Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Antithrombin III Deficiency”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 37 records · Page 2Linked to original sources
PubMed1994

Venous surgery in patients with congenital antithrombin III deficiency.

Congenital antithrombin III (ATIII) deficiency, although uncommon, is a condition of considerable surgical importance. Affected patients are at significant risk of venous thrombosis during a surgical illness and standard forms of prophylaxis may not be effective. In addition, sequelae of their thromboses may in turn make them a candidate for venous surgery. An ATIII concentrate is now available in Australia for clinical use. This report describes two patients who underwent surgery for postphlebitic limbs utilizing infusions of ATIII concentrate. The ATIII deficiency state is reviewed and a method is described that should allow safe surgery in these patients.

Adult↗
PubMed1992

Measurement of markers of activated coagulation in antithrombin III deficient subjects.

Functional antithrombin III levels were measured by factor Xa inhibition in 63 members of a large family with type 2 antithrombin III deficiency and individuals were classified as antithrombin III deficient or non-deficient according to the results. F1 + 2 and TAT complexes were measured using an ELISA and FPA levels were measured by radioimmunoassay. Thirty subjects (48%) were classified as antithrombin III deficient and 33 (52%) as antithrombin III non-deficient. The mean level of F1 + 2 was significantly higher in the deficient adults (0.87 +/- 0.26) compared to both the non-deficient adults (0.70 +/- 0.21) (p = 0.03) and the deficient adults receiving warfarin (0.16 +/- 0.01) (p less than 0.001). The differences in the mean values of TAT complexes and FPA between deficient and non-deficient individuals were not statistically significant. These findings suggest that untreated antithrombin III deficient subjects generate more thrombin than their non-deficient family members and that warfarin inhibits this thrombin formation. In this cross-sectional study, it is not possible to correlate the levels of the surrogate makers with future clinical outcome.

Adolescent↗
PubMed1994

Antithrombin III replacement in animal models of acquired antithrombin III deficiency.

Plasma antithrombin III (AT III) levels decrease early during Gram-negative septicaemia, and even a moderate decrease in this major inhibitor of the coagulation system is associated with serious disseminated intravascular coagulation (DIC). This study reports the efficacy of high dose (at least 250 units/kg) AT III replacement in three animal models of Escherichia coli endotoxaemia or bacteraemia. Highlights of our findings are: 1. Endotoxaemic rat model: DIC occurs early, before the appearance of deleterious cardiovascular abnormalities; AT III prophylaxis attenuates DIC; and AT III prophylaxis increases permanent survival. 2. Endotoxaemic sheep pulmonary dysfunction model: AT III prophylaxis prevents the typical decrease in arterial oxygen partial pressure and AT III prophylaxis combined with alpha 1-proteinase inhibitor significantly attenuates indices of pulmonary dysfunction. 3. E. coli bacteraemic baboon model: AT III prophylaxis and treatment significantly attenuates indices of DIC and organ damage, and prevents death in an otherwise 100% lethal bacterial challenge. In conclusion, prophylactic treatment with high doses of AT III may be efficacious in disease states of impending DIC such as Gram-negative septicaemia. Data presented herein demonstrate that plasma levels of AT III must be maintained at levels markedly higher than normal to be efficacious in animal models of Gram-negative endotoxaemia or bacteraemia.

Animals↗
PubMed1987

Antithrombin III microheterogeneity in antithrombin III deficiency and in the antithrombin III abnormality, "antithrombin III Toyama".

Antithrombin III (AT III) microheterogeneity was investigated in 12 cases of congenital AT III deficiency and 2 cases of congenital AT III abnormality by isoelectric focusing (IEF) and immunofixation. In congenital AT III deficiency, IEF and immunofixation revealed AT III as 8 bands which was indistinguishable from normal control in terms of the number of bands and the isoelectric point (pI) of each band. In the proband of the congenital AT III abnormality, however, IEF and immunofixation showed AT III as 8 bands which shifted slightly but definitely to the acidic side compared to those of normal subjects. This change in pI of the abnormal AT III was considered to reflect the amino acid replacement in the polypeptide chain of the abnormal AT III molecule.

Adult↗
PubMed1991

Effective prophylaxis of thrombosis by antithrombin III concentrate in a pregnant woman with congenital antithrombin III deficiency: relations between plasma antithrombin III activity and the plasma levels of hemostatic molecular markers.

