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 three cases of localized thrombotic purpura that appeared during the convalescence phase of common chickenpox. This rare complication is characterized by localized symmetric ecchymotic purpura often affecting the lower limbs and often accompanied with signs of disseminated intravascular coagulation. In two cases we found transient protein S deficiency.
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PMID:Localized thrombotic purpura: a rare complication of chickenpox. 958 Jul 85

We examined the incidence of thrombophilia in deep vein thrombosis (DVT). Of 38 cases, we found 4 cases of protein C abnormality, 2 cases each of protein S abnormality and lupus anticoagulant, 1 case of antithrombin III abnormality. The total incidence was 23.7%, whereas only 2 cases (6.2%) of plasminogen abnormality were found among 32 healthy individuals. The incidence of thrombophilia was apparently higher among patients with DVT than that of healthy subjects, although the incidence of Japanese DVT was lower than that of Caucasian DVT, as previously reported. By SSCP analysis in one case of protein C abnormality, we demonstrated an abnormality of exon 9-3. To establish laboratory diagnosis of thrombophilia, it is recommended that (1) severe liver diseases, DIC, and oral anticoagulant be ruled out, (2) abnormality be confirmed by repeated examination, (3) family study determine inheritance mode, if possible. It was strongly suggested that laboratory examination of thrombophilia should be routinely applied to cases of venous thrombosis including DVT, not only for diagnostic interest but also for appropriate treatment of these cases.
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PMID:[Laboratory diagnosis of congenital thrombophilia]. 913 96

A 41-year-old woman with complete protein S (PS) deficiency who developed diffuse proliferative lupus nephritis is reported. She was referred to our hospital with nephrotic syndrome and thrombocytopenia. Her medical history included colorectostomy and amputation of the extremities because of repeated thrombotic episodes during her teens without any evidence of systemic lupus erythematosus. The diagnosis of PS deficiency was made from the patient's clinical course, undetectable serum PS in either the active or inactive form, normal protein C activity, and no evidence of the antiphospholipid syndrome. However, there was no definitive family history. A depressed level of complements and a positive antinuclear acid antibody suggested a diagnosis of systemic lupus erythematosus. The patient had a rapidly progressive course and died of disseminated intravascular coagulation. An autopsy showed generalized thrombotic lesions and diffuse proliferative lupus nephritis on both ordinal light and immunoperoxidase microscopy. Our observations suggest that PS-deficient patients may have a hyperinflammatory response.
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PMID:A 41-year-old woman with protein S deficiency and diffuse proliferative lupus nephritis: is protein S deficiency associated with a hyperinflammatory response? 918 80

Purpura fulminans is associated with homozygous protein C and homozygous protein S deficiency or may follow bacterial or viral infections. We present 2 children from 2 unrelated Arab families with purpura fulminans who were double heterozygotes for factor V Leiden inherited from their fathers and protein S deficiency inherited from their mothers. No previous thrombotic events have occurred in either patient or their respective family members. In one patient sepsis accompanied by disseminated intravascular coagulation appeared to be the trigger of purpura fulminans. In the other patient varicella infection preceded purpura fulminans and was also associated with disseminated intravascular coagulation. This report emphasizes the need for evaluation of hereditary defects in the inhibitory mechanisms of blood coagulation in patients with purpura fulminans at any age.
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PMID:Purpura fulminans induced by disseminated intravascular coagulation following infection in 2 unrelated children with double heterozygosity for factor V Leiden and protein S deficiency. 924 37

