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

Young adults with cyanotic congenital heart disease have a high incidence of respiratory and haemostatic problems. Activated neutrophils release vasoactive and chemotactic factors which result in endothelial injury, lung parenchymal damage and the activation of platelets and coagulation pathways. To investigate the contribution of neutrophil activation to morbidity in young adults with cyanotic congenital heart disease, plasma neutrophil elastase levels were measured in 25 cyanotic patients and the results compared to patients with acyanotic heart disease and normal controls. Neutrophil elastase levels were significantly elevated in the group with cyanotic congenital heart disease (P < 0.001). Platelet activation was significantly increased in the patients with cyanotic heart disease (P < 0.001). Platelet aggregation was impaired only in those with haematocrits greater than 0.50 (P < 0.02). Whole blood coagulation, as determined by thrombelastography, was within normal limits. The reason for neutrophil activation in patients with cyanotic congenital heart disease is unclear, but activated neutrophils may contribute to the respiratory and haemostatic problems common to these patients.
 ...
PMID:Neutrophil activation and morbidity in young adults with cyanotic congenital heart disease. 818 Mar 36

Twenty-nine children 3 months to 17 years of age undergoing operations for congenital heart disease were included in this prospective study. Complement activation, activation of the plasma contact system, leukocytes, leukocyte elastase release, and C-reactive protein were studied during and after cardiopulmonary bypass for the first postoperative week and related to multiple system organ failure occurring in eight (27.5%) of the 29 children. During cardiopulmonary bypass complement activation via the alternative pathway as indicated by significant conversion of C3 (expressed by C3d/C3) and abnormally high C5a values at the end of cardiopulmonary bypass without consumption of C4 was shown in all children. At the end of cardiopulmonary bypass, C3 conversion was significantly higher in the eight patients with multiple system organ failure than in the others (p < 0.05), whereas no difference in C5a level was shown. All children had a significant increase in leukocyte count directly after protamine administration (p < 0.0001) and elastase release during cardiopulmonary bypass that was significantly higher in patients with multiple system organ failure than in those without (p < 0.05). Consumption of prekallikrein as an indicator of activation of the Hageman system was not detectable during cardiopulmonary bypass in any child. After cardiopulmonary bypass, in patients without multiple system organ failure, C3d/C3 decreased and reached preoperative values within the first postoperative week, whereas, in patients with multiple system organ failure, C3d/C3 increased further, reaching a maximal value on the third postoperative day. In comparison with patients without multiple system organ failure, patients with multiple system organ failure showed a severe decrease of C4 (with minimal values on the third postoperative day), suggesting consumption by activation of the classic pathway of the complement system or a hepatic synthesis deficiency. Prekallikrein values were also significantly lower in patients with multiple system organ failure than in the others, with a maximal difference on the third postoperative day (p < 0.005). C-reactive protein was significantly lower in patients with multiple system organ failure than in the others for the first 2 postoperative days (p < 0.05), probably because of severe hepatic failure in patients with multiple system organ failure. This study demonstrates that, in children, cardiopulmonary bypass induces complement activation principally via the alternative pathway. It suggests a relationship between complement activation and multiple system organ failure observed in the postoperative period. Furthermore, it points out the role of multiple system organ failure itself on the C3 conversion and on the synthesis of the markers of the inflammatory response in children after heart operations.
 ...
PMID:Complement activation during cardiopulmonary bypass in infants and children. Relation to postoperative multiple system organ failure. 824 80

Extramedullary hematopoiesis occurring in the myocardium has previously only been reported in a single case of a neonate with cyanotic congenital heart disease. Herein we report the incidental discovery of extramedullary hematopoiesis or pure erythropoiesis in four failing adult hearts with myocardial infarction. In two cases, extramedullary hematopoiesis or erythropoiesis was identified in cardiectomy specimens removed at orthotopic heart transplantation; in two other cases, erythropoiesis was found in left ventricular tissue removed at the time of implantation of left ventricular assist devices. Myocardial hematopoiesis/erythropoiesis was identified based on characteristic light-microscopic findings in routinely processed tissue and was confirmed by immunhistochemistry using monoclonal antibodies to the erythroid cell marker glycophorin A (positive in all cases), the megakaryocyte marker CD61, and the granulocyte marker neutrophil elastase (the latter two markers positive in one case only). None of the four patients had a myeloproliferative disorder or evidence of a myelophthisic process. No hematopoietic elements were identified in 109 cardiectomy specimens without acute or recent infarcts. Myocardial hematopoiesis or erythropoiesis could represent heretofore-unrecognized manifestations of altered cytokine expression in patients with heart failure due to myocardial infarction.
 ...
PMID:Hematopoiesis/erythropoiesis in myocardial infarcts. 1140 61