The value of antithrombin III (AT III) concentrate and a standard criterion for its use were examined in a pregnant woman with congenital AT III deficiency by continuous monitoring of plasma AT III activity and the plasma levels of hemostatic molecular markers. The rates of improvement of various markers after AT III administration (frequency of improvement/frequency of administration) were as follows: fibrinopeptide A (FPA) 82%, D-dimer 70%, fibrinopeptide B beta 15-42 73%, beta-thromboglobulin 60%, and platelet factor 4 50%. There was no significant correlation between the plasma AT III activity and all plasma FPA values, but FPA values of over 3.9 ng/ml showed a significant negative correlation with AT III activity: AT III activity (%) = -6.59 x FPA (ng/ml) + 125, r = -0.851, p less than 0.02. We therefore recommend continuous monitoring of the plasma FPA level and administration of AT III concentrate when the FPA level is elevated. According to the regression line shown above, plasma AT III activity should be raised to 100% to keep the FPA level below 6.0 ng/ml with 95% confidence limit.

Adult↗
PubMed1985

Antithrombin III metabolism in two colitis patients with acquired antithrombin III deficiency.

125I-Antithrombin III metabolism studies were performed in 2 patients with ischemic and ulcerative colitis, respectively. Both patients had acquired antithrombin III deficiency and objectively diagnosed deep venous thrombosis. A decreased 125I-antithrombin III plasma disappearance halflife and an increased fractional catabolic rate was found in both patients. The transcapillary flux ratio was elevated in the patient with ischemic colitis. A follow-up study of the first patient during a period when no signs of an ischemic colitis were present and no medication was taken showed completely normal tracer data. The data are consistent with both gastrointestinal loss and intravascular consumption of antithrombin III. The antithrombin III deficiency could not be explained by other causes such as proteinuria, liver dysfunction, or obvious disseminated intravascular coagulation. Reduced antithrombin III plasma levels were considered to have contributed to the development of deep venous thrombosis in both patients.

Aged↗
PubMed1985

[Retinal vascular occlusion in antithrombin III deficiency].

Deficiency of Antithrombin III (AT III), which is one of the main inhibitors of blood coagulation, may lead to severe thrombosis and embolism. The authors detected AT III deficiency in two patients with retinal vessel occlusion. Both patients were otherwise healthy. One had recurrent retinal vein thrombosis, the other cilioretinal occlusion. To the authors' knowledge, only one case of retinal vessel occlusion with AT III deficiency has been reported in literature. Furthermore, in the patients reported here retinal vessel occlusion may have been caused by AT III deficiency, which has considerable therapeutic and prophylactic consequences for patients with this disorder. AT III should therefore be determined in retinal vessel occlusion of unknown cause.

Aged↗
PubMedDate not supplied

[Anesthetic management for cesarean delivery in a pregnant patient with congenital antithrombin III deficiency and pulmonary thromboembolism].

Antithrombin III (AT III) deficiency is a rare hereditary disease that predisposes a patient to thromboembolic complications. Anticoagulation is essential for preventing recurrence of thrombi. Concentrated AT III replacement is reserved for acute periods of thromboembolism or moments of increased risk. Hemostatic anomalies are generally considered a contraindication for regional anesthesia, due to the potential risk of spinal hematoma. This paper describes a woman with congenital AT III deficiency and heparin-treated pulmonary embolism whose pregnancy of 29 weeks had to be terminated by cesarean section upon signs of fetal distress. We discuss the pathophysiology and treatment in such cases.

Adult↗
PubMed1982

Treatment of venous thrombosis in antithrombin III deficient patients with concentrates of antithrombin III.

Two patients with familial antithrombin III deficiency developed deep venous thrombosis of the lower limb. The diagnosis of venous thrombosis was made by the indium labelled platelet technique which also allowed for the daily assessment of thrombus size. Each patient received treatment with Warfarin, subcutaneous heparin, and infusions of antithrombin III concentrates. The authors conclude that infusions of antithrombin III concentrates may be of value in limiting the extent of acute thrombosis in patients with a severe deficiency of this protein and may help prevent pulmonary embolism. The haemorrhagic risk of continuing modest doses of heparin with high dose ATIII therapy appears small. In addition to its value in the diagnosis of venous thrombosis the indium platelet technique may give an early indication of thrombus extension and may thus indicate the effectiveness of treatment.

