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)

Between May 1988 and July 1991, 28 neonates and children underwent orthotopic heart transplantation at Children's Memorial Hospital in Chicago. Indications for heart transplantation were hypoplastic left heart syndrome (10), dilated cardiomyopathy (13), aortic stenosis with endocardial fibroelastosis (1), complex D-transposition of the great arteries after Senning repair (1), L-transposition of the great arteries with single ventricle after shunt (1), cor biloculare, pulmonary atresia, and situs inversus after Fontan (1), and chronic rejection after heart transplantation for hypoplastic left heart syndrome (1). The age at time of transplantation ranged from 2 days to 17 years (mean, 5.3 +/- 6.1 years). Early deaths were from intraoperative donor right ventricular failure (2) and acute rejection after a second transplant procedure at 21 days (1), for an in-hospital mortality rate of 10.7%. Immunosuppression was with cyclosporine, azathioprine, and prednisone, with an attempt to discontinue the prednisone in neonates at age 6 months as guided by endomyocardial biopsy. Rejection episodes were treated with methylprednisolone pulse (34) or with OKT3 (4). Endomyocardial biopsy (in patients older than 6 months) was used extensively, and acute rejection was diagnosed in 29 of 301 biopsies. Three late deaths occurred (mean follow-up, 16.3 +/- 11.8 months): one of acute rejection at 13 months, one of viral pneumonia at 7 months, and one of intraabdominal sepsis as a complication of peritoneal dialysis at 5 months. Actuarial survival at 2 years is 77% +/- 9% (standard error of the estimate). Heart transplantation for neonates and for children can be performed with acceptable operative mortality. Intermediate results with triple therapy immunosuppression and an intensive rejection surveillance regimen relying on endomyocardial biopsy are encouraging.
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PMID:Heart transplantation in neonates and in children. 157 37

Acute pulmonary embolus (less than 6 weeks old) has been considered an absolute contraindication to heart transplantation for fear of the potential problems of lung abscess, empyema, bronchopleural fistula, and systemic sepsis in an immunosuppressed patient. It is difficult to adhere to this principle because 30% to 50% of patients with dilated cardiomyopathy may have an acute pulmonary embolus and would be excluded from transplantation. Several centers have considered such patients for heart transplantation if they are young, on maximal medical therapy, and in extremis. The surgical management of the postoperative pulmonary problems can include bronchoscopy, antibiotics, surgical drainage, decortication, and pulmonary resection with or without muscle flaps. We describe our approach to two such patients who were managed successfully with lobectomies and latissimus dorsi muscle flaps to seal the bronchus and fill the pleural space.
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PMID:Successful heart transplantation in recipients with recent preoperative pulmonary emboli. 261 52

A familial etiology was identified on the basis of family history in 16 (8.75%) of 184 patients undergoing cardiac transplantation at Stanford for endstage dilated cardiomyopathy (DC). These 16 patients, from 11 families, included 5 sibling pairs. To help determine optimal management of such patients, their case histories and posttransplant courses were reviewed. Mean age of patients at presentation was 23 +/- 15 years. In sibling pairs, duration of symptoms from onset to diagnosis was 14 +/- 5 weeks for the first sibling, but only 4 +/- 2 weeks for the second. Progressive cardiac enlargement was documented radiographically in siblings of transplant recipients in 2 families before the onset of symptoms. The posttransplant course with regard to rejection incidence, infectious complications, coronary artery disease and malignancy was similar to that of the 168 patients with nonfamilial DC. Actuarial survival at 5 years after transplantation was 80%. Thirteen patients (including all sibling pairs) are alive 1 to 11 years after transplantation. Sepsis was the cause of death in 3 patients, occurring during the early postoperative period in 2 and following retransplantation for graft atherosclerosis 7 years after the initial transplant in the third patient. Thus, diagnosis of DC in childhood or adolescence mandates evaluation and surveillance of family members, because this disease can progress rapidly. The favorable results of cardiac transplantation for familial DC suggest that it should be promptly considered for such patients with end-stage disease.
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PMID:Frequency of familial nature of dilated cardiomyopathy and usefulness of cardiac transplantation in this subset. 264 93

