increase donor availability. We transplanted two infants with blood type O at the age of 7 and 5 months, respectively, with complex congenital heart disease. Intraoperative plasma exchange was performed during cardiopulmonary bypass followed by standard immunosuppression. Both recipients received a blood type A donor organ. Plasma was exchanged up to six times until anti-A antibodies were eliminated. No hyperacute rejection occurred, ventricular function is excellent and there have been no acute rejection episodes up to 4 months after transplantation. Anti-A antibody titers remained low and eventually disappeared. ABO-incompatible cardiac transplantation shows good short-term results in young infants and appears to be a safe procedure to reduce mortality on the waiting list.
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PMID:Successful ABO-incompatible heart transplantation in two infants. 1616 9


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