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)

A method of apheresis of plasma euglobulin fraction, cryoglobulins and Willebrand factor was developed. In one session of plasmapheresis 1500-1700 ml of patient's plasma were removed, fractionated and returned. The method was used in 2 patients with immune complex vasculitides. In one of them the disease developed against a background of chronic active hepatitis, in the other patient it manifested itself in cryoglobulin- and cryofibrinogenemia. Clinical improvement was noted in both cases: the absence of myalgia, arthralgia, hemorrhagic eruption, and ulcerative-necrotic skin changes. The normalization of increased ristomycin-cofactor activity of Willebrand factor and CIC levels was noted in one case. A decrease in the content of plasma cryoglobulins, cryofibrinogen, and urine protein concentration (from 1.5 up to 0.03%) was noted in the other case. A possibility of the use of the method in other pathological conditions (DIC-syndrome, unstable angina, atherosclerotic angiopathy) accompanied by endothelial damage, was discussed. Willebrand factor multimers form complexes with low density lipoproteins therefore the removal of these complexes may be useful in the treatment of hypercholesteremia and atherosclerosis.
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PMID:[Apheresis of euglobulins, cryoglobulins and the von Willebrand factor in vasculitis]. 295 14

In children, as in adults, Still's disease usually presents with a hectic fever, a characteristic rash and arthralgia or arthritis. Visceral involvement is however classical; the hepatic manifestations were studied with respect to two cases. Biochemical changes are common, often mild: the commonest abnormality is cytolysis. Jaundice is less frequent and hepatic involvement may in exceptional cases be life threatening, usually in cases of serious polyvisceral disease often with disseminated intravascular coagulation. These manifestations may be spontaneous or secondary to salicylate therapy; the anatomical changes are the same; the salicylate would therefore seem rather to unmask and aggravate an underlying hepatic abnormality.
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PMID:[Hepatic manifestations in Still's disease]. 307 93

We report a 20 year-old woman with hemophagocytic syndrome. In February 1993, she developed high fever, arthralgia, salmon-like pink eruption, leukocytosis and splenomegaly. She was diagnosed as adult Still's disease and successfully treated with intravenous immunoglobulin and oral prednisolone. In September 1993, she was re-admitted to our hospital complaining of general fatigue and low grade fever and treated with oral prednisolone at a daily dose of 15 mg. On October 2, 1993, she suddenly developed high fever and salmon-like pink eruption on her leg followed by the marked increase of serum transaminase and LDH levels (GOT 3,270 IU/l, GPT 1,880 IU/l, LDH 5,480 IU/l) on October 7. Since hepatic failure progressed, we started methylprednisolone pulse therapy and plasmapheresis. However, because of the progression of pancytopenia caused by hemophagocytosis, the treatment with VP-16 was initiated. However, she died of DIC on November 2, 1993. Autopsy revealed submassive necrosis of the hepatocytes with moderate infiltration of histiocytes. She was retrospectively diagnosed as hemophagocytic syndrome whose manifestations are very similar to those in adult Still's disease and acute viral hepatitis.
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PMID:[A case of hemophagocytic syndrome manifesting adult Still's disease and acute hepatitis]. 755 62

A case of pure red cell aplasia (PRCA) with various complications polyarthritis, angitis, acute renal failure and DIC was successfully treated with steroid pulse therapy was described. A 55-year-old woman was hospitalized with a 9-month of intermittent but progressive joint pain, morning stiffness, general fatigue, and fever. Her initial laboratory evaluation revealed a hemoglobin of 4.4 g/dl and absence of reticulocyte. Her bone marrow aspirate showed no erythroblast which was compatible with a diagnosis of PRCA. Marked leukocytosis and thrombocytosis, positive antinuclear antigen, elevation of gammaglobulin and C-reactive protein and the presence of polyarthritis and angitis which was confirmed by renal angiography, indicated an underlying autoimmune disorders. Steroid pulse therapy was administered at 500 mg/day for 3 days, resulting in the complete response in both red cell aplasia and above findings. PRCA is known to be associated with systemic lupus erythematosus and rheumatoid arthritis very rarely, but this case did not fulfill the criteria of known collagen diseases, and there is no previous report representing PRCA with various complications such as polyarthritis, angitis and acute renal failure. This case may help us to understand more about the relationship between PRCA and autoimmune disorders.
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PMID:[Pure red cell aplasia complicated with polyarthritis, angitis, and acute renal failure]. 825 11

