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59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between 1972 and 1985, 107 patients with chronic Idiopathic Thrombocytopenic Purpura underwent splenectomy. Platelet life span and sites of sequestration were studied with labelled platelets and external scanning. Medical treatment was always of scarce and transient effectiveness and had considerable side effects. Splenectomy had minimal complications and mortality and caused no hazard of overwhelming sepsis in adults. The results of splenectomy were very satisfying, especially when platelet sequestration was mainly splenic (remission in about 90% of patients). Surgical treatment is at present the most effective in patients with chronic ITP.
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PMID:Surgical treatment of chronic idiopathic thrombocytopenic purpura: results in 107 cases. 377 Nov 23

Myocardial performance in critically ill patients is primarily responsive to the need to supply O2 to the periphery. An increase in CO is the common finding in an acute illness characterized by an increase in systemic VO2 (for example, sepsis and trauma), since acute variations in flow are the most efficacious mode of augmenting systemic O2t to match the VO2. The lower systemic VO2 of a patient with an acute cardiac illness explains why the CO in this disease is not as elevated as that found in the acutely ill patient with sepsis or trauma. Endogenous compensatory mechanisms used to vary flow according to the need for O2t include heart rate, ventricular preload, contractility, and afterload. An increase in LV contractility and a reduction in afterload facilitate LV stroke volume, hence O2t. Conversely, pulmonary hypertension may result in a restriction of LV preload if RV pump failure ensues. Other factors relevant to the care of the critically ill that will decrease LV preload--and thus reduce the heart's left-sided adaptation to maintain O2t--include the presence of underlying cardiac disease, which will limit any necessary increase in contractility, and the use of PEEP, which will restrict venous return to the RV. Therapeutic intervention is required when O2t does not balance systemic VO2 and arterial lactate levels rise. The use of resuscitative fluid to improve flow by the Frank-Starling (preload) mechanism may be limited by the compliance properties of either ventricle, but it is a reasonable first choice, with guidelines for administration determined by the PCWP, which influences fluid flux across the pulmonary microvascular exchanging membrane. Vasodilators may be used to increase CO by reducing impedance to ventricular ejection; they may also improve LV compliance, thereby allowing the administration of more fluid (that is, increasing preload) without an untoward rise in the PCWP. If vasodilators are without effect or are potentially dangerous because of concomitant hypotension, inotropic support to increase O2t is required. A brief summary of interventional pharmacologic support in acute illness is depicted in Figure 8.
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PMID:Myocardial function in the critically ill: factors influencing left and right ventricular performance in patients with sepsis and trauma. 390 47

The most common hematologic and oncologic indications for splenectomy in childhood are hereditary spherocytosis, chronic idiopathic thrombocytopenic purpura, hypersplenism, and Hodgkin's disease. Because of the increased incidence of septic complications after splenectomy, benefits to be gained from the operation should be weighed against the risks. A retrospective study was done on the charts of 42 consecutive children with hematologic and oncologic disorders, who underwent splenectomy between 1967 and 1982. The incidence of septic complications after splenectomy was 12%; sepsis, however, only occurred in patients with severe underlying diseases (three patients with Hodgkin's disease, one patient with systemic lupus erythematosus, and one patient with chronic pseudo-malignant immunoproliferation). In contrast, none of the patients who were splenectomized for other reasons (mainly hereditary spherocytosis and chronic immune thrombocytopenic purpura) had a septic complication. Two patients with end-stage Hodgkin's disease (5%) experienced fatal septic complications. Although splenectomy is well established for diagnostic and therapeutic considerations in patients with Hodgkin's disease, not all of them might benefit from this operation, and studies with a more limited approach to splenectomy might prove to be of the same therapeutical value.
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PMID:Hematological and oncological indications for splenectomy in children. 392 22

