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1/12. Fatal pneumococcal waterhouse-friderichsen syndrome.

    There have only been six deaths reported in the English literature due to disseminated intravascular coagulation and waterhouse-friderichsen syndrome complicating pneumococcemia in otherwise healthy persons with normal spleens. Four of these deaths occurred in children and two in adults. A case of fulminant pneumococcemia complicated by disseminated intravascular coagulation and waterhouse-friderichsen syndrome in an otherwise healthy adult with a normal spleen is presented. The case is typical of the course of fulminant pneumococcal sepsis and highlights some of the difficulties experienced by rural general practitioners and rural retrieval services. Mechanisms by which Pneumococcus can elicit fulminant sepsis are discussed, although there is no explanation as to why this may occur in the setting of normal splenic function.
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ranking = 1
keywords = intravascular coagulation, coagulation, intravascular
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2/12. waterhouse-friderichsen syndrome after infection with group A streptococcus.

    We report a case of waterhouse-friderichsen syndrome associated with group A streptococcus (GAS) toxic shock syndrome in a previously healthy man. The patient presented with neck pain and fevers of 2 days' duration. Computed tomography of the neck revealed a mass in the retropharyngeal space, suggesting an abscess. Despite prompt treatment with appropriate antibiotics, the patient experienced a fulminant course and died within 8 hours of presentation. Antemortem blood cultures grew GAS positive for exotoxins A, B, and C. Postmortem examination revealed bilateral adrenal hemorrhage, consistent with waterhouse-friderichsen syndrome. Immunohistochemical analysis of the adrenal glands revealed the presence of GAS antigens. However, no disseminated intravascular coagulation was evident. This case demonstrates that adrenal hemorrhage can occur without associated coagulopathy and may result directly from the action of bacterial toxins.
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ranking = 0.50861711617982
keywords = intravascular coagulation, coagulation, intravascular, coagulopathy
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3/12. Human recombinant activated protein c in meningococcal sepsis.

    A 19-year-old woman presented with purpura fulminans and septic shock; subsequently, progressive coagulopathy, widespread purpura fulminans associated with meningococcemia, severe shock, respiratory, and renal failure developed. This clinical course was associated with depletion of functional protein c levels to < 5%. We describe her clinical course and therapy with human recombinant activated protein c.
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ranking = 0.0086171161798224
keywords = coagulopathy
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4/12. OPSI (overwhelming postsplenectomy infection) syndrome: a case report.

    Splenectomized patients are likely to suffer from severe infections, such as sepsis and meningitis. This syndrome is called overwhelming postsplenectomy infection (OPSI) in europe and America. We present an adult case of OPSI syndrome, which occurred as respiratory insufficiency, and thrombocytopenia. The course is rapid, the clinical symptoms are serious, and the prognosis is very poor. Clinical examination showed cyanosis, mandibular hypertonia, psychomotor anxiety and purpura. Laboratory findings were thrombocytopenia, leukocytosis, hypoglycemia and altered coagulation parameters. A chest X-ray showed right pulmonary aspecific thickening. The autopsy findings occurred as waterhouse-friderichsen syndrome.
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ranking = 0.038161465391996
keywords = coagulation
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5/12. purpura fulminans and adrenal hemorrhage due to group Y meningococcemia in an elderly woman.

    A 70-year-old previously healthy woman was admitted with a 1-day history of malaise, sore throat, nausea, vomiting, rigors, and confusion. She was found to be in septic shock with purpura fulminans and disseminated intravascular coagulation. She died within 36 hours of admission. blood cultures grew neisseria meningitidis group Y. Necropsy revealed evidence of shock and bilateral adrenal hemorrhage.
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ranking = 0.5
keywords = intravascular coagulation, coagulation, intravascular
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6/12. Fatal pneumococcal bacteremia with disseminated intravascular coagulation and waterhouse-friderichsen syndrome in a vaccinated splenectomized adult. Case report.

    Fatal pneumococcal bacteremia, disseminated intravascular coagulation and Waterhouse Friderichsen syndrome in a vaccinated, splenectomized adult were caused by serotype 22F (Danish classification), which was not included in the vaccine. Revaccination with a 23-valent pneumococcal vaccine and antibiotic prophylaxis are advocated for patients who have undergone splenectomy.
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ranking = 2.5
keywords = intravascular coagulation, coagulation, intravascular
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7/12. waterhouse-friderichsen syndrome caused by haemophilus influenzae type b in an immunocompetent young adult.

    A previously healthy 19-year-old woman had a febrile illness with hypotension, progressive cyanosis, and an evolving petechial rash. Despite aggressive therapy in the face of shock and disseminated intravascular coagulation, the patient suffered a cardiac arrest and could not be resuscitated. haemophilus influenzae type b was cultured from the blood and echovirus 30 from the cerebrospinal fluid post mortem. Fulminant H influenzae type b infection in an immunocompetent adult is rare but should be recognized as a possible cause of the waterhouse-friderichsen syndrome.
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ranking = 0.5
keywords = intravascular coagulation, coagulation, intravascular
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8/12. Pneumococcal waterhouse-friderichsen syndrome despite a normal spleen.

    A previously fit 56 year old female presented with a rapidly progressive and fatal pneumococcal septicaemia with disseminated intravascular coagulation. Post-mortem studies confirmed a waterhouse-friderichsen syndrome and revealed an anatomically normal spleen; intracellular diplococci were seen within splenic macrophages providing evidence of normal splenic function. This appears to be only the second case of waterhouse-friderichsen syndrome due to pneumococcal septicaemia in a patient with a normal spleen.
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ranking = 0.5
keywords = intravascular coagulation, coagulation, intravascular
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9/12. Haemophilus influenzae meningitis and waterhouse-friderichsen syndrome in an adult.

    An otherwise healthy 36-year-old man had abdominal pain, vomiting, sepsis, and disseminated intravascular coagulation (DIC). Negative exploratory laparotomy was shortly followed by death. autopsy showed Haemophilus influenzae (type B) meningitis, multiple organ involvement with DIC, and bilateral adrenal hemorrhagic necrosis (waterhouse-friderichsen syndrome). This patient is the fourth reported adult with H influenzae meningitis and hemorrhagic infarction of the adrenals, and the first such patient with an apparent abdominal catastrophe.
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ranking = 0.5
keywords = intravascular coagulation, coagulation, intravascular
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10/12. Overwhelming post-splenectomy infection with plesiomonas shigelloides in a patient cured of Hodgkin's disease. A case report.

    The immune deficiencies of Hodgkin's disease persist to some degree even after the patients are clinically cured; these may be amplified by loss of splenic immunologic functions after staging laparotomy and splenectomy. The authors submit a case report wherein a bacterium of relatively low virulence, plesiomonas shigelloides, was associated with a rapidly fulminant septicemia, disseminated intravascular coagulation, waterhouse-friderichsen syndrome, and death in a splenectomized patient free of Hodgkin's disease for approximately five years. This emphasizes the need for prolonged observation, rapid diagnosis, and aggressive intervention in immunocompromised patients, especially those supposedly cured of previous hematologic malignancy.
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ranking = 0.5
keywords = intravascular coagulation, coagulation, intravascular
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