The presence of acute pulmonary infarction is not uncommon in severely ill patients and is considered by some to be a contraindication to heart transplantation. It has been our policy to accept these patients for operation. The purpose of this investigation is to examine the results of this policy in patients receiving immunosuppression with cyclosporine A and azathioprine. Between September 1982 and April 1985, eight patients undergoing heart transplantation demonstrated clinical and radiographic evidence of acute preoperative pulmonary infarction. These patients represented 5.5% of our heart transplantation population during this period (total = 145). The age range in these eight patients was from 22 to 55 years. Congestive cardiomyopathy was present in four patients, and four patients had ischemic cardiomyopathy. All patients were New York Heart Association functional status class IV. Five of the eight patients were on inotropic support, and one patient had associated renal failure. Pulmonary infarcts were located in the right, middle, or lower lung field in seven patients and in the left lower lung field in one patient. All patients were treated perioperatively by intensive physiotherapy and specific antibiotics. In four patients the lesions resolved on medical treatment alone. Three patients developed extensive empyema and required chest drainage with or without decortication. Two of these patients survived with complete resolution of the lung lesions, and one patient died from prolonged renal failure and sepsis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The influence of acute preoperative pulmonary infarction on the results of heart transplantation. 330 55

Intracardiac masses are rare in infants and children. Early detection is essential to their successful management. We present seven patients in whom echocardiography established the diagnosis and was crucial in the management. Three of the masses were primary cardiac tumors and four were thrombi. Patient 1: an infant with a calcified left ventricular fibroma. Patient 2: a neonate who presented with cyanosis due to obstruction of the right ventricular inflow tract by a fibroblastic tumor. Patient 3: an infant with a right atrial myxoma presenting as sepsis. Patient 4: a child who had a pulmonary embolus after a pulmonary valvotomy and was found to have a right ventricular thrombus. Patient 5: a child with a right atrial thrombus following a Fontan procedure for univentricular atrioventricular connection. Patient 6: a child with a left ventricular thrombus due to a dilated cardiomyopathy in association with epidermolysis bullosa. Patient 7: An infant with bilateral lobar emphysema, an aorticopulmonary window with left ventricular fibroelastosis, who developed a left ventricular thrombus.
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PMID:Echocardiography of intracardiac filling defects in infants and children. 382 62

An X-linked recessive disease is reported in a large pedigree. The disease is characterised by a triad of dilated cardiomyopathy, neutropenia and skeletal myopathy. The untreated patients, all boys, died in infancy or early childhood from septicemia or cardiac decompensation. Ultrastructural abnormalities were observed in mitochondria in cardiac muscle cells, neutrophil bone marrow cells and to a lesser extent (0-9%) in skeletal muscle cells. Membrane-bound vacuoles were seen in neutrophil bone marrow cells. Intramuscular fat droplets were increased in type I skeletal muscle fibres. An affected patient had intermittent lactic acidemia, borderline low plasma carnitine, the latter decreasing during periods of illness, and low muscle carnitine (27% pretreatment; 35-40% posttreatment). While on treatment with oral carnitine he had less weakness and no cardiac complaints, but his neutropenia was not affected. Respiratory chain abnormalities were observed in this patient's isolated skeletal muscle mitochondria. These were: (1) diminished concentrations of cytochromes c1 + c, b and aa3 to 29, 47 and 64% of the averaged controls, and (2) a lowered P:0 ratio for oxidation of ascorbate + TMPD, with diminished uncoupler stimulated Mg2+-ATPase activity. Muscle AMP deaminase was deficient (5 resp. 17%). Only one previous report (Neustein et al. 1979) on X-linked mitochondrial cardiomyopathy exists, which probably refers to the same entity. Biochemical studies and haematological abnormalities (neutropenia) are reported for the first time.
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PMID:An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes. 614 97

Report of an unusually advanced, fibrosing, granulomatous myocarditis due to generalized sarcoidosis which presented itself clinically as a congestive cardiomyopathy in a 45-year-old man with a one-year's remission before death. Clinically cardiac sarcoidosis takes often a silent course. There is no exact information on its true incidence. Sudden death is not uncommon, mostly in young and middle-aged adults of either sex. Various cardiac structures may be damaged, especially the ventricular septal myocardium. The diagnosis is seldom established clinically; histological evaluation of specimens from other organs may be indicative. The response to antiarrhythmic agents and corticosteroids is considered with sepsis.
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PMID:[Cardial sarcoidosis: fibrosing granulomatous myocarditis]. 666 4

