Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective analysis of patients with hypertrophic obstructive cardiomyopathy treated by left ventricular myotomy and myectomy from 1972 to 1994 is reported. There were 158 patients (81 male and 77 female) with a mean age of 50.2(+/-17.2) years (range 12 to 80 years). One hundred nine patients (69%) were 60 years of age or younger, and 49 patients (31%) were older than 60 years. The overall mean follow-up period was 6.1(+/-4.8) years (range 0.1 to 19.3 years) and was 94% complete with a cumulative total of 956 patient-years. Preoperative exertional dyspnea was present in 84%, chest pain in 70%, presyncope in 54%, syncope in 31%, and cardiac arrest in 5% of patients. Preoperative cardiac catheterization was done in 150 patients, with mitral regurgitation detected in 104 patients (67%). The average maximal provocable left ventricular outflow tract gradient was 118 (+/-46) mm Hg (range 25 to 250 mm Hg). The average preoperative echocardiographic gradient at rest was 64 mm Hg, 20 mm Hg in the early postoperative period and 10 mm Hg in the late postoperative period. The mean septal thickness was 2.2 (+/-0.6) cm, 1.9 (+/-0.7) cm in the early postoperative period (p < 0.05 vs preoperative) and 1.7 (+/- 0.5) cm in the late postoperative period (p < 0.05 vs preoperative). The overall 30-day operative mortality rate was 3.2% (5/158), and 0% for 109 patients 60 years of age or younger. Causes of death included myocardial infarction and left ventricular free wall rupture, myocardial failure from septal perforation, sepsis, cerebrovascular accident caused by thromboembolism, and delayed cardiac tamponade in one patient each. Concomitant coronary artery bypass grafting was performed in 22 patients (19.3% of patients > or = to 40 years of age) and mitral valve replacement in 5 patients (3.2%). One hundred nine patients (69%) are alive, 10 patients (6.3%) were lost to follow-up, and 39 patients died (24.7%), including operative deaths). Actuarial survivals at 1, 5, 10, and 15 years were 92.4% +/- 2.2%, 85.4% +/- 3.1%, 71.5 +/- 4.6%, and 46% +/- 9%, respectively. The overall linearized death rate for discharged patients was 1.9%/pt-yr, and for cardiac related deaths it was 1.7%/pt-yr. Thirty-nine (36%) of the 109 survivors received beta-adrenergic blockers, and 30 (28%) received calcium channel blockers. Ninety-four patients had improvement in New York Heart Association functional class, 10 had improvement in symptoms but not in functional class, and 5 had no improvement in functional class or symptoms. Neither preoperative hemodynamic values nor routine echocardiographic measurements significantly correlated with quality of postoperative results. Left ventricular myotomy and myectomy is a safe and reproducibly effective operative treatment for medically refractory hypertrophic obstructive cardiomyopathy, especially for patients 60 years of age or younger. Improvement in functional class and symptoms can be expected in nearly all patients 60 years of age or younger. Improvement in functional class and symptoms can be expected in nearly all patients. The results of myotomy and myectomy serve as a standard for comparison with other interventions for medically refractory cardiomyopathy.
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PMID:Long-term results of left ventricular myotomy and myectomy for obstructive hypertrophic cardiomyopathy. 860 73

Advances in medical technology have made it possible to use emergency femoro-femoral bypass (FFB) for transport of hemodynamically unstable patients. In this study, we report on our experience of transport of patients with refractory heart failure by a special mobile mechanical circulatory support team (MMCST) using an intraaortic balloon pump (IABP) or FFB. A total of 22 patients (14 men, 8 women) were supported by the MMCST and transported to our clinic for further diagnostic or therapeutic procedures. The diagnoses in 12 patients was acute myocardial infarction, in 7 patients, dilatative cardiomyopathy (DCM), and in 3 patients, acute fulminant myocarditis. In 15 cases, FFB was implanted (5 in combination with IABP), and in 5 cases, IABP only was implanted. Two patients received maximal dosages of catecholamines. After arrival at our clinic, 11 patients received implants of a more sophisticated support system. From the myocardial infarction group, 3 patients received coronary artery bypass grafting, 1 patient received percutaneous transluminal coronary angioplasty, and 1 patient received heart transplantation as final therapy. In the myocarditis and DCM groups, 7 patients underwent heart transplantation. Finally, 11 patients (50%) survived, and 11 patients died of multiorgan failure or septicemia.
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PMID:Transport of hemodynamically unstable patients by a mobile mechanical circulatory support team. 885 15

