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PubMed1983

[Prevention of the thromboembolic risk during the operative period in a patient with a congenital antithrombin III deficiency].

The case of a woman with congenital antithrombin III (AT III) deficiency undergoing elective hysterectomy is reported. AT III deficient patients have high risks of thromboembolic disease, especially developing during and after surgery. It is usual practice to discontinue oral anticoagulants with antivitamin K activity and to obtain effective AT III activity by the transfusion of freeze-dried concentrates of AT III and/or fresh frozen plasma (FFP). Heparin is prescribed as soon as AT III activity reaches 70%. The oral anticoagulant regimen is reinstituted in the late postoperative period. In our case, transfusion of FFP, in the pre- and postoperative periods, results in an AT III activity of 63%. Heparin was not used because oral anticoagulants were readministered early, 12 h after operation. No clinical thrombo-embolic complication was observed.

Adult↗
PubMed1985

[Cerebral venous thrombosis with familial antithrombin III deficiency].

A case of cerebral venous thrombosis with familial antithrombin III (AT III) deficiency was reported and we discussed the anticoagulant therapy of cerebral venous thrombosis from the viewpoint of AT III. The patient, a 17-year-old boy, was admitted to our clinic with severe bifrontal headache, generalized convulsions and progressive disturbance of consciousness. He developed deep vein thrombosis in his right leg and pulmonary emboli two years earlier when he was placed on heparin and so forth, followed by warfarin sodium. Warfarin was terminated 9 months prior to his recent illness. On neurological examination on admission, he was semicomatous with blurred disc margins, roving eye movements with right abducens nerve palsy, nuchal stiffness and right flaccid hemiplegia. Left carotid angiogram and CT scan revealed extensive superior sagittal sinus thrombosis, complicated with hemorrhagic infarcts in bilateral frontal lobes. When examined for coagulation studies, the patient and his father had decrease in AT III activity and antigen levels. He was treated successfully with antiedematous agents and anticonvulsants during acute phase of illness. He was thereafter placed on warfarin 5-6 mg/day with no further clinical thromboembolic event for 2 years 9 months. There was no neurological abnormality when he was last examined, although he was treated with valproic acid 1,200 mg/day and phenytoin 250 mg/day to control occasional adversive seizures. A coagulation study following infusion of 5,000 units of AT III was carried out. Warfarin was discontinued the day before the study. 0.64 U/kg of AT III administration resulted in a 1% increase in AT III level after the infusion. The biological half life of AT III was 14.4 hours.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

Evaluation of the safety, recovery, half-life, and clinical efficacy of antithrombin III (human) in patients with hereditary antithrombin III deficiency. Cooperative Study Group.

Antithrombin III (Human) (AT III) was administered to 18 patients with documented hereditary AT III deficiency. In eight patients with no ongoing clinical symptoms of thrombosis, the percent increase per unit AT III infused per kilogram of body weight ranged from 1.56% to 2.74%, and the half-life from 43.3 to 77.0 hours. No significant difference was noted between patients receiving and those not receiving coumarin therapy. In clinically ill patients, the in vivo recovery was significantly lower and ranged from 0.64% to 1.90% increase per unit AT III infused/kg. Efficacy of AT III was evaluated in 13 patients for the prevention or treatment of thrombosis. AT III was efficacious as assessed by the absence of thrombotic complications after surgery and/or parturition, and the nonextension and nonrecurrence of thrombosis in patients exhibiting an acute thrombotic episode. No side effects were noted. Follow-up studies indicated no hepatitis B seroconversion and no alanine aminotransferase elevations in patients who were not transfused with other blood products.

Adult↗
PubMed1995

Use of antithrombin III concentrates to correct antithrombin III deficiency during vascular surgery.

