Antithrombin III Deficiency in a Japanese Family

1979 ◽  
Author(s):  
M. Nakagawa ◽  
T. Kawamura ◽  
H. Tsuji ◽  
Y. Okajima ◽  
S. Urano ◽  
...  
Keyword(s):  
Antithrombin Iii ◽  
Sinus Thrombosis ◽  
Elevated Serum ◽  
Peptic Ulcers ◽  
Autosomal Dominant Disorder ◽  
Japanese Family ◽  
Vein Thrombosis ◽  
Chromogenic Assay ◽  
History Of ◽  
Antithrombin Iii Deficiency

Antithrombin III has been reported to be decreased in the cases of several thrombotic disorders and the decreased Antithrombin III is known to induce the hypercoagulable state. This study was started from the 28 year-old male patient who developed the superior sagital sinus thrombosis after appendectomy and it was followed by deep vein thrombosis of extremities. Antithrombin III level was 19 mg/dl and activity was 68% by the progressive antithrombin assay and other laboratory examinations were within normal range excepts for the elevated serum lipids. Antithrombin III was assayed for his family members in three consecutive generations by single radial immunodifusion method, coagulation assay, and chromogenic assay. Four out of eight members were confirmed to have low Antithrombin III level and activity ranging from 59%-68% of normal values, although the two of this four members had no history of thrombosis. Mother of this propositus is deceased, but it was suspected of having the defect of Antithrombin III. History of peptic ulcers were found in all members of this family. The inheritance pattern of Antithrombin III deficiency was characteristic of an autosomal dominant disorder.

Antithrombin III Deficiency in A Japanese Family

1979 ◽  
Author(s):  
M. Nakagawa ◽  
T. Kawamura ◽  
H. Tsuji ◽  
Y. Okajima ◽  
S. Urano ◽  
...  
Keyword(s):  
Antithrombin Iii ◽  
Sinus Thrombosis ◽  
Elevated Serum ◽  
Peptic Ulcers ◽  
Autosomal Dominant Disorder ◽  
Japanese Family ◽  
Vein Thrombosis ◽  
Chromogenic Assay ◽  
History Of ◽  
Antithrombin Iii Deficiency

Antithrombin IEC has been reported to be decreased in the cases of several thrombotic disorders and the decreased Antithrombin III is known to induce the hypercoagulable state. This study was started from the 28 year-old male patient who developed the superior sagital sinus thrombosis after appendectomy and it was followed by deep vein thrombosis of extremities. Antithrombin III level was 19 mg/dl and activity was 68% by the progressive antithrombin assay and other laboratory examinations were within normal range excepts for the elevated serum lipids. Antithrombin III was assayed for his family members in three consecutive generations by single radial immunodifusion method, coagulation assay, and chromogenic assay. Four out of eight members were comfirmed to have low Antithrombin III level and activity ranging from 59%-68% of normal values, although the two of these four members bad no history of thrombosis. Mother of this propositus is deceased, but it was suspected^of having the defect of Antithrombin III. History of peptic ulcers were found in all members of this family. The inheritance pattern of Antithrombin III deficiency was characteristic of an autosomal dominant disorder.

scholarly journals Cerebral Vein Thrombosis due to Hereditary Antithrombin III Deficiency

1993 ◽  
Vol 20 (2) ◽  
pp. 158-161 ◽  
Author(s):  
Paul Tuite ◽  
Ferhaan Ahmad ◽  
Ian Grant ◽  
John D. Stewart ◽  
Stirling Carpenter ◽  
...  
Keyword(s):  
Antithrombin Iii ◽  
Sinus Thrombosis ◽  
Cerebral Vein ◽  
Cerebral Vein Thrombosis ◽  
Vein Thrombosis ◽  
Sagittal Sinus ◽  
Medical Disorders ◽  
Antithrombin Iii Deficiency ◽  
Sagittal Sinus Thrombosis ◽  
Superior Sagittal Sinus Thrombosis

ABSTRACT:Cerebral vein thrombosis, also called superior sagittal sinus thrombosis, is a well recognized clinical and radiologic entity associated with a variety of medical disorders. We report a patient with fatal cerebral vein thrombosis following myelography, in whom the cause was familial antithrombin III (AT3) deficiency. Unsuspected AT3 deficiency should be considered in cases of unexplained cerebral venous thromboses.

