Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 20-year-old woman who developed meningococcemia. The patient developed fever, vomiting and skin rash, then was sent to our hospital for shock. Physical and laboratory examination revealed septic shock and DIC. Her blood culture grew Neisseria meningitidis (W135). She recovered promptly with PCG, gabexate mesilate and intensive care for shock. Hemolytic activities of the patient's complement were less than 12/CH50 during the course. Screening for each component of the complements suggested that this patient had deficiency of C7. Meningococcal disease has seldom seen in Japan. Early recognition is essential so that appropriate antibiotic therapy and supportive care can be promptly started because shock and death may ensure within hours after onset of symptoms.
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PMID:[Meningococcemia associated with C7 deficiency]. 1106 66

Severe meningococcemia, which is associated with hemodynamic instability, purpura fulminans and disseminated intravascular coagulation, still has a high mortality rate, and patients who survive are often left invalids because of amputations and organ failure. Clinical studies have shown that levels of protein C are markedly decreased in patients with severe meningococcemia and that the extent of the decrease correlates with a negative clinical outcome. There is a growing body of data demonstrating that activated protein C, in addition to being an anticoagulant, is also a physiologically relevant modulator of the inflammatory response. The dual function of protein C may be relevant to the treatment of individuals with severe meningococcal sepsis. In the present review we give a basic overview of the protein C pathway and its anticoagulant activity, and we summarize experimental data showing that activated protein C replacement therapy clearly reduces the mortality rate for fulminant meningococcemia.
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PMID:Protein C replacement in severe meningococcemia: rationale and clinical experience. 1130 70

The clinical-and-pathogenetic significance of protein C was dynamically investigated within the infection processes in 23 patients (mean age 44 years) with meningococcemia. A reliably lower concentration of protein C in blood plasma (mean 37.8%) was registered versus the normal parameters (N = 70-130%) at exacerbation irrespective of a disease outcome. Leyden mutation was detected in 30.5% of patients. The presence of the above genetic defect denoted a predisposition to a severe disseminated intravascular coagulation (DIC) syndrome in patients of the studied group. A lower concentration of protein C was detected in blood plasma of patients with meningococcemia; it correlated with severity of the infection process and with the development of organic malfunction. The study of the concentration of protein C enabled an evaluation of the functional condition of anticoagulation mechanisms in the DIC development and can be regarded as an important extra criterion for the evaluation of thrombohemorrhagic syndrome in patients with meningococcemia.
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PMID:[A clinical and pathothysiological study of protein C in patients with generalized meningococcosis]. 1532 52

Sepsis-induced purpura fulminans is a rare but life-threatening disorder, characterized by hemorrhagic infarction of the skin caused by disseminated intravascular coagulation and dermal vascular thrombosis. The pathogenesis is linked to enhanced expression of the natural procoagulants and depletion of the natural anticoagulant proteins particularly protein C. Meningococcal sepsis is the most common cause, followed by pneumococcal sepsis in adults. The syndrome is associated with more than 50% mortality secondary to multiple organ dysfunction syndrome and is accompanied by long-term morbidity. Necrotic lesions usually progress to distal ischemia, and skin grafting and extremities or limb amputation are often required. Early antibiotic administration and intensive care management according to the recommendations of severe sepsis and shock is crucial for patients' survival. Adjuvant therapies against inflammatory and coagulation cascades and augmenting fibrinolysis are still controversial and need further assessment. Among them activated protein C and supplementation therapy have given promising results.
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PMID:Purpura fulminans in sepsis. 1717 Jun 24

Sepsis is a potentially life-threatening complication of an infection where cutaneous lesions often represent one of the early signs. A myriad of microorganisms including bacteria, fungi, yeasts, viruses, protozoas, helminths and algae can be implicated. A broad spectrum of clinical and histopathologic findings can be observed in the skin and the common denominator is a thrombotic vasculopathy. The pathogenesis of cutaneous septic vasculitis (SV)/vasculopathy is complex and includes five main mechanisms: disseminated intravascular coagulation, direct invasion and occlusion of blood vessel walls by microorganisms, hypersensitivity reaction with immune complex deposition into blood vessel walls, embolism from a distant infectious site and vascular effects of toxins. Herein we describe the clinicopathologic findings of some selected cases of SV recently observed in our hospital, including purpura fulminans, necrotizing fasciitis, cutaneous meningococcemia, malignant syphilis and disseminated alternaria infection. Histopathologically, a wide spectrum of histopathologic changes was observed in skin specimens from the various entities, involving the intensity and composition of the inflammatory infiltrate, the degree of vascular changes and the presence of microorganisms, that ranged from a predominant not inflammatory, thrombotic-occlusive vasculopathy in purpura fulminans to leukocytoclastic vasculitis like changes in cutaneous meningococcemia to a dermal angiomatosis-like pattern in disseminated Alternaria infection. The different pathologic presentations may be related to the microorganism involved, the main pathogenetic mechanism that induced the vascular injury and the individual immunologic burden. Early skin biopsy for histopathologic examination and microbiologic culture is a cornerstone in the diagnosis of life-threatening diseases that present with cutaneous septic vasculitis. Ancillary techniques, such as immunohistochemistry and polymerase chain reaction are additional novel and helpful tools to identify pathogens, leading to definite diagnosis in cases with challenging or ambiguous clinical and/or pathologic findings.
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PMID:Septic vasculitis and vasculopathy in some infectious emergencies: the perspective of the histopathologist. 2559 69

