Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:3.4.21.6 (thromboplastin)
13,278 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We investigated the functional and morphological changes of the liver after brain death for long period maintained with the combined administration of arginine vasopressin (ADH) and catecholamine. Twenty five brain-dead patients suffered from severe closed head injury were studied. The average age was 38.2 y.o. Systemic circulation was maintained normal for at least 6 days with ADH and catecholamine. ADH was infused constantly with an average dosage of 0.3 mU/kg/min. Simultaneous infusion of catecholamine was adjusted to maintain the mean arterial pressure above 80 mmHg. The morphological changes were not remarkable in the liver cells throughout the study. Levels of activated partial thromboplastin time (APTT) except in 5 patients remained within normal range for two weeks. According to this data, it can be considered that there is no marked lowering in the activity of protein synthesis of the liver. The progressive increase of serum alkaline phosphatase, LAP, gamma-GTP, and total bilirubin were observed as the characteristic changes of the liver after brain death. Serum levels of total bilirubin were markedly elevated especially in the patients who received massive transfusion. Histologically, cell infiltration into the Glisson's sheath became remarkable in all cases as day proceeded. These findings suggest the dysfunction of bile excretion might occur due to the denervation from the brain.
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PMID:[Functional and morphological changes of the liver after brain death maintained with the combined administration of vasopressin and catecholamine]. 187 93

In this paper we report the case of a new Italian family with severe cross-reacting material prekallikrein deficiency (CRM-). The proposita is a 22-year-old woman referred for evaluating an extremely prolonged activated partial thromboplastin time (APTT) detected during a routine screening. No clearcut bleeding history was reported. Prekallikrein antigen and activity were not measurable. The other contact-phase factors were within the normal range. Using an electromechanical coagulometer, six different commercial reagents yielded a markedly prolonged APTT (ratio greater than 2). By prolonging the incubation time up to 10 min, APTT was normalized only with reagents employing ellagic acid as activator. On the contrary, APTT remained markedly prolonged using particulate activators (i.e. micronized silica and celite). No differences were observed using either rabbit or bovine brain cephalin. APTT was also performed on a laser automated ACL instrument; in this case reagents using ellagic acid yielded only moderately prolonged APTT values (ratio 1.3 vs 1.4). The intrinsic fibrinolytic activity, as assessed by blood activator inventory test, was found to be moderately reduced (about 50% of normal) in the proposita, whereas normal values were measured in the heterozygous relatives. After infusion of 0.3 micrograms/kg 1-desamino-8-D-arginine vasopressin (DDAVP), kallikrein levels did not change in the proposita and her heterozygous relatives. A normal release of tissue-plasminogen activator, as assessed by fibrin-plate assay, was observed in all family members including the proposita.
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PMID:A new Italian family with severe prekallikrein deficiency. Desmopressin-induced fibrinolysis and coagulation changes in homozygous and heterozygous members. 212 71

A 67-year-old male with a prolonged activated partial thromboplastin time (APTT) of 43 seconds (normal, 25-40 seconds) was found to have laboratory features of von Willebrand's disease and IgA myeloma but had a normal bleeding time and no bleeding tendency. Plasma Factor VIII coagulant activity (F.VIII:C) was 80 U/L (0.08 U/mL), Factor VIII antigen (F.VIII:Ag) 70 U/L (0.07 U/mL), and von Willebrand's factor antigen (vWF:Ag) 50 U/L (0.05 U/mL) and ristocetin cofactor (vWF:RiCoF) 10 U/L (0.10 U/mL). The platelet vWF:Ag level was normal, whereas both platelet lysate and plasma vWF high molecular weight multimers were decreased. Patient plasma had no inhibitory effect on either F.VIII:C or vWF:RiCoF. However, when patient plasma was incubated with normal plasma, crossed immunoelectrophoresis for vWF:Ag demonstrated the presence of immune complexes. Infusion of 1-desamino-8-D-arginine vasopressin led to a transient correction of the plasma vWF:Ag multimer pattern. The survival of all components of vWF/F.VIII was decreased, as also occurred after cryoprecipitate. The levels of plasma F.VIII/vWF increased as the IgA values decreased after chemotherapy, whereas the platelet high molecular weight multimers remained decreased. The data suggest that the plasma vWF/F.VIII deficiency results from complexing of the IgA myeloma protein with vWF, resulting in premature clearance of the vWF/F.VIII complex. The absence of clinical bleeding likely results from the combination of a normal platelet vWF:Ag level and persistence of intermediate molecular weight vWF multimers.
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PMID:Absence of a bleeding tendency in severe acquired von Willebrand's disease. The role of platelet von Willebrand factor in maintaining normal hemostasis. 250 65