Congenital deficiency of antithrombin III (AT III) is the only inherited hypercoagulable disorder for which a concentrate of purified protein is available for replacement therapy during periods of increased thrombotic risk. This report describes how such concentrates have been used in a patient with congenital AT-III deficiency undergoing venous surgery. A 40-year-old woman with AT III deficiency was evaluated for bilateral grade 3 chronic venous insufficiency. Noninvasive venous assessment and ascending venography revealed incompetence of the lower leg perforators, a patent deep venous system, and competent greater and lesser saphenous veins. Staged subfascial ligations were performed. Pasteurized AT III was administered 1 hour before surgery and at 30 hours at a dose calculated to increase AT-III activity to at least 120%. Perioperative AT III activity levels were measured. Subcutaneous heparin and oral warfarin were initiated the evening of surgery. An infusion of AT III increased plasma AT III from the baseline activity of 51% to 180%; it was 87% 13 hours later. Two measurements of the initial half-life of AT III were 7 and 14 hours. No perioperative thrombotic complications occurred. The ulcers healed, and the patient remains symptom free. Pasteurized AT III concentrates are now commercially available, easily administered, and provide a useful adjunct to the anticoagulation regimen of patients with AT III deficiency undergoing vascular surgery.

Adult↗
PubMed2003

Pulmonary thromboembolectomy in congenital antithrombin III deficiency associated with acute pulmonary embolism -report of a case-.

Antithrombin III (AT III) deficiency is a rare hereditary disease that predisposes to thromboembolic complications. We report a case of AT III deficiency complicated with acute pulmonary thromboembolism, successfully treated with emergency pulmonary thromboembolectomy after insertion of an inferior vena cava filter. AT III activity before treatment was found to be 44% of normal value and remained less than 50% of normal throughout the postoperative course. In his family line, both the patient's aunt and deceased father had a history of pulmonary infarction. AT III activity of the patient's aunt was 47 to 58% of normal value. The patient was discharged on the 15th day after surgery and has been doing well for four years receiving warfarin as anticoagulant therapy. Careful follow-up is essential for early detection of the recurrent pulmonary thromboembolism resulting in pulmonary hypertension and/or right heart failure.

Acute Disease↗
PubMed1983

Arterial thromboembolism and associated antithrombin III deficiency.

A patient with antithrombin III (ATT) deficiency after arterial embolism manifested an increasing requirement for heparin to adjust a partial thromboplastin time. This was corrected by dramatically increasing doses of heparin and by conversion to warfarin sodium. "Heparin-resistance" should direct the physician to examine antithrombin III levels, and ATT levels should be determined as a baseline before anticoagulation therapy.

Antithrombin III Deficiency↗
PubMed1984

Antithrombin III deficiency: clinical aspects.

The clinical picture of antithrombin III (AT-III) deficiency is characterized by early manifestations of serious venous thromboembolism. The inheritance is autosomal dominant. The precipitating factors of the clinical signs are infections, trauma and pregnancy. For a correct diagnosis, application of various methods (immunological, amidolytic, electrophoresis) are needed. There are several types of AT-III defect; they are characterized by a decrease in amount and function, a functional decrease, or a pathological heparin-binding. Adequate treatment of thromboembolic episodes caused by AT-III deficiency is a lifelong coumarin therapy. In pregnancy and after surgery heparin can only be given together with AT-III substitution. The decreased AT-III activity may change during treatment and this has a diagnostic importance.

Antithrombin III Deficiency↗
PubMed1994

Detection of multiple thromboembolism in congenital antithrombin III deficiency with indium-111-platelet scintigraphy.

Congenital deficiency of antithrombin III (AT III), a physiological inhibitor of blood coagulation, may lead to increased thromboembolism. Here, we report a case of multiple thromboembolism associated with congenital AT III deficiency. The diagnosis of systemic thromboembolism was made by 111In-labeled platelet scintigraphy (In-scan) which allowed for the daily assessment of thromboemboli size. Thromboemboli were found not only in sites predicted clinically, but also in unexpected sites. In these patients, thus, a careful whole-body examination is necessary for full detection of thromboemboli. In-scan may be a useful technique for such screening.

Adolescent↗
PubMed1980

Inherited antithrombin III deficiency and cerebral thrombosis in a child.

