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 147,016 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The study objective was to describe the clinical, biologic, and hemodynamic features of adult overwhelming meningococcal purpura and to examine the prognostic factors by multivariate analysis at the time of admission to the intensive care unit. Thirty-five patients (greater than or equal to 13 years of age) with meningococcal infection, circulatory shock, and generalized purpuric lesions of abrupt onset were recorded in eight intensive care units from 1977 to 1989. The patients were young (mean age, 26.6 years; range, 13 to 68 years) and had been previously healthy. The female-to-male ratio was 3:1. Mortality was 54.3%, with most deaths occurring within the first 48 hours, usually secondary to irreversible shock with multiple organ failure. Ischemic complications (eight cases), prolonged heart failure (seven cases), and secondary septicemia (five cases) were the chief complications among survivors. Initial hemodynamic study after volume loading showed low stroke volume index (mean +/- SD, 29.4 +/- 13 mL/m2) and tachycardia (mean +/- SD, 138 +/- 16 beats per minute), a profile suggesting a greater myocardial depression than usually observed in gram-negative bacillary septic shock. Univariate prognostic analysis showed that four variables at the time of admission were associated with fatal outcome: a plasma fibrinogen level of 1.5 g/L or less, a factor V concentration of 0.20 or less, a platelet count lower than 80 x 10(9)/L, and a cerebrospinal fluid leukocyte count of 20 x 10(6)/L or less. Stepwise regression analysis showed that low fibrinogen level (less than or equal to 1.5 g/L) was the sole adverse prognostic variable (odds ratio = 2, 95% confidence interval, 1.5 to 2.7). Adult overwhelming meningococcal purpura is still associated with high mortality and morbidity. Low fibrinogen level at time of admission may permit early recognition of the most severely ill patients.
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PMID:Adult overwhelming meningococcal purpura. A study of 35 cases, 1977-1989. 199 58

Plasma protein C exerts anticoagulatory effects by inactivating factors V and VIII. Hereditary protein C deficiency is transmitted as an autosomal dominant disorder. Homozygous individuals usually develop purpura fulminans as newborns; heterozygous protein C-deficient individuals are at increased risk for venous thrombosis and pulmonary embolism. However, arterial thrombosis has been only rarely observed. We describe a young patient with heterozygous protein C deficiency who experienced a severe stroke due to thrombotic occlusion of the left middle cerebral artery.
Stroke 1990 Jul
PMID:Ischemic stroke due to protein C deficiency. 219 15

Cerebral aspergillosis is one of the most common mycotic infections in the central nervous system causing different clinical features such as brain abscess, granuloma, meningitis, and encephalitis. Cerebral aspergillosis, however, may lead to a cerebral vascular accident such as intracranial hemorrhage or cerebral infarction. In this report, we present two patients with cerebral aspergillosis accompanied by intracranial hemorrhage. A total of 124 reported cases of cerebral aspergillosis are reviewed to ascertain the pathogenesis of the associated vascular lesion. The first patient was a 9-year-old girl, who developed drowsiness with a headache during the medical treatment for acute myelocytic leukemia. CT disclosed subarachnoid and intraventricular hemorrhage. The autopsy revealed that the aspergillus arteritis was the cause of repeated hemorrhage. The second patient was a 15-year-old boy with allergic purpura and renal failure, who suddenly developed a stupor with convulsive seizure. CT disclosed an intracerebral hemorrhage in the right parieto-occipital area. The patient gradually deteriorated and died in spite of the surgical removal of the hematoma. The autopsy revealed that the hemorrhage was caused by the aspergillus arteritis. Cerebral aspergillosis has two routes of infection to the central nervous system: hematogenous dissemination from the distant site (usually the lung) and direct extension from the contiguous site (usually the paranasal sinuses or orbit). The primary mechanism of neuropathology is different between these two types. Primary cerebral arteritis is most often seen in patients with the former type, whereas primary basal meningitis occurs in the latter. The incidence of clinico-pathological features is different between hematogenous dissemination type and direct extension type.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cerebral aspergillosis as a cerebral vascular accident]. 339 19