In April, 1991, a 61-year-old man was admitted to our hospital because of pancytopenia and disseminated intravascular coagulation (DIC). Five years prior to admission he had developed high fever, skin eruption and arthralgia which had been improved by antibiotics, but recurred. Steroid therapy was ineffective for pancytopenia and DIC. Laboratory findings were as follows: RBC count, 274 x 10(4)/microliters; WBC count, 470/microliters; Platelets, 6.4 x 10(4)/microliters; fibrinogen, 153mg/dl; FDP, 67.0 micrograms/ml; FDP-D.Dimer, 13040ng/ml; thrombin-antithrombin complex, > 60.0ng/ml; and plasmin alpha 2-plasmin inhibitor complex, 10.3 micrograms/ml. As we suspected adult onset Still's disease on the basis of clinical course, we treated him with methylprednisolone pulse therapy, which was, however, ineffective. leukocytopenia, thrombocytopenia and DIC improved after cyclosporine treatment. Since cyclosporine is known to be very effective to autoimmune diseases, we speculate that in this patient immunological mechanism may be involve in the pathogenesis of DIC.
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PMID:[Cyclosporine therapy of adult onset Still's disease with disseminated intravascular coagulation]. 849 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

A 17-year-old female was admitted to our hospital because of pyrexia and thrombocytopenia in May 2002. Laboratory examination showed a platelet count of 50,000/microliter with an increased level of fibrinogen degradation product, leading to a diagnosis of disseminated intravascular coagulation (DIC). Gabexate mesilate was intravenously administrated without any effects. Several days later, erythema, joint pain and neck lymphadenopathy developed sequentially. The patient was diagnosed as having adult-onset Still disease (AOSD) complicated with DIC. Moreover, serum inflammatory cytokine levels had increased and activated macrophages were observed in the bone marrow, suggesting the presence of macrophage activation syndrome. After additional treatments with dalteparin and aspirin, the clinical symptoms and laboratory findings associated with AOSD and DIC disappeared. Although this was a severe case of AOSD associated with preceding DIC, the AOSD symptoms resolved in this patient with the treatment of the DIC and with aspirin only without any relapse.
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PMID:[Adult-onset Still disease presenting with disseminated intravascular coagulation]. 1516 49

Avascular necrosis of bone (osteonecrosis) that is atraumatic is most frequently associated with corticosteroid excess or alcoholism and usually involves the femoral head. We report a case of multifocal avascular necrosis in a 38-year-old woman with autoimmune Addison's disease taking corticosteroid replacement therapy. The onset of joint symptoms occurred 6 months after a pregnancy complicated by acute fatty liver and disseminated intravascular coagulation. Although both knees and ankles were involved, an unusual feature is that the hips were spared. As illustrated in this patient, avascular necrosis is frequently misdiagnosed in cases of joint pain of acute onset and may occur in the context of physiologic replacement doses of corticosteroids. Etiologic factors can precede the onset of symptoms and the diagnosis by several months.
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PMID:Postpartum multifocal avascular necrosis: what are the possible etiologies? 1703 87

Spider bites are a worldwide problem. Brown recluse spider bites can lead to severe local or systemic clinical effects, such as edema, necrotic ulcer, rashes, fever, chills, nausea, vomiting, malaise, arthralgia, myalgia, hemolysis, leukocytosis, disseminated intravascular coagulation, renal failure, and death. Eyelid bites from brown recluse spiders are rare. We report a child with severe facial edema and a dermonecrotic ulcer on the eyelid. Upon laboratory examination, leukocytosis with a significant left shift was detected. The patient was treated with antibiotics, systemic corticosteroid and conservative therapy that included saline compresses and ocular lubrication. No surgical excision was required. Vision was not impaired. A dermonecrotic ulcer is a severe complication of brown recluse spider bites. Since the diagnosis is difficult, clinical and epidemiological findings and a detailed history are important for an accurate diagnosis.
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PMID:A rare cause of severe periorbital edema and dermonecrotic ulcer of the eyelid in a child: brown recluse spider bite. 2153 46