Adult↗
PubMed1992

Prophylactic antithrombin III administration during pregnancy immediately reduces the thrombin hyperactivity of congenital antithrombin III deficiency by forming thrombin-antithrombin III complexes.

We examined the changes of haemostatic molecular markers after antithrombin III (AT III) administration in a 22-year-old woman with congenital AT III deficiency in the third trimester of pregnancy who did not have thrombosis. Various markers including fibrinopeptide A (FPA), thrombin-antithrombin III complex (TAT), prothrombin fragment F1 + 2 (F1 + 2), plasmin-alpha 2antiplasmin, D-dimer, beta-thromboglobulin, and platelet factor 4 were measured before and just after 3,000 U of AT III concentrate, which was given three times per week from the 34 week of pregnancy until delivery. Just after AT III administration, F1 + 2 and FPA levels decreased on most occasions, while TAT sometimes increased. Plasma FPA levels were markedly decreased on all 8 occasions when the plasma FPA levels was above 2.0 ng/ml before AT III administration. Plasma FPA levels were always greater than or equal to 6.4 ng/ml before AT III administration on the 4 occasions when TAT increased to above 115%. The changes of plasma F1 + 2 levels were significantly correlated with the AT III level. These results suggest that prophylactic AT III administration in the third trimester immediately inactivates intravascular thrombin to form TAT and reduce the plasma FPA level. Thus, the transient TAT elevation following AT III administration may not only be due to extraction of thrombin from the fibrin clots of thrombi but also to intravascular thrombin which is not attached to thrombi. FPA is the best molecular marker for thrombin hyperactivity and it should be monitored in AT III-deficient pregnant women in the third trimester.

Adult↗
PubMed1986

[Clinical aspects of acquired antithrombin III deficiency].

The significance of acquired antithrombin III (AT III) deficiency must be interpreted in close relation to the underlying disease process. In patients with acute or chronic liver impairment, the AT III activity is related to a decrease of procoagulatory factors, whereas, in protein loss syndromes such as nephrotic syndrome, the AT III indicates an increased risk of thromboembolic events. The effect of oral contraceptives (OC) on AT III levels in young healthy females (n = 30) was determined prospectively. AT III decreases during OC usage could not be related to the estrogen content of the examined oral contraceptives, and there was no parallel decrease of AT III activity and concentration in each type of OC. In a prospective study, the extent of AT III decrease was determined in patients undergoing cardiopulmonary bypass operations (CPB) receiving different anticoagulant schedules during extracorporeal circulation (n = 49). There was no significant influence on the effectiveness of anticoagulation by the observed AT III decreases. AT III deficiency during CPB was primarily the result of hemodilution. However, the AT III kinetics were significantly influenced by the different protamin dosages and were not affected by the different heparin dosages. Correction of diminished AT III levels by substitution of AT III concentrates is beneficial in cases, in which an interruption of an enhanced coagulatory process such as disseminated intravascular coagulation is necessary or in patients requiring high dosage heparinization as in deep vein thrombosis. In those cases the quality of AT III correction correlates to the course of the disease. However, the potency of concentrates as well as the individual AT III recovery and half-life must be considered for an appropriate treatment with AT III substitution.

Adult↗
PubMed1987

[Prevention of thromboembolism during pregnancy in 3 sisters with congenital antithrombin III deficiency and decreased inducible fibrinolysis].

Antithrombin III (AT III) deficiency is associated with a high risk of venous thromboembolism, particularly in pregnancy. As prophylactic treatment it has been recommended that plasma levels of AT III be normalized by use of AT III concentrates, in addition to heparin. - We report on the prophylactic treatment of three sisters (age 21, 25, and 32 years) with congenital AT III deficiency (38-53%, normal 80-120%) and reduced inducible fibrinolytic activity (1.2, 5.8, 1.2%, normal greater than 8.5%), who had already had severe thromboembolism. During pregnancy prophylactic measures were taken individually, depending on the plasma level of beta thromboglobulin (BTG) determined every 2-3 weeks. In two patients prophylaxis with s.c. heparin (2 X 7500 IU/d) was started at the time of the first increase of BTG (around 10th week of gestation), leading to normalization of BTG. When BTG was again elevated, the dose of heparin was increased stepwise to 2 x 15,000 IU/d; in this way functional AT III levels remained in the range of 28-50%. Thus, these two patients received only heparin throughout pregnancy. However, in the third patient BTG levels could not be normalized by heparin alone (60-130 ng/ml, normal less than 43 ng/ml). Injections of AT III concentrate (1000 IU) led to reduction of BTG within 2 hours (60----42,220----61 ng/ml). Therefore, AT III was given from the 25th week of gestation in increasing amounts up to 5000 IU/week (funct. AT III in plasma 51-72%) in addition to heparin (2 x 12,500 IU/d), resulting in BTG levels of 33-51 ng/ml.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2026