Anti-phospholipid syndrome, originally called anticardiolipin syndrome, is characterized by the presence of anti-phospholipid antibodies and a marked tendency to both arterial and venous thrombosis. The little information available on the implications of this syndrome for anesthesia derive from the recent description of the disease. We describe 2 patients, each with 1 of the 2 forms of antiphospholipid syndrome that have been described to date, and each needing surgery for a different reason. The first was a 24-year-old woman who was admitted to the hospital with diarrhea, fever and metrorrhagia in her fifth month of pregnancy. Blood tests revealed a weakly positive title of anti-cardiolipin antibodies. Steroid and antiplatelet therapy was begun. Delivery was at 35 weeks by elective cesarean with epidural anesthesia due to oligoamnios. The second patient was 52-year-old woman with a history of 13 miscarriages, cerebrovascular accident and deep venous thrombosis. She had been diagnosed as having systemic lupus erythematosus with anti-phospholipid syndrome and was receiving corticoid and antiplatelet therapy. She had been admitted on 2 occasions for epistaxis, purpura in the lower extremities and severe thrombocytopenia. The last condition did not respond well to immunosuppressant therapy and a splenectomy was therefore performed with the patient under general anesthesia. In both cases recovery was good in spite of the serious complications of anesthetic management.
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PMID:[Anesthetic implications in antiphospholipid syndrome. 2 clinical cases]. 779 18

We report six cases of protein S deficiency secondary to varicella. Five cases were complicated by thrombotic and vascular events, namely purpura fulminans and necrotic vasculitis, deep vein thrombosis and stroke. Two cases were associated with protein C deficiency and one case revealed a heterozygous factor XII deficiency. The underlying mechanism of this acquired protein S deficiency is unclear but could be related to a direct effect of zoster virus.
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PMID:Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children. 795 22

A decrease in levels of circulating anticoagulant protein C has been shown to occur following autologous BMT, and this deficiency may contribute to a hypercoagulable state placing patients at risk for thromboembolic events. We report four patients who suffered a variety of thrombotic complications following BMT (non-bacterial thrombotic endocarditis, superior vena cava thrombosis, thrombotic stroke, purpura fulminans, small bowel infarction secondary to diffuse microvascular thrombosis), which were preceded by or temporally related to decreased levels of protein C. Treatment with fresh frozen plasma (FFP) led to slight, temporary increases in protein C levels but infusions of FFP did not prevent either death or extension of the thrombus in these four cases, suggesting the need for higher protein C doses and/or concomitant anticoagulation. Though no direct causal relationship between these thrombotic complications and the protein C deficiency can be proved, a generalized hypercoagulable state caused by protein C deficiency may have contributed to the development, severity or progression of these complications.
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PMID:Thrombotic complications of BMT: association with protein C deficiency. 843 11

Individuals vary considerably in their susceptibility to infection and in their ability to recover from apparently similar infectious processes. These differences can be partially explained by polymorphisms of the genes encoding proteins involved in mediating and controlling the innate immune response, the inflammatory cascade, coagulation, and fibrinolysis. It is evident from experimental studies that dysregulation of the coagulation system, which is characteristic of the pathophysiology of septic shock (a procoagulant and antifibrinolytic state), contributes to systemic inflammation and death in sepsis. Several genetic variations in proteins that increase coagulation or impair anticoagulation and fibrinolysis have been described. Thus, polymorphisms have been reported in prothrombin, fibrinogen, factor V, tissue factor, endothelial protein C receptor, and plasminogen activator inhibitor-1 genes. Some of them are associated with an increased risk of pulmonary emboli, acute myocardial infarction, stroke, and severe sepsis. Hence, the deletion polymorphism (4G) within the promoter region of the plasminogen activator inhibitor-1 gene leads to impaired fibrinolysis and influences the severity and outcome of meningococcal disease and the susceptibility to severe sepsis and multiple organ failure after trauma. The factor V Leiden mutation is associated with thrombotic events and has been reported to exacerbate purpura fulminans in meningococcal infection. Surprisingly, this genetic variant seems to provide a survival advantage in severe sepsis, underlying the extreme complexity of the interaction between inflammation and coagulation. The study of genetic polymorphisms might provide important insights into the pathogenesis of severe sepsis and could make it possible to identify individuals who are at risk of developing or dying of severe infections. As genetic associations are discovered, medical practice can become more preemptive, using the predictive ability of genetics to anticipate disease and recommend therapy.
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PMID:Importance of hemostatic gene polymorphisms for susceptibility to and outcome of severe sepsis. 1511 37