Solvent/detergent virus-inactivated plasma (VIP) contains markedly reduced protein S (PS) and alpha 2-antiplasmin (APL) beside other slightly decreased inhibitors. This could possibly be critical for the balance of hemostasis in diseases in which plasma inhibitors are reduced. A heterogeneous group of 14 patients with 18 plasma transfusions (12 FFP/24 VIP, 2 units per transfusion) was investigated. The patients suffered from dilution coagulopathy, liver disease, disseminated intravascular coagulation (DIC), hyperfibrinolysis, or received massive transfusions. Prothrombin fragment 1 + 2, fibrin monomers, D-Dimers, thrombin-AT III complexes, antiplasmin-plasmin complexes and fibrinogen degradation products as markers of activated coagulation (MAC) were measured. Blood samples were taken before and after plasma replacement. Significant differences between VIP and FFP should be recognized by comparing the ratio of MAC after/MAC before plasma transfusion. Patients showed an average inhibitor plasma level of AT III 51%, protein C 44%, PS 63%, and APL 52%. Only the F 1 + 2 ratio was obviously higher in the VIP group but not significantly. So the remaining MAC ratios did not show any significant difference. Our preliminary data showed no indication for a higher state of activation of coagulation in patients receiving VIP in comparison with those receiving FFP, if the VIP had the quality required. Solvent/detergent (SD) inactivation of transfusion-relevant viruses in plasma was successfully performed by Horowitz et al. [1]. The procedure leads to a partial reduction of the activity of clotting factors [2]. PS and APL are more severely affected. Therefore, treatment with VIP might activate or at least increase the activation of coagulation, especially in patients with reduced plasma inhibitors. To clarify this problem, the following disorders with the indication for plasma replacement were included in a prospective randomized study of FFP vs. VIP: massive transfusion; dilution coagulopathy; disturbance in liver synthesis; disseminated intravascular coagulation (DIC); primary hyperfibrinolysis. Low PS levels could induce hypercoagulability by reduced F VIII and FV inhibition, and low APL could induce hyperfibrinolysis by reduced plasmin inhibition.
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PMID:Protocol and preliminary results of a clinical study for comparison of solvent/detergent-inactivated plasma VIP versus FFP with special consideration of the balance of hemostasis. 942 23

Pneumonia is the most common serious complication of varicella infection in adults. A variety of thrombotic complications including purpura fulminans and disseminated intravascular coagulation have been reported in children with varicella but not in adults. Two men with varicella pneumonia who had profound lower extremity ischemia caused by thrombosis of the profunda femoris and tibial arteries are reported. Both patients had free protein S deficiency and vascular thrombosis in association with varicella pneumonia without overt evidence of disseminated intravascular coagulation or purpura fulminans. Antiphospholipid immunoglobulin G and immunoglobulin M antibodies were present in one, whereas the other had evidence of the lupus anticoagulant. The proposed pathogenesis and management options including intraarterial thrombolytic therapy with urokinase and the need for long-term anticoagulation are discussed.
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PMID:Spontaneous tibial artery thrombosis associated with varicella pneumonia and free protein S deficiency. 954 47

Blood coagulation tests are useful to diagnose some thrombotic diseases. Particularly, these tests are valuable for the diagnosis of familiar thrombophilia, antiphospholipid antibody syndrome (APS) and disseminated intravascular coagulation (DIC). For the diagnosis of thrombophilia, determinations of both biological activity and antigen level of antithrombin III, protein C and protein S are important for initial screening. Since activated protein C (APC) resistance is extremely rare in Japanese, APC resistant test that based on APTT, is unnecessary to include as one of the screening tests. Detection of activity and antigen level of either plasminogen or fibrinogen is recommended to screen the plasminogen deficiency or dysfibrinogenemia. Determination of lupus anticoagulant is needed for the diagnosis of APS. At this time, the dilute phospholipid APTT (dAPTT) or the dilute Russell viper venom time (dRVVT) may be useful as a screening test for LA because procedure of these tests are basically simple to perform in Japanese laboratory. In the next step, cross mixing test of dAPTT (or APTT) should be perform to make a diagnose of LA more solid. Final confirm tests can be conveniently carried out with kit of either STACLOT or LA-CONFIRM. Platelet count and FDP (or FDP D dimer) assay are two essential tests for the diagnosis of DIC. Criteria of diagnosis for DIC recommended by Blood Coagulation Research Group of Japanese Ministry of Health and Welfare is not unnecessarily appropriate for practical use. TAT and PIC can be a good laboratory tests for early detection of hypercoagulable state in patients with DIC.
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PMID:[Clinical diagnosis of thrombosis and blood coagulation tests]. 956 63

