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Query: UMLS:C0012739 (disseminated intravascular coagulation)
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Between 1 September and 24 October 1976, 318 cases of acute viral haemorrhagic fever occurred in northern Zaire. The outbreak was centred in the Bumba Zone of the Equateur Region and most of the cases were recorded within a radius of 70 km of Yambuku, although a few patients sought medical attention in Bumba, Abumombazi, and the capital city of Kinshasa, where individual secondary and tertiary cases occurred. There were 280 deaths, and only 38 serologically confirmed survivors.The index case in this outbreak had onset of symptoms on 1 September 1976, five days after receiving an injection of chloroquine for presumptive malaria at the outpatient clinic at Yambuku Mission Hospital (YMH). He had a clinical remission of his malaria symptoms. Within one week several other persons who had received injections at YMH also suffered from Ebola haemorrhagic fever, and almost all subsequent cases had either received injections at the hospital or had had close contact with another case. Most of these occurred during the first four weeks of the epidemic, after which time the hospital was closed, 11 of the 17 staff members having died of the disease. All ages and both sexes were affected, but women 15-29 years of age had the highest incidence of disease, a phenomenon strongly related to attendance at prenatal and outpatient clinics at the hospital where they received injections. The overall secondary attack rate was about 5%, although it ranged to 20% among close relatives such as spouses, parent or child, and brother or sister.Active surveillance disclosed that cases occurred in 55 of some 550 villages which were examined house-by-house. The disease was hitherto unknown to the people of the affected region. Intensive search for cases in the area of north-eastern Zaire between the Bumba Zone and the Sudan frontier near Nzara and Maridi failed to detect definite evidence of a link between an epidemic of the disease in that country and the outbreak near Bumba. Nevertheless it was established that people can and do make the trip between Nzara and Bumba in not more than four days: thus it was regarded as quite possible that an infected person had travelled from Sudan to Yambuku and transferred the virus to a needle of the hospital while receiving an injection at the outpatient clinic.Both the incubation period, and the duration of the clinical disease averaged about one week. After 3-4 days of non-specific symptoms and signs, patients typically experienced progressively severe sore throat, developed a maculopapular rash, had intractable abdominal pain, and began to bleed from multiple sites, principally the gastrointestinal tract. Although laboratory determinations were limited and not conclusive, it was concluded that pathogenesis of the disease included non-icteric hepatitis and possibly acute pancreatitis as well as disseminated intravascular coagulation.This syndrome was caused by a virus morphologically similar to Marburg virus, but immunologically distinct. It was named Ebola virus. The agent was isolated from the blood of 8 of 10 suspected cases using Vero cell cultures. Titrations of serial specimens obtained from one patient disclosed persistent viraemia of 10(6.5)-10(4.5) infectious units from the third day of illness until death on the eighth day. Ebola virus particles were found in formalin-
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PMID:Ebola haemorrhagic fever in Zaire, 1976. 30 56

Seven patients, aged 12 to 19 years, had atypical measles. Prodromal symptoms of fever, malaise, myalgia, headache, nausea, and vomiting were commonly followed by coryza, sore throat, conjunctivitis, photophobia, nonproductive cough, and pleuritic pain. The characteristic rash was erythematous, maculopapular, and progressed frequently to vesicular, petechial, or purpuric lesions. It initially involved palms and soles with subsequent spread to proximal extremities and the trunk, sparing the face. Six of six chest roentgenograms showed infiltrates. Findings not previously described in atypical measles included liver enzyme elevations, thrombocytopenia, disseminated intravascular coagulation, possible transmission among three siblings, and suspected cardiac involvement. Measles complement fixation titers compatible with recent infection were seen in all patients. All patients had previously received killed measles vaccine. A substantial number of persons who are older adolescents or young adults may be at risk of developing atypical measles.
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PMID:Atypical measles in adolescents and young adults. 44 83

A 13-year-old girl was admitted to a hospital because of fever and sore throat. Staphylococcus aureus was obtained on blood culture, and she was treated with antibiotics under the diagnosis of sepsis and DIC. Echocardiography showed huge vegetation attached to the posterior leaflet of mitral valve and severe mitral regurgitation. CT scan revealed multiple heterogeneous high density areas in her brain. She was transferred to our hospital for further examination and treatment. Large verrucae on the mitral valve, severe regurgitation and repeated embolism urged us to the emergency mitral valve replacement. Debridement of abscess on the posterior wall of the left atrium and ventricle necessitated patch plasty of those structures and mitral ring as well. Operative and postoperative examination showed mycotic aneurysm of right coronary artery, multiple brain hemorrhage, arterial obstructions of extremities and splenic infarction. Sooner she recovered except for slight macular degeneration caused by retinal embolism.
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PMID:[A case of infective endocarditis with multiple embolic complications]. 140 96

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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PMID:Purpura fulminans and adrenal hemorrhage due to group Y meningococcemia in an elderly woman. 190 68