We mainly refer to the acute setting of meningococcemia. Meningococcemia is an infection caused by Neisseria meningitidis, which has 13 clinically significant serogroups that are distinguishable by the structure of their capsular polysaccharides. N. meningitidis, also called meningococcus, is a Gram-negative, aerobic, diplococcus bacterium. The various consequences of severe meningococcal sepsis include hypotension, disseminated intravascular coagulation (DIC), multiple organ failure, and osteonecrosis due to DIC. The gold standard for the identification of meningococcal infection is the bacteriologic isolation of N. meningitidis from body fluids such as blood, cerebrospinal fluid (CSF), synovial fluid, and pleural fluid. Blood, CSF, and skin biopsy cultures are used for diagnosis. Meningococcal infection is a medical emergency that requires antibiotic therapy and intensive supportive care. Management of the systemic circulation, respiration, and intracranial pressure is vital for improving the prognosis, which has dramatically improved since the wide availability of antibiotics. This review of the literature provides an overview of current concepts on meningococcemia due to N. meningitidis infection.
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PMID:Meningococcemia in Adults: A Review of the Literature. 2698 70

Febrile patient with thrombocytopenia is commonly encountered by physicians especially during monsoon and perimonsoon period. Infections with protozoa, bacteria and viruses can cause thrombocytopenia with or without disseminated intravascular coagulation. Commonly dengue, malaria, scrub typhus and other rickettsial infections, meningococci, leptospira and certain viral infections present as fever with thrombocytopenia. Occasionally these patients can go on to develop a stormy course with multiorgan dysfunction requiring intensive care unit admission associated with high morbidity and mortality.1,2 Infections cause decrease in platelet count both due to effects on platelet production and platelet survival.3 Thrombocytopenia in bacterial infections can occur as a part of sepsis with disseminated intravascular coagulation. Patients with sepsis may also develop hemophagocytic histiocytosis with phagocytosis of platelets and leucocytes in the bone marrow histiocytes. Both Gram-positive and Gram-negative bacterial infections can lead to sepsis. Elevated platelet-associated IgG has been implicated. Platelets tend to adhere to damaged vascular surfaces in meningococcemia.
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PMID:Infections and Thrombocytopenia. 2773 Jul 74

Meningococcemia is notorious for evasion of the host immune system and its rapid progression to fulminant disease, and serves as a unique model for pediatric sepsis. Illness severity is determined by complex interplays among host, pathogen, and environment. The inflammatory host response, including proinflammatory and anti-inflammatory responses in innate and adaptive immunity, skews toward a proinflammatory state. This leads to endothelial dysfunction and activation of the hemostatic response, which may lead to disseminated intravascular coagulation. This article reviews the pathogenesis of sepsis, in particular the inflammatory and hemostatic response in meningococcal sepsis.
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PMID:The Inflammatory and Hemostatic Response in Sepsis and Meningococcemia. 3217 20

Neisseria meningitidis-induced acute systemic meningococcal disease is an emergency and a fatal condition that has a high mortality rate. In patients with a fulminant infection, a maculopapular petechial eruption, purpura fulminans, or an ecchymotic lesion are worrisome signs reflecting disseminated intravascular coagulation (DIC) and hint at Waterhouse-Friderichsen syndrome (WFS). Here, we describe a rare case of a patient with a fulminant Neisseria meningitidis-induced acute systemic meningococcal disease presenting with high-grade fever without meningitis symptoms. Fatal septicemia with DIC and multiple organ failure was noted. WFS was chiefly suspected. We highlight the clinical features and pathogenesis of Neisseria meningitidis-induced meningococcemia and WFS. We propose that they should be kept in mind, especially in patients presenting with a petechial eruption and purpura fulminans.
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PMID:Neisseria meningitidis Induced Fatal Waterhouse-Friderichsen Syndrome in a Patient Presenting With Disseminated Intravascular Coagulation and Multiple Organ Failure. 3219 3


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