Facial Dysmorphism and Severe Vascular Phenotype in TRNT1 Deficiency with Concomitant Antithrombin III Deficiency.

TRNT1 deficiency (SIFD syndrome) is a rare inborn error of immunity characterized by sideroblastic anemia, immunodeficiency, periodic fevers, and developmental delay. We report two Romanian patients with genetically confirmed TRNT1 deficiency presenting with characteristic hematologic and immunologic abnormalities and a distinctive facial dysmorphism. One patient additionally developed severe gastrointestinal ischemia and intracranial hemorrhage in the setting of concomitant hereditary antithrombin III deficiency. These observations expand the phenotypic spectrum associated with TRNT1 deficiency and highlight the marked clinical variability of this disorder. The contribution of TRNT1-associated inflammation to the vascular phenotype remains speculative and cannot be distinguished from the effects of the concomitant thrombophilic condition.

Humans↗
PubMed1982

Treatment of congenital antithrombin III deficiency with concentrates.

Antithrombin III concentrates were administered to a patient with hereditary AT III deficiency undergoing orthopaedic surgery. The plasma AT III level (heparin cofactor activity) was maintained at values in excess of 100% postoperatively. A mean dose of 0.74 u/kg (range 0.64 - 0.83) of AT III resulted in a 1% increase in AT III level after concentrate infusion. The half-life of AT III (mean 66 h; range 58-76) was shorter during surgery and in the early postoperative period suggesting an increased consumption of the inhibitor. At III antigen was consistently higher than heparin cofactor activity during the first 24 h after concentrate infusions. This discrepancy was associated with the presence in the patient's plasma of an AT III fraction showing a slower mobility than the main AT III peak on two-dimensional immunoelectrophoresis in the presence of heparin. A biological assay is preferred to an immunological assay in monitoring replacement therapy with AT III concentrates.

Adult↗
PubMed1988

Pathophysiology of antithrombin III deficiency.

This article focuses on the pathophysiology of thrombosis in patients with acquired antithrombin III deficiency. Antithrombin III is an important natural inhibitor of the hemostatic mechanism, and a hypercoagulable state is often induced in diseases causing antithrombin III deficiency. Laboratory determination of antithrombin III activity is particularly useful in the clinical evaluation and therapeutic management of patients with disseminated intravascular coagulation, nephrotic syndrome, and severe hepatopathy.

Animals↗
PubMed1990

[Management with antithrombin III concentrate in a pregnant woman with hereditary antithrombin III deficiency].

Pregnant women with hereditary antithrombin III (AT-III) deficiency are frequently associated with thromboembolic disorders. We have treated a pregnant woman with hereditary AT-III deficiency, who had suffered from thromboembolic disorders at her past three gestations, with AT-III concentrate. Dosage of AT-III concentrate to maintain plasma AT-III activity over 80% was 3,500 units per week during second and third trimesters, but more frequent administration was necessary around delivery. In recent reports, pregnant women with hereditary AT-III deficiency had been treated with heparin or warfarin except for during abortion and delivery, in which time AT-III concentrate was widely utilized. But the use of heparin or warfarin during gestation is occasionally harmful, AT-III concentrate should be chosen for management in pregnancy in women with hereditary AT-III deficiency.

Adult↗
PubMed1984

Prophylactic antithrombotic therapy with stanozolol in patients with familial antithrombin III deficiency.

Three patients with familial antithrombin III deficiency underwent a trial of prophylactic antithrombotic therapy with stanozolol. An increase in plasma fibrinolytic activity and antithrombin III was seen. Acute venous thrombosis occurred in two patients; in the first patient thrombosis was precipitated by venography but in the second no precipitating factor was found. Our experience suggests that stanozolol is not a suitable prophylactic agent in patients with familial antithrombin III deficiency.

Antithrombin III↗