The Prevalence of Hereditary Antithrombin-III Deficiency in Patients with a History of Venous Thromboembolism

1985 ◽  
Vol 54 (04) ◽  
pp. 744-745 ◽  
Author(s):  
R Vikydal ◽  
C Korninger ◽  
P A Kyrle ◽  
H Niessner ◽  
I Pabinger ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Antithrombin Iii ◽  
Positive Family History ◽  
Normal Range ◽  
The Family ◽  
History Of ◽  
Antithrombin Iii Deficiency ◽  
Recurrent Venous Thrombosis ◽  
Antithrombin Iii Activity ◽  
Slight Deficiency

SummaryAntithrombin-III activity was determined in 752 patients with a history of venous thrombosis and/or pulmonary embolism. 54 patients (7.18%) had an antithrombin-III activity below the normal range. Among these were 13 patients (1.73%) with proven hereditary deficiency. 14 patients were judged to have probable hereditary antithrombin-III deficiency, because they had a positive family history, but antithrombin-III deficiency could not be verified in other members of the family. In the 27 remaining patients (most of them with only slight deficiency) hereditary antithrombin-III deficiency was unlikely. The prevalence of hereditary antithrombin-III deficiency was higher in patients with recurrent venous thrombosis.

Congenital Antithrombin III Deficiency in 3 Families (7 Affected Members)

1979 ◽  
Author(s):  
J. Conard ◽  
M. Samama ◽  
M. H. Horellou ◽  
B. Cazenave ◽  
P. Griguer ◽  
...  
Keyword(s):  
Deep Vein Thrombosis ◽  
Antithrombin Iii ◽  
Oral Anticoagulants ◽  
Vena Cava ◽  
Oral Contraception ◽  
Vein Thrombosis ◽  
Heparin Treatment ◽  
At Iii ◽  
Antithrombin Iii Deficiency ◽  
Deep Vein

A congenital Antithrombin III (AT III) deficiency affecting 7 members of 3 families is reported.The first throrabo-embolic accidents were observed between the age of 22 and 35 : they were spontaneous or occured after delivery or oral contraception. in one patient, a deep vein thrombosis was observed during heparin treatment. in 2 cases, recurrent pulmonary embolic episodes required vena cava ligation. No thromboembolic accident was observed during oral anticoagulation.AT III was measured by an amidolytic method and by the Mancini method on plasma and serum ; the antithrombin activity was determined on serum by the von Kaulla method. in 7 patients, a decreased AT III was found by all the methods performed. The AT III level was around 50 % in patients treated or not by oral anticoagulants One patient was studied during heparin treatment and then under oral anticoagulants : AT III levels were lower under heparin.

The Significance of the Chronic Anticoagulant Treatment in Recurrent Thromboembolic Caused by Hereditary Antithrombin III Deficiency

1975 ◽  
Author(s):  
I. Nagy ◽  
H. Losonczy
Keyword(s):  
Functional Activity ◽  
Antithrombin Iii ◽  
Young Patients ◽  
The Other ◽  
Anticoagulant Treatment ◽  
Vein Thrombosis ◽  
Modified Method ◽  
Recurrent Venous Thromboembolism ◽  
Antithrombin Iii Deficiency ◽  
Deep Vein

