Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0243026 (sepsis)
52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Each year between 71,000 and 142,000 cases of septic shock are estimated to occur. Shock is a derangement in the homeostatic mechanisms of the body secondary to decreased tissue perfusion. The proper treatment of septic shock is based on complete understanding of the altered physiologic events, and, consequently, adequate therapy for shock should be approached from the standpoint of applied physiology in the clinical setting. Currently, sepsis is seen in the patients suffering multiple trauma or burns, undergoing immunosuppressive therapy for cancer or connective tissue disease, or who have undergone organ transplants. The initial therapy needs to consider carefully whether subtle clinical parameters are consistent with sepsis. Time and effort should be given to the prevention of septic shock. Good drainage procedures, proper nutritional therapy, and hydration needs to be maintained at all times. An early aggressive approach with regard to diagnosis can beneficially influence the outcome of infection by reducing the duration and complexity of therapy. Focus is on bacteriology and antimicrobial therapy, hemodynamic and fluid therapy, metabolic and nutritional aspects, complications, and steroids. 4 major complications of sepsis are abnormalities in the clotting pathway and changes in the renal, pulmonary, and cardiovascular systems. Steroids have been recommended for septic shock, for they are stabilizing to the lysosomal membranes in the splanchnic circulation. Experimentally, this has led to a decreased production of myocardial depressant factor. To use steroids properly large doses should be administered early in the shock state.
...
PMID:Therapy for septic shock. 10 43

We examined the cardiovascular and metabolic response to RBC transfusion in patients with circulatory shock after volume resuscitation. Data were analyzed from 36 transfusions in 32 patients who were undergoing continuous hemodynamic monitoring. Transfusions were administered for moderate to severe anemia, mean Hgb 8.3 g/dl. The diagnosis were sepsis (19/36), cardiogenic shock (14/36), connective tissue disease (2/36), and severe hypocalcemia (1/36). Benefit from transfusion was defined as an improvement in tissue oxygen utilization (increased oxygen consumption [VO2] or decreased lactate), a decrease in myocardial VO2 (MAP x HR), or a decrease in myocardial work (left ventricular work index). Mean transfusion volume was 577 ml over 4.5 h. Hgb and oxygen delivery (DO2) increased by 27% and 28%, respectively, while pulmonary artery wedge pressure and cardiac index were unchanged. No significant change was noted in VO2, or lactate, after augmentation of red cell mass. An increase occurred in myocardial work indices and MAP x HR. No changes were identified when subgroups were analyzed based on diagnosis, pretransfusion Hgb, lactate, or VO2 levels. We conclude that selective increase in DO2 by augmentation of RBC mass and oxygen-carrying capacity did not improve the shock state in these volume-resuscitated patients, regardless of the etiology of the shock.
...
PMID:Cardiovascular and metabolic response to red blood cell transfusion in critically ill volume-resuscitated nonsurgical patients. 239 7

Scleromyxedema is a rare fibromucinous connective tissue disorder characterized by papular skin lesions associated with sclerosis and a serum monoclonal gammopathy. Little is known about either the natural history or the systemic manifestations of this disease. We reviewed the medical records of 19 patients with biopsy-proven scleromyxedema seen from 1950 to 1985 for evidence of systemic disease. There were 10 males and 9 females with a median age at diagnosis of 53 years. Monoclonal gammopathy was present in 13 patients. Eight patients complained of dysphagia; 3 had proximal esophageal dysfunction and 1 had total esophageal aperistalsis on barium swallow. Proximal muscle weakness was noted in 5, with an inflammatory myopathy in 3. Six patients complained of dyspnea on exertion. Of these, 5 had reduced diffusing capacity, 3 had reduced volumes, and 2 developed cor pulmonale. Pathologic changes characteristic of "scleroderma kidney" were demonstrated in 1 patient at postmortem. One patient had Raynaud's phenomenon and 2 had arthralgias/arthritis with noninflammatory synovial fluids. Although 8 of 12 patients treated with melphalan noted regression of their skin changes, no consistent improvement in the extracutaneous manifestations was demonstrated. Furthermore, 2 patients died of sepsis related to melphalan-induced myelosuppression, and 4 developed hematological malignancies following melphalan therapy. In conclusion, systemic manifestations in scleromyxedema are more prevalent than previously recognized, and can resemble those of scleroderma. Significant toxicity occurred with the use of alkylating agents in these patients, with treatment-related complications developing in 45% of patients treated with melphalan. The lack of definitive data regarding the natural history of this disease complicates the question of optimal therapy, but the use of alkylating agents should be reserved for those patients with severe debilitating skin disease.
...
PMID:Scleromyxedema: a scleroderma-like disorder with systemic manifestations. 333 81