Four-factor PCCs are most frequently used for replacement of vitamin K-dependent clotting factors and inhibitors proteins C and S in patients bleeding after phenprocoumon or warfarin overdose, in vitamin K-deficient patients presenting life-threatening bleeding, and liver disease. Since many of these patients are prone to thromboembolic complications including DIC, all conceivable measures should be taken against the thrombogenic potential of PCC preparations. This thrombogenic potential of PCCs is obviously dependent on several factors including activated clotting factors, lack of inhibitors of blood coagulation, and coagulation factor overload, as well as predisposing factors referred to recipients and drug interactions. The composition of PCC should meet the following criteria: Antithrombin in addition to heparin for the neutralization of FIXa and FXa should be present in the preparations; no overloading with FII and FX; substantially lower FVII than FIX potencies in order to minimize contamination with or generation of FVIIa; and substantial protein C as well as protein S activities. Quality control should include determinations as recommended by the European Pharmacopoeia. Specific assays for quantification of FIXa and FXa are urgently required, and validity of these assays must be proven in surveys. All lots should also be tested for their FVIIa content. Furthermore, the safety of PCCs must be proven by suitable animal models. Whenever possible, patients receiving PCCs should be under low-dose heparin prophylaxis; simultaneous administration of heparin-neutralizing drugs or antifibrinolytic agents must be avoided.
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PMID:Production and composition of prothrombin complex concentrates: correlation between composition and therapeutic efficiency. 1049 3

Previous studies suggest that there is a systemic activation of clotting and fibrinolysis in preterm infants with advanced respiratory distress syndrome (RDS). However, there are no data on the hemostatic status in the early stages of the disease; therefore, we studied some of the hemostatic parameters in these patients and made several studies at different times in preterm infants who did or did not develop RDS, using similar protocols. We found normal plasma fibrinogen, protein C, protein S, C4b-binding protein, thrombomodulin, antithrombin III, thrombin-antithrombin III complex, prothrombin fragment 1.2, plasminogen, tissue plasminogen activator, alpha-1 antitrypsin, alpha-2-macroglobulin and protein Z. However, lower D-dimer and higher plasminogen activator inhibitor and von Willebrand factor antigen levels were found within six hours of life in infants who later developed RDS compared to the control group. These findings suggest that disseminated intravascular coagulation is not prominent in the early stages of RDS. Moreover, reduced D-dimer and increased plasminogen activator inhibitor and von Willebrand factor antigen levels are probably related to the abnormalities in the fibrinolytic mechanism due to lung damage in RDS, but further studies are needed to show their pathogenic significance in RDS.
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PMID:Hemostatic system in early respiratory distress syndrome: reduced fibrinolytic state? 1077 Jan 17

Background: Reduced levels of protein S (PS) and alpha(2)-antiplasmin alpha(2)-AP) in solvent/detergent virus-inactivated plasma (S/D-VIP) might induce an imbalance of plasma coagulation factors and inhibitors in patients transfused. We investigated 40 patients (23 fresh frozen plasma (FFP), 17 S/D-VIP, random distribution by a list calculated by statisticians) who suffered from dilution coagulopathy, liver disease, disseminated intravascular coagulation (DIC), polytrauma or were connected to extracorporeal circulation. Study Design and Methods: The following markers of activated coagulation (MAC) were measured: Prothrombin fragment F1+2 (F1+2), fibrin monomers (FM), D-dimers (DD), thrombin-antithrombin (TAT) and plasmin-antiplasmin (PAP) complexes as well as fibrinogen degradation products (FgDP), and additionally antithrombin III (antithrombin), protein C (PC), PS and alpha(2)-AP. Blood was taken only just before and 1 h after the first plasma replacement (2 units). No additional blood products were transfused before the second blood withdrawal. Pre- and posttransfusion (pre/post) values of all parameters measured were compared within the same group and between both groups. Statistical evaluation of the data was done by Wilcoxon's paired test for data in the same plasma group and by the test of Mann and Whitney for data comparison between both plasma groups. Results: Average pretransfusion values of all inhibitors for both plasma groups were in the same range and increased after transfusion, except for PS in both groups. Whereas the pre/post values did not differ significantly in the FFP group, antithrombin (p = 0.02), PC (p = 0.0005), and alpha(2)-AP (p = 0.02) showed a significantly higher increase in the S/D-VIP group. Considering the pre/post differences between both plasma groups, there were no significant differences. The same was true for MAC measured pre- and posttransfusion. Conclusion: Data showed no significant difference between both plasma groups, indicating that S/D-VIP plasma behaves as FFP under the study conditions employed. Copyright 2000 S. Karger GmbH, Freiburg
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PMID:Comparison of Solvent/Detergent-Inactivated Plasma and Fresh Frozen Plasma under Routine Clinical Conditions. 1087 83


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