Intravenous gammaglobulin is effective therapy of ITP and other autoantibody-mediated immune cytopenias. All children as well as adults unresponsive to splenectomy or with known immune deficiency are probably the best candidates for treatment with IVGG. Its major advantage, in addition to its efficacy of treatment and possible remission-inducing effect, is that it has the fewest side effects of any treatment of ITP so that it is the best maintenance therapy of patients when effective. Future uses of IVGG remain to be determined. Premature infants with a high mortality from sepsis and with hypogammaglobulinemia due to termination of pregnancy prior to transplacental antibody transfer may benefit from IVGG. A preliminary study suggested such benefit and also showed safety of IVGG treatment in that there was no impaired immune responsiveness of these prematures at 2 years of age (28). Another potential usage of IVGG involves the treatment of the hypogammaglobulinemia associated with certain types of malignancy. Patients with CLL, especially in the advanced stages, are often hypogammaglobulinemic. Multiple myeloma and Waldenstrom's macroglobulinemia are two other B-cell malignancies associated with antibody production defects which might benefit from antibody replacement therapy. Therapeutic IgG levels may be harder to obtain due to hypercatabolism of immunoglobulin. The issue of immune hyporesponsiveness during intensive chemotherapy is also unexplored. Secondary antibody responses do not seem to be impaired, but primary responses, as tested in numerous immunization studies, are decidedly impaired. Certain protocols, especially those treating high-risk acute leukemias and neuroblastoma during induction therapy are intensive with high rates of sepsis, and may warrant trials of prophylactic IVGG. Similarly, some form of humoral prophylaxis is becoming an important part of the handling of the patient undergoing bone marrow transplantation not only to prevent bacterial sepsis but also to prevent cytomegalovirus (CMV) interstitial pneumonitis. A likely additional usage is gammaglobulin replacement for patients undergoing plasmapheresis, especially if performed multiple times. Finally, the broad spectrum of antibacterial and antiviral antibodies present in the preparations (such as anti-CMV, anti-Group B strep, and antiendotoxin) and the ease and safety of delivery allow the preparations to be used in situations where a hyperimmune preparation might be desired and/or where more than one pathogen is possible. In summary, IVGG is a treatment capable of safely conferring significant benefits to selected patients.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Intravenous usage of gammaglobulin: humoral immunodeficiency, immune thrombocytopenic purpura, and newer indications. 404 Jul 95

Circulating immune complexes were studied using 3.5% polyethyleneglycol precipitation in 312 children with various diseases whose ages ranged from 1 month to 14 years. One hundred and one patients (32.6%) were positive and the groups with the highest percentage were those with viral hepatitis (90%), sepsis (80.7%), collagen diseases (76.4%) and Schonlein-Henoch purpura (57.1%). We found immune complexes less frequently in idiopathic thrombocytopenic purpura than in published series of adult cases, possibly due to the fact that the diseases in children is due to a different pathogenetic mechanism. The composition of the immune complexes was tested by 1% agarose immunodiffusion against a panel of antisera. IgG and IgM were found most frequently, and IgA was very uncommon except in some cases of hepatitis. C4 was the most frequently found complement component, followed by C3. Important differences between the various diseases studied were noted. Our results are very similar to those previously published by other authors. Whereas serum autoantibodies and autoimmune diseases are less common in children than in adults, circulating immune complexes seem to have a similar frequency in children to that already reported for adults. It is difficult to assess the significance of circulating immune complexes. They might be (a) a mere "marker" of no pathogenic significance (b) a mechanism of tissue damage by intravascular deposition, or (c) they might interfere with the cell membrane receptors of macrophages, producing a defect in phagocytosis. However, we were unable to demonstrate an increased number of infections in these patients.
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PMID:[Incidence of circulating immune complexes in pediatric diseases. Comparative study with adults]. 645 Nov 57

The aim of this study was to determine if hearts removed from rats in sustained hypermetabolic sepsis possessed normal physiological reserves as defined by Frank-Starling curves. The resting cardiac output of these hearts is elevated in vivo and this is mediated by an increased heart rate. In this study mechanical function was assessed under in vitro conditions in which preload could be varied and controlled. Isolated perfused working hearts from septic animals showed depressed cardiac performance, both in terms of peak systolic pressure development and cardiac output, over a range of left atrial filling pressures. In spite of this depressed function, most hearts from septic animals were able to recover cardiac output and pressure development following an acute ischemic episode. Thus, a loss of myocardial reserve is demonstrable in hearts isolated from septic rats, even though mean arterial blood pressure, cardiac output, and coronary blood flow are well maintained in vivo. This dysfunction is primarily evidenced by an inability to respond appropriately to increased preloads.
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PMID:Depressed function of isolated hearts from hyperdynamic septic rats. 672 92