Combined deficiency of factor V and factor VIII, a rare bleeding disorder, was found in a 43 year-old male. He had often presented manifestations of easy bruising since childhood, but none of his family had shown evidence of a bleeding tendency. We examined him and his family as far as we could and his abnormality of blood coagulation was apparent, but the members of his family were normal. The prothrombin time and activated partial thromboplastin time of this patient were prolonged, but his thrombin time was normal. Factor V and factor VIII coagulant activity were low, but von Willebrand factor antigen and activity (ristocetin cofactor activity) levels were normal. Protein C and Protein C inhibitor antigen and activity levels were also found to be normal. Following 1-deamino-8-D-arginine vasopressin (DDAVP) injection, he had immediate increases in factor VIII coagulant activity, but both von Willebrand factor antigen, activity levels and factor V coagulant activity remained low. Moreover, there was no rapid decline in factor VIII complex activity. These findings suggest that the endogenous factor VIII in this patient is metabolized normally and that at least the deficiency of factor VIII does not result from accelerated degradation in plasma.
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PMID:[DDAVP administration in a case of congenital combined factor V and factor VIII deficiency]. 260 19

A study of the absorption of 300 micrograms of 1-deamino-8-D-arginine vasopressin (DDAVP) given intranasally to normal blood donors was carried out to determine (a) the correlation between plasma levels of DDAVP and the percent rise of factor VIII procoagulant activity (VIII:C) and (b) the efficacy, specificity and safety of this treatment in increasing the recovery of factor VIII:C in donated blood. The maximum drug concentration was highly correlated to the maximum percent rise of VIII:C (r = 0.858, p less than 0.01). A differentiated effect of DDAVP on increases of VIII:C, VIII:Ag, vWF:Ag and vWF multimers was observed. A transient rise of fibrinopeptide A from 5 to 16 ng/ml, 30 min post-DDAVP, was not accompanied by changes in fibrinogen levels or generation of detectable factor Xa or thrombin. DDAVP had no effect on the factor XII-dependent pathway of plasminogen activation, or on the donor's vital signs and hematological parameters. Side effects were minor and of short duration. Intranasal DDAVP treatment of blood donors is considered to be a practical means of improving the recovery of VIII:C from normal donors.
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PMID:Effectiveness, specificity and safety of intranasal 1-deamino-8-D-arginine vasopressin treatment of normal blood donors. 297 94

Five patients with glycogen storage disease type I (GSD-I) were evaluated for a bleeding diathesis and subsequently were given an infusion of 1-deamino-8-D-arginine vasopressin (DDAVP). Although platelet counts were normal or slightly elevated, the baseline template bleeding times were prolonged in four of the patients. Prothrombin times and activated partial thromboplastin times were normal, while ADP- and epinephrine-induced platelet aggregations were absent in the three patients tested. Ristocetin- and collagen-induced platelet aggregations were abnormal. Laurell and immunoradiometric determinations of the factor VIII-related antigen (vWf antigen) were decreased. Glyoxyl agarose gel electrophoresis of the patients' plasma revealed abnormal multimer patterns in four of the five patients. After the DDAVP infusion the platelet aggregation abnormalities persisted; however, the bleeding time and the von Willebrand antigen and activity corrected. We conclude that GSD-Ia patients may have a metabolically acquired form of von Willebrand's syndrome as well as an acquired intrinsic platelet defect, and that DDAVP may be useful in the management of bleeding in these patients.
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PMID:DDAVP infusion in five patients with type Ia glycogen storage disease and associated correction of prolonged bleeding times. 308 38

To evaluate the effect of 1-desamino-8-D-arginine vasopressin (desmopressin) on blood loss in surgery, we conducted a randomized, double-blind trial of the drug in 35 patients with normal hemostatic function who were having spinal fusion with Harrington rod instrumentation. Seventeen patients were designated to receive 10 micrograms/m2 of desmopressin, and 18, to receive a placebo. Preoperative testing showed that desmopressin increased factor VIII coagulant activity, von Willebrand antigen concentrations, glass bead platelet retention, and prothrombin consumption and decreased the partial thromboplastin and bleeding times (p less than or equal to 0.0003). During surgery, desmopressin reduced blood loss by 32.5% (547 mL; 95% confidence interval [CI], 19 to 1075; p = 0.015) and reduced the need for concentrated erythrocyte transfusions by 25.6% (0.86 units; 95% CI, 0.08 to 1.65; p = 0.022). After surgery, desmopressin reduced the duration of treatment with analgesic agents by 13.1% (34.0 hours; 95% CI, -5.2 to 72.7; p = 0.105), presumably by decreasing bleeding in the surgical wound. When adjusted for the origin of the scoliosis by two-way analysis of variance, this effect was even more evident (p = 0.014). Multiple regression analysis showed that the best three predictors of blood loss in surgery and transfusion requirements were the bleeding time, glass bead platelet retention, and the use of desmopressin.
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PMID:1-Desamino-8-D-arginine vasopressin (desmopressin) decreases operative blood loss in patients having Harrington rod spinal fusion surgery. A randomized, double-blinded, controlled trial. 363 84