Twenty-one patients with severe Raynaud's phenomenon were treated on 29 occasions with prostaglandin E1 (PGE1), a potent vasodilator and pyrogen. A history of finger sepsis or necrosis was absent in 8 (group I) and present in 13 (group II). Three group I and eight group II patients had an associated connective tissue disease, and previously eight upper limbs had been sympathectomized in six group I patients and 14 upper limbs in eight group II patients. A total of 12 fingers had been amputated in six group II patients. Treatment comprised antibiotics for sepsis, PGE1 intravenously for 72 hours, and subsequent surgical debridement of septic and necrotic tissue in 30 fingers of eight group II patients under general anesthesia. Finger skin temperature measured half-hourly in a temperature-controlled ward cubicle (23.7 degrees +/- 0.7 degrees C), Doppler-detectable digital arterial flow, and finger/brachial systolic pressure index with local finger cooling to 10 degrees C were not improved by the administration of 0.9% saline for 72 hours, but were all significantly improved after PGE1 administration. Finger skin temperature was significantly elevated 11 weeks after treatment. The symptoms did not improve after PGE1 administration in group I patients but did improve in 12 of 13 group II patients. No finger deteriorated, and all debrided fingers healed after surgery. Nail bed removal in 11 fingers met with patient approval and prevented recurrent sepsis and necrosis. PGE1 provides a means of increasing finger blood flow during acute exacerbations of finger sepsis and necrosis; unlike sympathectomy, it is a minor procedure without prolonged side effects and is repeatable.
...
PMID:Prostaglandin E1 in severe Raynaud's phenomenon. 689 Jul 19

Extravascular fibrin formation and dissolution is a pivotal event in numerous inflammatory and malignant diseases. In inflammatory cells such as monocytes/macrophages, neutrophil granulocytes appear to interact intimately with hemostasis and regulate the activity of the cascade systems of coagulation and fibrinolysis. Proteases such as neutrophil elastase are thought to influence components of hemostasis, and furthermore provide an alternative pathway of fibrinolysis. Histological, experimental, and clinical data suggest that extravascular fibrinolysis, mediated both by the plasmin system and by proteases like neutrophil elastase, is a prominent finding in various diseases such as lung cancer, chronic inflammatory bowel disease, vasculitis and connective tissue disease, bacterial sepsis, and septic shock.
...
PMID:The role of inflammatory cells and their proteases in extravascular fibrinolysis. 912 14

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder, a group that includes Ehlers-Danlos syndrome, Marfan's syndrome and pseudoxanthoma elasticum. OI is a heterogeneous disease of collagen I biosynthesis characterized by variable clinical phenotypes, including skeletal and cardiovascular manifestations. A 65-year-old man with OI who had extensive prior successful cardiac valve surgeries is described. He survived for 18 years after his initial valve surgery, but died of multiorgan failure and sepsis after repair of a spontaneous type A aortic dissection. This is the fourth reported case of aortic dissection secondary to OI and illustrates the extensive cardiovascular pathology associated with OI. Aggressive management of arterial dissection risk factors, such as systemic arterial hypertension, is advocated for patients with OI.
...
PMID:Aortic dissection: a rare complication of osteogenesis imperfecta. 1052 81