Pituitary apoplexy can occur as a complication of idiopathic thrombocytopenic purpura. We report here a new case of such association. A male patient aged 59 years, complaining of decreased libido for one year, was referred to the emergency department for purpura and severe thrombocytopenia (4000 platelets/mm3). 24 hours after the cutaneous rash the patient presented with clinical symptoms of bilateral cavernous sinus compression comprising ptosis, bilateral ophtalmoplegia and right supraorbital hypoesthesia. Cranial CT scan showed an enlarged sella and a pituitary mass with signs of intrapituitary haemorrhage. Hormonal evaluation showed hyperprolactinemia (50 ng/mL) and hypopituitarism, and the patient needed substitution with hydrocortisone and levothyroxine. Immunoglobulins and corticosteroids were given to the patient to treat thrombocytopenia, then worsening of neurological and ophtalmological symptoms led to pituitary surgery. Histopathological examination found necrotical pituitary tissue. Immunostaining with an anti-prolactin antibody was positive in several groups of cells. Neurological symptoms subsided and thrombocytopenia was corrected by treatment. In conclusion, we report a case of pituitary apoplexy due to severe thrombocytopenia occurring as a complication of a preexisting macroprolactinoma.
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PMID:Pituitary apoplexy and idiopathic thrombocytopenic purpura: a new case and review of the literature. 1632 68

Pregnancy is an initiating event for acute thrombotic thrombocytopaenic purpura (TTP). There is a high risk of relapse during pregnancy and of foetal morbidity. We describe five cases with successful maternal and foetal outcomes in patients with a history of TTP. Cases 1 and 2 presented with TTP in their first pregnancy and had second-trimester foetal losses. Case 3 had four TTP relapses and soon after achievement of clinical remission became pregnant. Case 4 presented with TTP and left-sided stroke in pregnancy. ADAMTS-13 activity was less than 5% at presentation in four patients and prior to therapy during pregnancy in cases 1-4. Case 5, who had a single acute episode of TTP and became pregnant 6 years later, had normal ADAMTS-13 activity throughout pregnancy. Only case 3 had evidence of an inhibitor on mixing studies. All five patients underwent close haematological and obstetric monitoring and continued low-dose aspirin throughout pregnancy. Patients 1-4 had regular plasma exchange and received low molecular weight heparin during pregnancy. Patient 4 also received rituximab during the third trimester with no observed maternal or neonatal toxicity. Live healthy infants were delivered in all five cases in the third trimester. These findings suggest that successful pregnancy outcome is achievable in patients with a history of TTP and that patients with severely reduced ADAMTS-13 activity at the onset of pregnancy, necessitates regular plasma exchange during pregnancy.
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PMID:Successful management of pregnancy in women with a history of thrombotic thrombocytopaenic purpura. 1690 49

Protein C is best known for its mild deficiency associated with venous thrombosis risk and severe deficiency associated with neonatal purpura fulminans. Activated protein C (APC) anticoagulant activity involves proteolytic inactivation of factors Va and VIIIa, and APC resistance is often caused by factor V Leiden. Less known is the clinical success of APC in reducing mortality in severe sepsis patients (PROWESS trial) that gave impetus to new directions for basic and preclinical research on APC. This review summarizes insights gleaned from recent in vitro and in vivo studies of the direct cytoprotective effects of APC that include beneficial alterations in gene expression profiles, anti-inflammatory actions, antiapoptotic activities, and stabilization of endothelial barriers. APC's cytoprotection requires its receptor, endothelial cell protein C receptor, and protease-activated receptor-1. Because of its pleiotropic activities, APC has potential roles in the treatment of complex disorders, including sepsis, thrombosis, and ischemic stroke. Although much about molecular mechanisms for APC's effects on cells remains unclear, it is clear that APC's structural features mediating anticoagulant actions and related bleeding risks are distinct from those mediating cytoprotective actions, suggesting the possibility of developing APC variants with an improved profile for the ratio of cytoprotective to anticoagulant actions.
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PMID:The cytoprotective protein C pathway. 1711 Apr 53


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