It has been known since the publication of Egeberg (1965, 1970) and Marciniak (1974) that herditary antithrombin III deficiency could be the cause of recurrent venous thromboembolism.The authors observed in 5 cases of severe repeated venous thrombosis in young patients an antithrombin III decrease, which proved to be a hereditary abnormality. In the case of a 15 years old girl the late introduced anticoagulant treatment could not save the life of the patient, she died after repeated deep vein thrombosis. In the other cases the long-lasting anticoagulant treatment resulted in a perfect clinical improve, while the behaviour of antithrombin III was different; in some cases its quantity (determined by radial -immunodiffusion) and functional activity (examined by modified method of Gerendás and Rák) remained decreased, while in the other cases its functional activity increased during the anticoagulant treatment as it was found by Marciniak, too.It is most likely, that there are two types of hereditary antithrombin III decrease; in one of them the quantitative and functional decrease goes parallel, in the other there is mainly a functional decrease, which improves during the chronic anticoagulant treatment. The authors demonstrated the significance of the prolonged anticoagulant treatment in the patients with hereditary antithrombin III decrease.

scholarly journals Familial Thrombosis Due to Antithrombin III Deficiency: An Extensive Family Study

1977 ◽  
Author(s):  
Bruce Bennett ◽  
Michael Mackie ◽  
Alexander S. Douglas
Keyword(s):  
Small Bowel ◽  
Antithrombin Iii ◽  
Massive Pulmonary Embolism ◽  
Autosomal Dominant Disorder ◽  
Bowel Infarction ◽  
North West ◽  
Mesenteric Vein ◽  
The North ◽  
The Family ◽  
Antithrombin Iii Deficiency

A family living in Lewis (a Hebridean Island off the north west coast of Scotland) affected by antithrombin III deficiency has been studied. Two members have died, one of massive pulmonary embolism and one of major mesenteric infarction secondary to mesenteric vein occlusion. A further individual has sustained major small bowel infarction secondary to mesenteric vein thrombosis but survived after two small bowel resections followed by anticoagulant therapy. Other members of the family have suffered from nonfatal thrombotic events particularly during pregnancy. 57 individuals representing several generations have been studied and will be presented. Levels of antithrombin III measured by functional assays correlated well with those of antithrombin III measured immunologically. 12 members of the family showed moderate to severe deficiency of antithrombin III, the occurrence of thrombotic symptoms correlating well with deficiency of this protein. Transmission of the disorder as an autosomal dominant disorder is confirmed in the patients studied and by historical evidence over many generations.

Portal Vein Thrombosis Due To Antithrombin III Deficiency

Angiology ◽  
1995 ◽  
Vol 46 (11) ◽  
pp. 1043-1047 ◽  
Author(s):  
Yoshikazu Yonemitsu ◽  
Katsuhiko Yanaga ◽  
Takashi Matsumata ◽  
Keizo Sugimachi
Keyword(s):  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Antithrombin Iii ◽  
Vein Thrombosis ◽  
Antithrombin Iii Deficiency

Inherited Deficiency Of Antithrombin III In Two Italian Families. Different Behaviour After Long-Term Anticoagulant Treatment

1981 ◽  
Author(s):  
C Manotti ◽  
M Pini ◽  
R Poti ◽  
R Quintavalia
Keyword(s):  
Antithrombin Iii ◽  
Oral Anticoagulants ◽  
Immunological Methods ◽  
Anticoagulant Treatment ◽  
Normal Pattern ◽  
Vein Thrombosis ◽  
Congenital Deficiency ◽  
At Iii ◽  
History Of

An inherited deficiency of antithrombin III (AT III), measured with four different, functional and immunological, methods, was found in 8 out of 11 examined members and in 3 out of 11 examined members of two Italian families (D.M. and A. families). Biological activity, measured with Abildgard’s clotting assay and with an amidolytic method, ranged between 17 and 75%. Cross immunoelectrophoresis, with or without heparin, performed in the two propositi and in 1; other relatives, showed a normal pattern of migration.A different behaviour of AT III after anticoagulation with acenocoumarin was seen in two long-term treated subjects. The proposita of the D.M. family, who had a history of recurrent thrombotic accidents, did not show any increase of AT III levels, measured in the first two weeks and after 6 and 12 months of therapy. A significant (about 50%) increase both with the functional and immunological methods was on the contrary observed in the propositus of A. family, who had undergone surgery because of mesenteric vein thrombosis. Until now both patients have been free of thrombotic recurrences.Our findings confirm previous reports of variable effects of oral anticoagulants on AT III levels in subjects with congenital deficiency.

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