In the multiple organ dysfunction syndrome of sepsis and septic shock the heart is one of the organs subject to failure. Many new insights into the mechanisms underlying septic cardiomyopathy were gained in the last years. Experimental work with neonatal and adult cardiomyocytes considerably contributed to this progress, facilitating the documentation of direct attenuation of the contractions of the heart muscle cell by toxins and mediators, as well as investigating the underlying cellular mechanisms. With this respect, contractile-depressant effects have been found in cardiomyocytes for many toxins and sepsis mediators, with endotoxin, Pseudomonas exotoxin A, tumor necrosis factor alpha, interleukin-1 and nitric oxide being the most relevant ones identified. These substances interfere at clinically relevant concentrations with several main inotropic axes, not only with the beta-adrenoceptor/adenylyl cyclase and with the NO-cGMP-system-on which most of the interest is focused at present-but also with the alpha 1-adrenoceptor/phosphoinositide pathway and the Ca2+ homeostasis of the cardiomyocyte, the latter representing the common final inotropic pathway. Not a single cardiodepressant factor, but more likely a total bunch of toxins and mediators with different attack mechanisms seem to contribute to the picture of septic cardiomyopathy.
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PMID:Elucidating molecular mechanisms of septic cardiomyopathy--the cardiomyocyte model. 897 69

Dysfunctioning of the heart forms part of the multiple organ dysfunction syndrome (MODS) in sepsis and SIRS. This acute septic cardiomyopathy is often underestimated in degree and relevance, although yet in fact 10% of all sepsis fatalities are due to intractable heart failure. This potentially reversible cardiomyopathy is characterized by a considerable pump failure, is not primarily ischemic, coronary blood flow being normal or even enhanced; left and right ventricle are enlarged as a consequence of an increased ventricular compliance. Damage of the heart can further be aggravated in case of an additional right ventricular impairment due to pulmonary hypertension in ARDS. SIRS-cardiomyopathy in non-infectious MODS has common traits with acute septic cardiomyopathy. The pathogenesis of heart disease in sepsis and SIRS is multifactorial, the endotoxin/TNF-alpha/NO/cGMP-cascade representing a main negative inotropic axis. Therapy of acute septic cardiomyopathy and SIRS-cardiomyopathy at present still is mainly symptomatic (volume substitution, inotropic/vasoactive agents), causal therapeutic principles are, however, put to test in the context of a comprehensive concept of causal sepsis treatment.
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PMID:[The heart in infection and MODS (multiple organ dysfunction syndrome)]. 917 72

We report 81 of 107 cases of hemolytic uremic syndrome (HUS), admitted between July 1994 and February 1996, following an outbreak of Shigella dysenteriae type 1 dysentery in Kwazulu/Natal. All patients, excluding 1, were black with a mean age of 38 months (range 1-121); 50 (61.7%) were males. The mean duration of dysentery was 11.3 days (range 1-41) and HUS 15 days (range 1-91). Most patients had acute oliguric renal failure (90.1%), 42 (51.6%) required peritoneal dialysis. Complications included encephalopathy 30 (37.0%), convulsions 12 (14.8%) and hemiplegia 2 (2.3%), gastrointestinal perforation 8 (9.9%), protein losing enteropathy 26 (32.1%), toxic megacolon 4 (4.9%), rectal prolapse 5 (6.2%), hepatitis 11 (13.6%), myocarditis 5 (6.2%), congestive cardiac failure 3 (3.7%), cardiomyopathy 3 (3.7%), infective endocarditis 1 (1.2%), septicemia 15 (18.5%), disseminated intravascular coagulation 17 (21%). Leukemoid reactions were found in 74 (91.3%) patients, hyponatremia in 56 (69.1%), and hypoalbuminemia in 67 (82.7%). Stool culture for Shigella dysenteriae type I was positive in only 7 (8.6%) patients; Shiga toxin assays were not performed. Outcome was as follows: recovery 32 (39.5%), impaired renal function 8 (9.9%), chronic renal failure 26 (32.1%), end-stage renal disease 1 (1.2%), and death 14 (17.3%) patients.
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PMID:Post-dysenteric hemolytic uremic syndrome in children during an epidemic of Shigella dysentery in Kwazulu/Natal. 932 80