Humans exposed to hypoxia usually increase their plasma procoagulant VIII activity (VIII:C) with no change in the concentration of VIII related antigen (VIIIR:Ag). This case report describes an apparently normal subject who developed marked qualitative and quantitative changes in all components of the factor VIII complex while inhaling an 11% oxygen/balance nitrogen gas mixture for 2 h. Blood from fresh venepunctures was drawn at baseline, during and after exposure to hypoxia for the following: a partial thromboplastin time, a prothrombin time, fibrin monomer, factor VIII:C, VIII procoagulant antigen (VIII:CAg); ristocetin cofactor activity (VIIIR:Co); VIII von Willebrand factor (VIII:vWF) multimer pattern; and arginine vasopressin. During hypoxia VIII:C, VIII:CAg, VIIIR:Ag and VIIIR:Co increased 4 to 5 fold; the VIII:vWF multimer pattern showed increasing low molecular weight complexes, fibrin monomer appeared and arginine vasopressin (AVP) levels increased from 5.5 pg . ml-1 to 73.8 pg . ml-1. These changes are compatible with both the release of the VIIIR:Ag by AVP and protease induced fragmentation of the VIII complex.
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PMID:Hypoxia-induced vasopressin release and coagulopathy in a normal subject. 393 69

To evaluate the effect of 1-deamino-8-D-arginine vasopressin (DDAVP) in various bleeding disorders, 10 micrograms/m2 DDAVP was administered to subjects with von Willebrand disease (13), platelet function defects (12), von Willebrand disease and platelet defects together (8), or isolated prolongation of the bleeding time (5). DDAVP shortened the bleeding time similarly in all patient groups. Shortening of the bleeding time was also observed in 2 patients with aspirin-induced platelet defects and in 2 normal subjects. DDAVP administration was associated with falls in the platelet count, mean platelet volume, and partial thromboplastin time, and rises in platelet adhesion, factor VIII coagulant activity, factor VIII related antigen, and von Willebrand factor activity. The basal bleeding time was the only predictor of the magnitude of the bleeding-time correction. Normal haemostatis was achieved with DDAVP plus epsilon-aminocaproic acid and no blood product support during operations in 18 patients with bleeding disorders.
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PMID:Shortening of bleeding time by 1-deamino-8-D-arginine vasopressin in various bleeding disorders. 614 76

The aim of this double-blind, placebo-controlled crossover study was to investigate the effect of 1-deamino-8-D-arginine vasopressin (dDAVP) on hemostasis in patients with chronic liver disease. Nine consecutive patients with biopsy-proven liver cirrhosis and related coagulation abnormalities received in a random order dDAVP, 0.5 microgram/kg, or saline intravenously. Blood samples were taken before dDAVP infusion and 30, 60 and 180 min after its end. dDAVP infusion induced a statistically significant shortening of the bleeding time from 9 min (range 6.5-15.5) to 6 min (range 4.5-9.5) at 1 h after the infusion. The activated partial thromboplastin time was significantly shortened at 30 and 60 min after dDAVP infusion. Plasma levels of factor VIII, XI and XII coagulant activities were significantly increased at all sampling times after dDAVP infusion. The maximum increase over basal values in plasma levels of factor VIII, XI and XII was 63, 22 and 40%, respectively. dDAVP did not induce any significant changes of prothrombin time, thrombin clotting time, fibrinogen, plasma levels of factor II, V, VII, IX, X, factor XII antigen, protein C (activity and antigen), antithrombin III, plasminogen and alpha 2-antiplasmin. Placebo infusion did not produce any significant changes in the evaluated parameters. We conclude that dDAVP can positively influence the hemostatic system in patients with liver cirrhosis. The clinical relevance of this hemostatic improvement deserves further evaluation.
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PMID:Effects of desmopressin on hemostasis in patients with liver cirrhosis. 748 63


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