A 71 year-old man with adult onset Still's disease was admitted to our hospital because of fever, sore throat, myalgia and macular nonpruritic salmon pink eruption. He was treated with prednisolone, 40 mg daily and these symptoms disappeared. When the dose of prednisolone was reduced to 30 mg daily, he began to notice fever. 5 days later he developed adult respiratory distress syndrome (ARDS). The dose of prednisolone was increased to 50 mg daily and oxygen administration was started. All symptoms began to improve immediately and the dose of prednisolone was decreased to 40 mg daily. 10 days later he noticed fever and skin rash. Laboratory investigation showed platelet counts of 69,000/mm3, a ferritin of 37,000 ng/ml, and increased fibrinogen degradation product, indicating increased activity of adult onset Still's disease associated with disseminated intravascular coagulation (DIC). The dose of prednisolone was again increased to 60 mg daily, and 100 mg of nafamostat mesilate was administrated intravenously. All above symptoms associated with adult onset Still's disease and DIC disappeared. The dose of prednisolone was gradually decreased and the clinical course was uneventful with daily administration of 10 mg of prednisolone. Although there are a couple of case report which described the association of adult onset Still's disease with either ARDS or DIC, the association of adult onset Still's disease with both ARDS and DIC have not been reported yet.
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PMID:[A case of adult onset Still's disease complicated with adult respiratory distress syndrome and disseminated intravascular coagulation]. 755 55

A 60-year-old woman who was previously in good health presented with a sore throat, fever, and a flu-like syndrome. Treated initially with acetaminophen and fluids for a presumed viral infection, she had a syncopal episode 4 days later, was admitted to the hospital, and died 3 hours after admission. Laboratory test results suggested sepsis with disseminated intravascular coagulation (DIC), whereas blood cultures grew group A beta-hemolytic streptococci. A postmortem diagnosis of streptococcal toxic shock syndrome was established. It was of particular interest that the pulmonary microcirculation was filled with thrombi that contained numerous gram-positive cocci. Although death from sepsis with DIC is not uncommon, septic pulmonary thrombosis has not been previously described. We speculate that this paradox may reflect unique properties of the virulent strains of Streptococcus pyogenes that are associated with streptococcal toxic shock syndrome.
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PMID:Septic pulmonary thrombosis in streptococcal toxic shock syndrome. 755 52

Epstein-Barr viral (EBV)-infection usually presents as fever, sore throat, fatigue, lymphadenopathy and atypical lymphocytosis. We describe a patient with disseminated intravascular coagulation as the presenting symptom caused by a primary EBV infection.
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PMID:Disseminated intravascular coagulation as an unusual presentation of an Epstein-Barr virus infection. 1100 94

Adult onset Still's disease (AOSD), the adult variant of the systemic form of the juvenile rheumatoid arthritis, is an uncommon disorder of unknown origin. Although the pathogenesis has not yet been clarified, an immunologically mediated inflammation occurs in active AOSD. High spiking fever, evanescent maculo-papular skin rash, arthralgias/arthritis, neutrophilic leukocytosis, negative rheumatoid factor and antinuclear antibodies, as well as a marked hyperferritinemia are the major features of AOSD. Sore throat, lymphadenopathies, hepato-splenomegaly, abdominal pain, polyserositis, respiratory distress syndrome, multiple organ dysfunction and disseminated intravascular coagulation may also occur. The clinical course of AOSD is extremely variable and unpredictable and can be divided into three main patterns: a self-limited or monocyclic pattern, a polycyclic or intermittent course, with one or more flares of the disease and complete remission among the episodes, and a chronic course, characterized by persistently active disease, usually due to a chronic, destructive arthritis. Since there are not pathognomonic laboratory parameters or histological findings, the diagnosis of AOSD requires the exclusion of infectious, malignant and autoimmune disorders. Some sets of criteria for classification have been proposed, but so far not validated. The prognosis of AOSD is usually considered relatively benign, although a destructive arthritis may cause severe disability and the multisystemic life-threatening complications of the disease may determine a fatal outcome. Treatment usually consists in nonsteroidal anti-inflammatory drugs and corticosteroids, but a more aggressive approach with disease modifying antirheumatic or immunosuppressive drugs may be required.
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PMID:[Adult onset Still's disease]. 1185 Jun 12

A 24-year-old woman was admitted to our hospital because of a high fever that had persisted for two weeks. She complained of a sore throat and arthralgia, and had evanescent rash, lymphadenopathy, liver dysfunction, and hyperferritinemia. Tests for RF and ANA were negative. Adult-onset Still's disease was diagnosed. On the fifth day of hospitalization, acute respiratory distress syndrome (ARDS) and disseminated intravascular coagulation (DIC) developed. Treatment consisted of mechanical ventilation and administration of steroid pulse-therapy and gabexate mesilate. Analysis of fluid obtained by bronchoalveolar lavage showed increases in the total cell count, predominantly of neutrophils and lymphocytes. Bilateral pulmonary infiltration seen on chest radiographs was alleviated, and the arterial blood gas data gradually improved. After cyclosporine was given, all the above symptoms associated with adult-onset Still's disease disappeared. Plasma levels of inflammatory cytokines decreased with the improvement of the patient's clinical condition.
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PMID:[A case of adult-onset Still's disease complicated with acute respiratory distress syndrome]. 1264 11

We report here the case of a patient suffering from hemophagocytic syndrome (HPS) associated with bile ductopenia. A 24-year old man was admitted after suffering fever, sore throat and general malaise for 7 days and jaundice for 2 days. Clinical studies showed hepatic dysfunction with hyperbilirubinemia. Epstein-Barr viral DNA from two bone marrow samples was detected. Bone marrow aspiration disclosed findings of HPS. Liver biopsy showed centrilobular cholestasis with lack of interlobular bile duct. Repeated therapeutic plasma exchange was effective for decreasing serum bilirubin and interleukin-6 levels. The patient received liver transplantation, however, he finally died of alveolar hemorrhage resulting from disseminated intravascular coagulation and acute rejection.
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PMID:Effect of plasma exchange on the circulating IL-6 levels in a patient with fatal hemophagocytic syndrome associated with bile ductopenia. 1655 42


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