Identification of a family affected by antithrombin III-heparin cofactor (AT-III) deficiency was made after diagnosis of the index case, a 15-year-old boy who suffered cerebral thrombosis. The proband had a two-year history of recurrent thrombosis involving the lower extremities. His mother and sister were also affected. Studies showed a decreased biological activity (AT-IIIc) and antigen (AT-IIIag) by the Laurell technique in the proband (AT-IIIc = 0.32, AT-IIIag = 46%), his sister (AT-IIIc = 0.29, AT-IIIag = 47%), and his mother (AT-IIIc = 0.41, AT-IIIag = 56%). Crossed immunoelectrophoresis (CIE) of the affected individuals' plasma in agarose-containing heparin demonstrated a normal pattern of migration. Treatment with warfarin sodium (Coumadin) resulted in an increase in activity in two of three affected family members, and in antigen in all three. Anticoagulant therapy did not affect the pattern of AT-III on CIE. This family represents a quantitative deficiency in antithrombin III. A review of the reported cases of antithrombin III deficiency indicates that individuals with this disorder may have thromboembolic disease in childhood.

Adolescent↗
PubMed2003

Effects of argatroban as an anticoagulant for haemodialysis in patients with antithrombin III deficiency.

BACKGROUND: In congenital or acquired antithrombin III-deficient patients undergoing haemodialysis, coagulation or residual blood in the blood circuit and dialyser is commonly observed under anticoagulation with heparin. Argatroban, a synthetic thrombin antagonist, directly inhibits thrombin activity in a manner that is different from that of heparin, thereby displaying an anticoagulating effect without the activation of antithrombin III. For this reason, the anticoagulating effect of argatroban in haemodialysis patients with antithrombin III deficiency was investigated. METHODS: A retrospective nationwide survey was conducted among patients with congenital or acquired antithrombin III deficiency who had undergone haemodialysis with argatroban as an anticoagulant from April 1996 to April 2000. Inclusion criteria were patients with antithrombin III activity <70% of normal, and patients in whom blood coagulation or residual blood in the extracorporeal circuit could not be prevented by the use of heparin during haemodialysis. RESULTS: Of 80 patients who underwent haemodialysis with argatroban, 59 met the inclusion criteria. Compared with the data before the administration of argatroban, significant improvements of residual blood in the dialyser and arterial and venous drip chambers were observed at the last administration of argatroban. A significant rise in antithrombin III activity was also observed. Among 80 safety analysis cases, no adverse events were reported in 66 patients (82.5%). As severe adverse events, one showed bleeding tendency and one had prolongation of prothrombin time. CONCLUSION: Argatroban was an effective and safe anticoagulant for haemodialysis in patients with congenital or acquired antithrombin III deficiency.

Aged↗
PubMed1991

Mortality in hereditary antithrombin-III deficiency--1830 to 1989.

To determine whether antithrombin-III (AT-III) deficiency leads to an excess mortality, we studied 171 individuals from ten families with a proven hereditary deficiency. 73 were classified as certainly deficient either by direct measurement of AT-III concentration or by mendelian inheritance patterns. 98 individuals had a high probability (0.5) of deficiency. The 64 deaths recorded did not exceed those expected for the general population adjusted for age, sex, and calendar period. We suggest that a policy of prophylactic anticoagulation for patients with AT-III deficiency cannot be recommended.

Adolescent↗
PubMed1992

[Prophylaxis of thrombosis in a pregnant woman with congenital antithrombin III deficiency: changes in hemostatic molecular markers before the onset of thrombosis].

A pregnant woman with congenital antithrombin III (AT III) deficiency was given AT III concentrate and warfarin at the first and after 36th week of gestation periods and at the other gestation period, respectively. The patient developed thrombosis in the left leg, but fibrinopeptide A (FPA) and thrombin-AT III complex (TAT) had already shown high values one week before, suggesting the possibility of their being forecast markers of thrombosis. The administration of AT III concentrate caused an improvement in thrombosis. Therefore, in case of high FPA and TAT values as determined one or two times a week, the administration of AT III concentrate was thought necessary. In case of warfarin, however, non-administration during the 6th-9th weeks of gestation for the purpose of avoiding teratogenicity and frequent blood coagulation tests taking heed of overdosage for the purpose of avoiding fetal central nervous system abnormalities were suggested necessary. Delivery was uneventful, both mother and child being doing very well; umbilical blood AT III activity was 18%. It is generally difficult for us to form the diagnosis as AT III deficiency only from AT III activity at neonatal stage, but in the present study, we analyzed the restriction fragment length polymorphism of the AT III gene using umbilical blood, and succeeded in diagnosing the neonatal child as AT III deficiency.