The patient was a 23-year-old woman with HTLV-I carrier and was diagnosed as pityriasis rubra pilaris soon after her birth. In November 1990 at her age of 16, she began to have fever and polyarthralgia which were not improved despite the administration of antibiotics. Her laboratory findings showed the positive antinuclear antibody and anti-RNP antibody suggesting one of collagen diseases. A tentative diagnosis as unclassified connective tissue disease (UCTD) was made since her symptoms and laboratory findings were not satisfied with any criteria for rheumatic disorders. The steroid therapy was started in February 1991 and showed a good response. On April 9th, 1996, however, she was admitted to our hospital because of recurrence of high fever and chills. This time, she had sepsis because of the evidence that Enterococcus faecalis was detected in blood culture. Although her condition was improved by antibiotics on June 4th 1996, high fever re-appeared and followed by convulsion and disseminated intravascular coagulation. After the doses of prednisolone per day was increased to 40 mg with antibiotics and anticoagulant, her condition gradually improved. This patient was a rare case of pityriasis rubra pilaris associated with UCTD and sepsis in clinical course.
...
PMID:[A case of pityriasis rubra pilaris associated with unclassified connective tissue disease and sepsis in clinical course]. 1115 93

Pulmonary arterial hypertension is a life-threatening disease for which continuous intravenous prostacyclin has proven to be effective. However, this treatment requires a permanent central venous catheter with the associated risk of serious complications such as sepsis, thromboembolism, or syncope. Treprostinil, a stable prostacyclin analogue, can be administered by a continuous subcutaneous infusion, avoiding these risks. We conducted a 12-week, double-blind, placebo-controlled multicenter trial in 470 patients with pulmonary arterial hypertension, either primary or associated with connective tissue disease or congenital systemic-to-pulmonary shunts. Exercise capacity improved with treprostinil and was unchanged with placebo; the between treatment group difference in median six-minute walking distance was 16 m (p = 0.006). Improvement in exercise capacity was greater in the sicker patients and was dose-related, but independent of disease etiology. Concomitantly, treprostinil significantly improved indices of dyspnea, signs and symptoms of pulmonary hypertension, and hemodynamics. The most common side effect attributed to treprostinil was infusion site pain (85%) leading to premature discontinuation from the study in 8% of patients. Three patients in the treprostinil treatment group presented with an episode of gastrointestinal hemorrhage. We conclude that chronic subcutaneous infusion of treprostinil is an effective treatment with an acceptable safety profile in patients with pulmonary arterial hypertension.
...
PMID:Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. 1189 47

"It is quite useless to argue the questions concerning the development of intimal scleroses if we study and discuss the late stages of the disease alone. If we wish to gain insight into the complex question of arterio-sclerosis we must attempt to follow the lesion from its earliest beginning" (Klotz and Manning, J Path Bact 1911: 16; 211-20). Over thirty years ago Boneu and colleagues published a report of raised levels of plasma von Willebrand factor (vWf) in patients with arteritis, diabetes and sepsis. They concluded that raised levels of this molecule indicate endothelial damage, and may possibly be a contributory factor in thrombosis in arterial disease. The former aspect of this conclusion is now accepted, and numerous studies on the risk factors for atherosclerosis provide mechanisms for this damage. Other studies have demonstrated raised levels in cancer and in connective tissue disease. Numerous long-term follow-up studies have also demonstrated that increased vWf predicts major cardiovascular end points. However, the link between these studies, and the latter aspect of Boneu's conclusion, that raised vWf contributes to thrombosis is, although attractive, nevertheless unproven. Despite this, vWf remains the most important plasma marker of endothelial damage/dysfunction and as such attracts clinical attention.
...
PMID:Plasma von Willebrand factor, thrombosis, and the endothelium: the first 30 years. 1654 61

Systemic sclerosis (scleroderma) is a connective tissue disorder of unknown aetiology characterised by immune abnormalities, which has been related to an increased risk of malignancy. Lung cancer is the most prevalent among these patients. We present a clinical case of a 42 years old non smoker female with systemic sclerosis. She presented progressive general health status worsening and an exudative pleural effusion, with no evidence of infection or malignancy. Chest high resolution computed tomography revealed pulmonary fibrosis. Bronchoscopy, bronchial and bronchoalveolar lavage were normal. Extra- -pulmonary malignancies were excluded. Pleural nodularities were found on pleuroscopy and the biopsy was compatible with lung adenocarcinoma. Chemotherapy was then started, which complicated with febrile neutropenia, sepsis and patient death 12 days after. The purpose of this case report is to bring attention to the relationship, although rare, between systemic sclerosis and lung cancer, stressing the importance of adequate pulmonary surveillance in these patients.
...
PMID:[Lung adenocarcinoma associated with systemic sclerosis: a case report]. 1914 91


1 2 Next >>