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1/37. Disseminated adenovirus infection in two premature infants.

    We present two premature infants with disseminated neonatal adenovirus infection, whose epidemiology, clinical course and outcome differ to a great extent. The first infant, born vaginally at 35 weeks gestational age after premature rupture of membranes and maternal illness, developed pneumonia, hepatitis and coagulopathy and died of circulatory failure at the age of 17 days. The other infant, delivered by cesarean section at 36 weeks gestational age, did - in contrast to all documented cases in the literature - not show any signs of pneumonia and survived meningitis without sequelae. The mode of transmission of the viral infection may have been via the maternal birth canal in the first infant and transplacental in the second one. diagnosis was obtained by direct immunofluorescent test and serology in the first patient and by maternal serology and the detection of viral antigen in tracheal aspirates (ELISA) in the second patient. Disseminated neonatal adenovirus infection has a high mortality and should be considered in the differential diagnosis of neonatal sepsis, especially when pneumonia, hepatitis and neurologic symptoms develop together with thrombocytopenia or disseminated intravascular coagulopathy.
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2/37. Invasive Group C streptococcus infection associated with rhabdomyolysis and disseminated intravascular coagulation in a previously healthy adult.

    Infections with Group C Streptococci can lead to severe disease, particularly in individuals with underlying illnesses such as cardiovascular disease, malignancy or immunosuppression. We report the first case of rhabdomyolysis and disseminated intravascular coagulation secondary to Group C streptococcus in a previous healthy male. A toxic shock-like syndrome associated with Group C and Group G Streptococci has been reported. However, unlike with Group A Streptococci, production of endotoxins by these organisms is less well defined. We tested the patient's isolate for its ability to produce superantigenic toxins and to induce a mitogenic response. Although it is not known whether Group C Streptococci require special growth conditions for the production of superantigens, we could not demonstrate either the production of exotoxins or the induction of a mitogenic response.
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3/37. Increased warfarin sensitivity as an early manifestation of occult prostate cancer with chronic disseminated intravascular coagulation.

    Increased sensitivity to warfarin anticoagulation is usually attributed to liver disease, vitamin k deficiency, or drug interactions. We describe a patient with unexplained sensitivity to warfarin and mildly elevated prostate-specific antigen levels in whom subsequent developments indicated that warfarin sensitivity was the first manifestation of occult prostatic cancer. A review of all published cases of coagulopathy associated with cancer of the prostate shows that, unlike other solid tumors with secondary disseminated intravascular coagulation (DIC), in prostate cancer increased bleeding is more common than thrombotic phenomena. Chronic DIC due to occult prostate cancer should be included in the differential diagnosis of excessive prothrombin time prolongation in patients receiving anticoagulants.
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4/37. Lupus anticoagulant and protein s deficiency in a child who developed disseminated intravascular coagulation in association with varicella.

    Varicella is not always a benign disease it may cause serious complications. We report a two-year-old boy with disseminated intravascular coagulation in association with varicella. The patient had the lupus anticoagulant, the antiphospholipid antibody, acquired free protein s deficiency, and increased concentrations of the prothrombin F 1 2 fragment. Intravenous immunoglobulin was administered due to its potential antibody-blocking activity, and the patient responded well. We recommend that children with varicella and disseminated intravascular coagulation should be examined for the lupus anticoagulant, the free protein S antigen, the prothrombin fragment F 1 2 and the other coagulation parameters. Intravenous immunoglobulin administration could be useful in such conditions because of its antibody-blocking activity.
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5/37. disseminated intravascular coagulation and PC-SPES: a case report and literature review.

    BACKGROUND: PC-SPES is a herbal remedy gaining acceptance amongst prostate cancer patients and health care providers due to credible laboratory and clinical studies. However, PC-SPES has not been assessed in the standard rigorous approval process mandated for conventional agents. OBJECTIVES: To present a case of a patient with prostate cancer who, while using PC-SPES, developed disseminated intravascular coagulation (DIC). A review of the literature was conducted to determine if there is a relationship between PC-SPES and hemorrhagic disorders. methods: Searches were conducted in medline (1966-December 2000) and the Cochrane Collaboration's database. RESULTS: There are 116 clinical and laboratory based studies of PC-SPES published to date. There are no randomized controlled trials. Clinical studies have demonstrated a significant reduction in prostate specific antigen (PSA) levels within 6 weeks. Improved quality-of-life, reduction in the volume of tumor deposits and reduction in analgesic use has been demonstrated in hormone refractory patients. Laboratory studies suggest that the beneficial effects of PC-SPES are unrelated to physiologic estrogens. However, PC-SPES has a side-effect profile similar to diethylstilbestrol. There is data demonstrating a <5% risk of thromboembolic events, but this is the first report of DIC. CONCLUSION: The study of PC-SPES is in its infancy. This case may serve as a cautionary note to health care providers and patients regarding herbal remedies. Those using PC-SPES should have an increased level of surveillance for bleeding disorders.
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6/37. Fatal disseminated herpes simplex virus infection in a previously healthy pregnant woman. A case report.