Adult↗
PubMed1979

Central retinal vein occlusion in a patient with familial antithrombin III deficiency: case report.

A 60-year old man with familial antithrombin III deficiency, a primary cause of hypercoagulation, developed central retinal vein occlusion. Familial antithrombin III deficiency is inherited as an autosomal dominant condition. It is characterized by recurrent episodes of thromboembolism. The occurrence of central retinal vein occlusion in a person with familial deficiency of antithrombin III suggests the possibility of a casual relationship.

Animals↗
PubMed1985

Hereditary antithrombin III deficiency and pregnancy: report of two cases and review of the literature.

The pregnancy and the serum antithrombin III levels during the antenatal and postpartum period of two patients with hereditary antithrombin III deficiency is described. Both antithrombin III antigen and activity levels dropped to their lowest levels immediately after delivery. A review of the literature emphasizes the high risk for thromboembolism in patients with hereditary antithrombin III deficiency. Important considerations for the obstetrician concerning hereditary antithrombin III deficiency are discussed, including: 1) the need to therapeutically anticoagulate these patients postpartum, 2) the need to consider prophylactic anticoagulation throughout pregnancy especially in patients with a history of thrombosis, 3) the practical aspects of assaying antithrombin III in plasma rather than serum, 4) the normally low antithrombin III levels in normal newborns, and 5) the need to provide prepregnancy counseling, including information about the autosomal dominant inheritance of hereditary antithrombin III deficiency.

Adult↗
PubMed1987

Mesenteric venous thrombosis and antithrombin III deficiency.

Of the 123 patients with acute mesenteric infarction treated over the past 12 years, 16 (13%) had mesenteric venous thrombosis. Eight of the patients with mesenteric venous thrombosis survived the initial episode; two have since died. The remaining six patients were studied for evidence of haemostatic deficiencies or abnormalities. Antithrombin III deficiency, which is known to be associated with recurrent venous thrombosis, was found in three patients. It is recommended that all patients with mesenteric venous thrombosis should be screened for antithrombin III deficiency as treatment with coumarin anticoagulants may be indicated, providing effective prophylaxis against further thrombotic episodes.

Adult↗
PubMed1977

Familial thrombosis due to antithrombin III deficiency in a Greek family.

A Greek family with hereditary antithrombin III (AT III) deficiency associated with venous thrombosis is reported. 5 members of the family were affected. In these patients, AT III and heparin cofactor activities were decreased. Immunoreactive AT III showed a positive correlation to both AT III and heparin cofactor activities. alpha2-Macroglobulin and alpha1-antitrypsin were normal. The pattern of inheritance of the defect is autosomal dominant.

Adult↗
PubMed1989

Molecular genetics of inherited antithrombin III deficiencies.

The cloning of antithrombin III (ATIII) complementary deoxyribonucleic acids and the determination of the ATIII gene structure have permitted a systematic evaluation of the molecular basis for inherited ATIII deficiencies. Sixteen kindreds with the most common form of the deficiency, in which plasma ATIII antigen levels and activity are proportionately reduced, were studied. Two polymorphic deoxyribonucleic acid markers were used to resolve parental ATIII alleles and to trace their inheritance patterns. In 15 of 16 cases, the structure of the affected ATIII allele was indistinguishable from normal, suggesting that relatively small mutations, resulting in gene inactivation, are responsible for the lower ATIII levels in these affected families. In the remaining kindred, complete deletion of one ATIII allele was seen. Also investigated was the molecular basis for a qualitative form of ATIII deficiency in a French-Canadian family with normal levels of immunoreactive protein but only half the expected levels of serine protease inhibitor activity. Using polymorphic markers, the abnormal allele was identified, cloned, and partially sequenced from the propositus. A single G----A transition was seen in the first base of codon 382, resulting in an alanine----threonine substitution in the defective protein. This mutation, together with others in this vicinity, defines a minimal length for a fully functional thrombin-binding domain.

Antithrombin III↗