Mixed connective tissue disease (MCTD) was first reported 25 years ago. This report provides an assessment of the course of juvenile (J) MCTD in 224 patients available in the literature until 1996, including our own 33 patients. Most patients improved and remissions were observed in 3-5% (up to 27%). Among the long-term problems, a loss in joint function was seen in up to 29% of the cases, renal involvement in up to 47%, restrictive lung disease in up to 54% and gastrointestinal manifestations consisting of oesophageal dysmotility in up to 29%. Cerebral involvement was rare but severe. Cardiovascular problems observed include cardiomyopathy, myopericarditis and pulmonary hypertension. Among other long-term problems were Raynaud's phenomenon and scleroderma-like skin changes in up to 86% of the patients. Seventeen of the 224 patients had died (7.6%) because of sepsis or infection (7), cerebral complications (3), heart failure (2), pulmonary hypertension (2), renal failure (2) or gastrointestinal bleeding (1). The mortality rate of JMCTD seems to be in the same range as that of juvenile systemic lupus erythematosus, dermatomyositis and scleroderma. When compared with the other connective tissue diseases, however, mainly minor long-term problems are seen in the surviving patients.
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PMID:Course of mixed connective tissue disease in children. 945 80

We treated 40 children, aged between 15 d and 17 y, diagnosed with acute respiratory distress syndrome and/or pulmonary hypertension, with inhaled nitric oxide. The most frequent underlying diagnosis associated with ARDS were bronchopneumonia (eight), cardiac surgery (five), and sepsis (three). Pulmonary hypertension was secondary to cardiomyopathy in 2 patients and occurred in the postoperative period of cardiac surgery in 17 patients--the most frequent were ventricular septal defect (5), transposition of great arteries (4), and atrioventricular septal defect (3). In 11 patients, sudden discontinuation of nitric oxide induced a decrease in oxygenation associated in some of the patients with an increase in pulmonary artery pressure. In two patients discontinuation of nitric oxide induced severe pulmonary hypertension, extreme bradycardia and hypoxaemia, which required cardiopulmonary resuscitation. When exogenous nitric oxide is abruptly interrupted, hypoxaemia and pulmonary hypertension are found in some patients, due to a decrease in the nitric oxide concentration in the pulmonary circulation. This may be caused by the exogenous nitric oxide administration that may have inhibited endogenous production. We recommend making a progressive withdrawal of inhaled nitric oxide to avoid the side effects observed in the sudden discontinuation.
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PMID:Life-threatening effects of discontinuing inhaled nitric oxide in children. 947 12

Bolus application of endotoxin to healthy volunteers results in reversible hemodynamic alterations, such as observed in septic cardiomyopathy. Currently, endotoxin-induced cardiodepression is mainly attributed to the endotoxin-induced release of proinflammatory cytokines into the circulation, particularly of tumor necrosis factor alpha and interleukin-1, the serum levels of these cytokines being enhanced in sepsis and septic shock, and also in various heart diseases. In this study, we report a proinflammatory effect of endotoxin (1-10 micrograms/ml, 24-h incubation period) on neonatal rat cardiomyocytes in serum-free culture, evidenced by induction of inducible nitric oxide synthase, enhanced release of nitrite (protein synthesis-dependent) and interleukin-6 into the supernatant, as well as an increase in cell-associated interleukin-1 and a specific cardiodepressant profile: endotoxin disrupts beta-adrenoceptor-mediated increase in pulsation amplitude, but alpha-adrenoceptor-induced increase in pulsation amplitude and arrhythmias are not suppressed. In the presence of dexamethasone (0.1 microM), the endotoxin-mediated blockade of beta-adrenergic responsiveness, as well as induction of inducible nitric oxide synthase, enhanced nitrite release and interleukin-1/-6-production are inhibited. In contrast, tumor necrosis factor alpha at a low concentration (10 U/ml) depresses alpha- and beta-adrenergic responsiveness in the presence of dexamethasone in a nitric oxide-independent manner. These data suggest a stimulatory effect of endotoxin on the cardiomyocyte and a specific proinflammatory and nitric oxide-dependent cardiodepressant profile of endotoxin.
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PMID:Endotoxin and tumor necrosis factor alpha exert a similar proinflammatory effect in neonatal rat cardiomyocytes, but have different cardiodepressant profiles. 961 43