Nitric oxide (NO), produced by either constitutive or inducible isoforms of NO synthase (cNOS or iNOS), influences myocardial inotropic and chronotropic responses. This pathway has been studied using NO donors or NOS inhibitors or by immune-mediated stimulation of iNOS. Although inhibition of constitutive NO activity in the heart does not influence indices of myocardial contractility, NO donors, in some species and preparations, may exert a negative inotropic effect as well as an enhancement of diastolic relaxation. The best documented cardiac action of NO is inhibition of the positive inotropic and chronotropic responses to beta-adrenergic receptor stimulation. Basal NO production, presumable via cNOS, appears to exert a mild tonic inhibition of beta-adrenergic responses. On the other hand, excessive NO production mediated by iNOS may contribute to the myocardial depression and beta-adrenergic hyporesponsiveness associated with conditions such as sepsis, myocarditis, cardiac transplant rejection, and dilated cardiomyopathy. Muscarinic cholinergic stimulation of the heart appears to stimulate NO production that mediates, at least partially, parasympathetic slowing of heart rate and inhibition of beta-adrenergic contractility. NO-stimulated production of 3',5'-cyclic guanosine monophosphate via guanylyl cyclase accounts for many of the observed physiological actions of NO. 3',5'-Cyclic guanosine monophosphate inhibits the beta-adrenergic-stimulated increase in the slow-inward calcium current and reduces the calcium affinity of the contractile apparatus, actions that could contribute to a negative inotropic effect, an abbreviation of contraction, and an enhancement of diastolic relaxation. Biochemical, immunocytochemical, and molecular biological techniques have been used to show the presence of both cNOS and iNOS within the myocardium. cNOS is expressed in myocytes, endothelial cells, and neurons in the myocardium, and there is evidence for iNOS in myocytes, small vessel endothelium, vascular smooth muscle cells, and immune cells that infiltrate the heart. Taken together, these observations suggest that NO influences normal cardiac physiology and may play an important role in the pathophysiology of certain disease states associated with cardiac dysfunction.
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PMID:Role of nitric oxide in the regulation of myocardial function. 756 4

Eleven cases of intracardiac thrombi caused by different factors including protein-C deficiency are presented for discussion of the etiology and predisposing factors of intracardiac thrombi during infancy and childhood, and to stress the importance of protein-C deficiency as an etiological factor. Thrombi were localised in the left heart in five patients and right heart in five patients. One patient had both-sided thrombi. Four of our patients had dilated cardiomyopathy, one had mitral valve hypoplasia, and one had pulmonary valvar stenosis as the predisposing factors for thrombus formation. In three patients whose cardiac anatomies were completely normal, we determined protein-C deficiency as an etiological factor of thrombus formation. One of these had congenital protein-C deficiency and the other two had acquired temporary protein-C deficiency due to sepsis. In conclusion we recommend that protein-C deficiency should be investigated as an etiological factor in all cases of intracardiac thrombi irrespective of whether or not another predisposing factor is identified.
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PMID:Intracardiac thrombosis diagnosed by echocardiography in childhood: predisposing and etiological factors. 822 59

The most important technical improvements of implantable cardioverter-defibrillators (ICD) of the latest generation comprise more sophisticated antitachycardia pacing options, stored intracardiac electrograms and biphasic shock capabilities which virtually always allow ICD implantation without thoracotomy. The present study summarizes the first clinical experience with these new devices. In 37 consecutive symptomatic (near sudden death 17, syncope 16, pre-syncope 4) patients aged 56 +/- 10 years with refractory ventricular arrhythmias (presenting arrhythmia: ventricular fibrillation 14, ventricular tachycardia 22, not documented 1), an ICD (Jewel PCD 7219, Medtronic) was implanted. Coronary artery disease was present in 21, dilated cardiomyopathy in 5, valvular heart disease in 2 and various conditions in 8 patients; the mean left ventricular ejection fraction was 43 +/- 18%. In 29 patients (78%), the ICD was inserted in a pectoral and in 8 (22%) in an abdominal position. A non-thoracotomy lead (NTL) configuration was successfully implanted in 36/37 patients (97%) (purely transvenous systems in 30, in combination with subcutaneous patch electrode in 6). Surgical complications comprised one pneumothorax, one hemorrhage and one death due to sepsis; during a mean follow-up of 5 +/- 3 months, another patient died of heart failure and 2 revisions (5.4%) for lead problems (1 connector, 1 SQ-patch) became necessary. In 23/37 patients (62%), the ICD was activated after 74 +/- 89 days post implant. 22 of these 23 patients (96%) received one or more appropriate shocks (9 +/- 22 shocks per patient). The actuarial survival was 95% at 6 months. In the present study, an ICD of the newest generation was successfully implanted without thoracotomy in > or = 97% and with purely transvenous systems in > or = 84%. Compared to older systems, this has made the implantation procedure remarkably easier and will most likely lead to a further reduction in mortality and morbidity. Despite the relatively short follow-up, the high incidence of appropriate ICD utilization underscores the high recurrence rate of arrhythmias in this population and suggests that the ICD may be very effective in preventing unnecessary rehospitalizations.
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PMID:[Initial clinical results with a novel implantable cardioverter-defibrillator: a prospective evaluation in 3 Swiss university hospitals]. 855 30


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