    BACKGROUND: In contrast to the frequent occurrence of localized herpes simplex virus (HSV) infections during pregnancy, disseminated disease has rarely been reported. CASE: A 21-year-old woman in the 27th week of gestation developed a catastrophic illness characterized by fever, progressive pneumonia, respiratory failure, leukopenia, disseminated intravascular coagulation (DIC), anicteric hepatitis, septic shock and acute renal failure. Initial studies for an infectious etiology were negative. In spite of empiric broad-spectrum antimicrobial therapy, her condition continued to deteriorate. Sparse vesicular skin lesions suggestive of HSV infection subsequently appeared. Despite initiation of acyclovir therapy, the patient died. HSV type 2 was cultured from a skin vesicle, and at autopsy there was extensive necrosis of the liver and lung with immunohistochemical stains positive for HSV antigen. CONCLUSION: In the third trimester of pregnancy, HSV can occasionally disseminate in immunocompetent women. A clinical syndrome of unexplained fever, pneumonia, anicteric hepatitis, leukopenia and DIC without mucocutaneous lesions should prompt investigation and possible treatment for disseminated HSV infection.
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7/37. Chronic disseminated intravascular coagulation and childhood-onset skin necrosis resulting from homozygosity for a protein C Gla domain mutation, Arg15Trp.

    A toddler of Haitian descent presented with an 18-month history of chronic consumption coagulopathy, followed by catastrophic skin necrosis. protein c deficiency (1% to 3% of control) was noted by functional assay; chromogenic assay and antigen levels were 30% of control. plasma infusion abrogated the disseminated intravascular coagulation-like state. The authors identified a homozygous mutation, C1432T, resulting in a missense, Arg15Trp, in the gamma-carboxyglutamate domain of the protein. Chronic consumption coagulopathy without purpura fulminans or venous thrombosis is a rare presentation of defective protein C pathway. The result of this mutation is a mixed type I (low antigen) and type II (low function) phenotype.
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8/37. amniotic fluid embolism with involvement of the brain, lungs, adrenal glands, and heart.

    The case of a healthy 31-year-old woman in the 40th week of second pregnancy is presented. During preparation for an emergency caesarean section, she developed an amniotic fluid embolism (AFE) with unusual and unique features. The acute onset of disease with cardiorespiratory failure with hypotension, tachycardia, cyanosis, respiratory disturbances and loss of consciousness, suggested at first a pulmonary thromboembolism, but the appearance of convulsions led to the diagnosis of AFE. The patient died after 5 days due to an untreatable brain edema. At autopsy, AFE with the usually associated disseminated intravascular coagulation was found in the lungs, brain, left adrenal gland, kidneys, liver and heart. Eosinophilic inflammatory infiltrates were found in the lungs, hepatic portal fields and especially in the heart, suggesting a specific hypersensitivity reaction to fetal antigens. Moreover, intravascular accumulation of macrophages in the lungs also favored a non-specific immune reaction to amniotic fluid constituents.
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9/37. Rapid improvement of disseminated intravascular coagulation by donor leukocyte infusions in a patient with promyelocytic crisis of chronic myelogenous leukemia after reduced-intensity stem cell transplantation from an HLA 2-antigen-mismatched mother.

    Donor leukocyte infusion (DLI) is recognized as effective therapy for relapse after stem cell transplantation in patients with chronic myelogenous leukemia (CML). However, the clinical efficacy of DLI in the advanced phase of CML or other types of leukemia has not been clearly defined because of its varying degree of success. We describe a 22-year-old male patient with promyelocytic crisis of CML who had a relapse after peripheral blood stem cell transplantation, under reduced-intensity conditioning, from his HLA 2-antigen-mismatched mother. Complete hematologic remission was obtained after transplantation. However, a relapse that occurred on day 66 posttransplantion was characterized by an increase in number of leukemic promyelocytes with simultaneous exacerbation of disseminated intravascular coagulation (DIC). The patient received DLI containing 1 x 10(7)/kg CD3 cells on day 73. Because rapid improvement of DIC paralleled the decrease in leukemic cells and because it was observed soon after DLI and before the development of acute graft-versus-host disease (GVHD), we hypothesized that leukemia-specific cells other than natural killer cells or cytotoxic T-cells unrelated to GVHD played a role in the graft-versus-leukemia effect observed in our patient. In addition, this may be the first report of effective correction of DIC by DLI after stem cell transplantation.
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ranking = 5
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10/37. Refractoriness to platelet transfusion following partial hepatectomy for hepatocellular carcinoma: report of two cases.

    Refractoriness to platelet transfusion indicates a condition in which an expected increase in platelet count is not attained after platelet transfusion. We report herein two cases of refractoriness to platelet transfusion that were caused by the presence of antibodies against the human leukocyte antigen following partial hepatectomy for hepatocellular carcinoma. Due to low postoperative platelet counts in the first case, the patient was transfused with 75 units of platelet concentrate for 3 days after surgery, but was unable to gain a significant increase in the platelet count because of platelet transfusion refractoriness due to anti-human leukocyte antigen antibodies. This case was not complicated and had a favorable clinical course. Despite being transfused with 60 units of platelet concentrate during the 2 days after surgery, the patient's platelet count in the second case did not increase because of the presence of anti-human leukocyte antigen antibodies. Bleeding from the cut surface of the liver into the intraperitoneal cavity was found on the second postoperative day because of a decrease in platelet count. On postoperative day 5, disseminated intravascular coagulation occurred. Perioperative refractoriness to platelet transfusion is an intractable complication since no efficient treatment is available. Preoperative examinations for anti-platelet antibodies should be performed in patients undergoing hepatectomy for hepatocellular carcinoma. This is especially true in cases of decreased platelet counts due to preceding liver cirrhosis and when the prediction for postoperative platelet transfusions is necessary.
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ranking = 3
keywords = antigen
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