Between 1956 and 1981, 306 splenectomies for hematologic diseases were performed at the UCLA Medical Center. Of these operations, more than 75% were performed for therapeutic reasons to control anemia, thrombocytopenia, neutropenia, or painful symptoms of splenomegaly. Of the 65 patients who had idiopathic thrombocytopenic purpura, 77% showed an excellent response, and of the 39 patients who had hereditary spherocytosis, 90% responded. Other diseases with predictably good response rates were autoimmune hemolytic anemias, Felty's syndrome, and hairy cell leukemia. Forty patients with Hodgkin's disease had splenectomies for diagnostic purposes the last 10 years. The overall morbidity and mortality were 24% and 6%, respectively, the most common complications being pneumonia, wound infections, and local postoperative bleeding, and the most common cause of death being sepsis. The review supports the thesis that in carefully selected patients, therapeutic splenectomy can have desirable palliative effects and that diagnostic splenectomy has a sufficiently low risk to warrant its consideration in patients with Hodgkin's disease.
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PMID:Splenectomy for hematologic disease. The UCLA experience with 306 patients. 673 25

Of 478 patients who underwent splenectomy for hematologic disease between 1947 and 1978, 142 had thrombocytopenia as the dominant indication, while in 32 this was an associated preoperative factor. Of 120 patients with idiopathic thrombocytopenic purpura (ITP) 88% had long-term remission subsequent to removal of the spleen. Ninety percent of the responders had normal platelet counts at the end of 1 week subsequent to the operation. Six of 13 patients with thrombotic thrombocytopenic purpura (TTP) were long-term survivors and were considered cured. Thrombocytopenia, associated with a variety of disorders, as a dominant indication for splenectomy was improved significantly in the majority of cases. The same results were noted when thrombocytopenia was an associated factor. Postoperative death usually was related to intracerebral bleeding, and no increased incidence of thromboembolic disease or sepsis was noted. Electron microscopic evaluations of the spleens defined marked platelet sequestration in patients with ITP and TTP.
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PMID:Splenectomy for thrombocytopenia. 719 81

The construction of a Frank-Starling myocardial function curve relating heart work to left ventricular preload is clinically utilized to assess therapeutic protocols in critically ill patients. The pulmonary capillary wedge pressure (PWP) is the index of left ventricular filling pressure most frequently utilized as representative of left ventricular preload. The authors assessed the relationship between left ventricular preload measured as the left ventricular end-diastolic volume (LVEDV), and the PWP, in acutely ill patients with sepsis and cardiac disease. Within each group, no relationship was found between the LVEDV and the PWP; however, when omitting the effect of PEEP, a modest correlation was noted (r = 0.302; p less than 0.01). Of the left ventricular ejection fraction, LVEDV and PWP, the PWP accounted for less than 5% of the explained variance in the stroke volume index. The PWP is a poor predictor of left ventricular preload, probably because of abnormalities of left ventricular compliance in critically ill patients.
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PMID:Does the pulmonary capillary wedge pressure predict left ventricular preload in critically ill patients? 722 63

Autoimmune thrombocytopenic purpura (AITP) is generally a chronic disorder in affected adults. Twenty-five percent of these patients will become refractory to routine therapy (corticosteroids and splenectomy), as well as most other available agents. Intravenous pulse cyclophosphamide therapy was used to treat 20 patients with severe refractory AITP who had previously failed to achieve a sustained remission with a mean of 4.8 agents (range 2 to 8). Patients received 1 to 4 doses (mean 2.0) of 1.0 to 1.5 g/m2 intravenous cyclophosphamide per course. Of the 20 patients treated with pulse cyclophosphamide therapy, 13 patients (65%) achieved a complete response (CR), four (20%) a partial response (PR), and three patients (15%) failed to respond. Of the 13 complete responders, eight have remained in remission with stable platelet counts during followup intervals of 7 months to 7 years (median 2.5 years). Five patients developed recurrent AITP 4 months to 3 years following a CR. Of these, two patients responded to subsequent courses of pulse cyclophosphamide therapy with current remissions of 1 and 4 years. Of the four patients who obtained a PR, two remain in partial remission after 10 months and 4 years; one relapsed after 18 months and, after retreatment, is still in remission at 6 months. Of the patient characteristics examined, duration of disease was most strongly associated with response to pulse cyclophosphamide. Side-effects of treatment included neutropenia (three patients, one of whom developed staphylococcal sepsis), acute deep venous thrombosis (two patients), and psoas abscess (one patient). Intravenous pulse cyclophosphamide should be strongly considered in the treatment of patients with refractory AITP. There is a relatively low incidence of side-effects, and it can be administered easily on an out-patient basis.
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PMID:Pulse cyclophosphamide therapy for refractory autoimmune thrombocytopenic purpura. 779 54


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