Recent advances in the diagnosis and treatment of inborn errors of metabolism have improved substantially the prognosis for many of these conditions. This makes it essential that the practicing pediatrician be familiar with the clinical presentation of these disorders. A practical clinical approach to the recognition of inborn errors of metabolism in the young infant is presented in this review. Indications for specific laboratory studies are discussed. Guidelines are provided for the stabilization and emergency treatment of critically ill infants. This approach will identify those infants who will benefit from additional evaluation and specific treatment. Many of the inborn errors of metabolism, including urea cycle defects, organic acidemias, and certain disorders of amino acid metabolism, present in the young infant with symptoms of an acute or chronic metabolic encephalopathy. Typical symptoms include lethargy, poor feeding, apnea or tachypnea, and recurrent vomiting. Metabolic acidosis and/or hyperammonemia are observed in many of these conditions, but there are notable exceptions, including nonketotic hyperglycinemia and molybdenum co-factor deficiency. Therefore, appropriate laboratory testing for metabolic disorders should be performed in any infant who exhibits these findings. Although sepsis may be the initial consideration in a neonate with these symptoms, inborn errors of metabolism should always be in the differential diagnosis, particularly in a full-term infant with no specific risk factors. Hypoglycemia may be the predominant finding in a number of inborn errors of metabolism, including glycogen storage disorders, defects in gluconeogenesis, and fatty acid oxidation defects. The latter disorders, among the most common encountered, exhibit marked clinical variability and also may present as a sudden death, a Reye's-like episode, or a cardiomyopathy. Jaundice or other evidence of hepatic dysfunction is the mode of presentation of another important group of inborn errors of metabolism including galactosemia, hereditary tyrosinemia, neonatal hemochromatosis, and a number of other conditions. A subset of lysosomal storage disorders may present very early with coarse facial features, organomegaly, or even hydrops fetalis. Specific patterns of dysmorphic features and congenital anomalies characterize yet another group of inherited metabolic disorders, such as Zellweger syndrome and the Smith-Lemli-Opitz syndrome. Each of these symptom complexes, and the appropriate evaluation of the affected infants, is discussed in more detail in this review.
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PMID:Inborn errors of metabolism in infancy: a guide to diagnosis. 983 97

Cardiodepressant effects of tumor necrosis factor-alpha (TNF-alpha have been documented in numerous experimental settings in vivo and in vitro. In vivo administration of TNF-alpha mimicks the cardiovascular pattern of sepsis including septic cardiomyopathy. Serum levels of TNF-alpha were found to be elevated both in sepsis and in numerous non-septic heart disorders. Although an involvement of TNF-alpha in the pathogenesis of septic cardiomyopathy seems likely, presently no definite conclusion can be drawn with regard to the role of TNF-alpha in chronic heart failure. The origin and trigger mechanisms for the release of TNF-alpha in heart failure are a matter of debate, endotoxin (LPS) from intestinal translocation in venous congestion being one possible player. The negative inotropic impact of TNF-alpha is frequently ascribed to the induction of inducible nitric oxide (NO) synthase (iNOS). Results from in vitro studies rather suggest a complex interaction of TNF-alpha with the heart, with pleiotropic effects on cardiomyocyte performance, including an induction of iNOS at higher TNF-alpha concentrations, but NO-independent cardiodepression at low, pathophysiologically more relevant concentrations. TNF-alpha effects on the heart also vary with regard to the kinetics of the process: rapidly occuring cardiodepressant effects include a release of sphingosine and a suppression of the calcium transient, while chronic administration of TNF-alpha was shown to depress the synthesis of precursors for the phosphoinositide pathway and inhibit pyruvate dehydrogenase activity and mitochondrial function. Whether secondary cytokines induced by TNF-alpha in cardiomyocytes contribute to cardiodepression or whether apoptotic signals activated by TNF-alpha are involved in the cardiodepressive pathways is presently unknown.
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PMID:Cardiodepression by tumor necrosis factor-alpha. 988 17


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