Acute Aortic Thrombosis in Antithrombin III Deficiency

Shapiro, Michael

doi:10.1001/jama.1981.03310420049031

Cited by 52 publications

(5 citation statements)

References 7 publications

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“…However, in those studies, an MTAA was detected in a small number of cases without identifi able atherosclerotic changes in the aorta [4,5] . There are scattered reports of various conditions other than atherosclerotic changes that may predispose to intra-aortic thrombosis, such as thrombocythemia [8] , polycythemia [9] , antithrombin III defi ciency [10] , protein C defi ciency [4] , antiphospholipid antibody syndrome [4] , malignant tumor [2] , blunt chest trauma [11] , chest gunshot wound [12] or systemic fungal infection [13] . (However, these abnormalities were not seen in the present cases.)…”

Section: Discussionmentioning

confidence: 99%

Brain Embolism Caused by a Mobile Aortic Thrombus with Iron Deficiency Anemia

Yakushiji

Terasaki²,

Otsubo³

et al. 2005

Cerebrovasc Dis

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Section: Discussionmentioning

confidence: 99%

Brain Embolism Caused by a Mobile Aortic Thrombus with Iron Deficiency Anemia

Yakushiji

Terasaki²,

Otsubo³

et al. 2005

Cerebrovasc Dis

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“…The mechanism of inhibition involves the stoichiometric formation of protease-antiprotease complexes and the rate of complex formation is greatly enhanced in the presence of heparin, which is a well-known anticoagulant used clinically in myocardial infarction and surgery (6,7). Deficiency of antithrombin III is a hereditary disorder that is associated with recurrent thrombophlebitis, acute aortic thrombosis, and thromboembolism (8)(9)(10). Heterogeneity of the classical antithrombin III deficiency has been observed (11).…”

mentioning

confidence: 99%

Isolation and sequence characterization of a cDNA clone of human antithrombin III.

Chandra¹,

Stackhouse²,

Kidd³

et al. 1983

Proc. Natl. Acad. Sci. U.S.A.

138

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A human liver cDNA library was constructed by using poly(A)-containing RNA isolated from a human liver biopsy specimen. This library is comprised of 40,000 independent transformants with an average inserted DNA length of 1,200 base pairs. By using the previously cloned baboon antithrombin III cDNA as a specific hybridization probe, >30 human antithrombin mII cDNA clones were identified from this library. The Antithrombin III is a plasma protease inhibitor synthesized in the liver. The glycoprotein has a molecular weight of 55,000 and its entire amino acid sequence has almost been completed (1). It is a natural anticoagulant in that it specifically inhibits a number of serine proteases that participate in the blood coagulation cascade, including thrombin, factors IXa, Xa, XIa, and XIIa (2-5). The mechanism of inhibition involves the stoichiometric formation of protease-antiprotease complexes and the rate of complex formation is greatly enhanced in the presence of heparin, which is a well-known anticoagulant used clinically in myocardial infarction and surgery (6, 7). Deficiency of antithrombin III is a hereditary disorder that is associated with recurrent thrombophlebitis, acute aortic thrombosis, and thromboembolism (8-10). Heterogeneity of the classical antithrombin III deficiency has been observed (11). Abnormal antithrombin III also has been isolated from deficient patients and partially characterized (12), suggesting that the deficiency could be the result of mutations in the antithrombin III gene itself. Therefore, the genetic deficiency can be analyzed in molecular detail if the human antithrombin III gene can be isolated and characterized. We recently have reported the purification of antithrombin III mRNA from a baboon liver by polysome immunoprecipitation and the cloning of its cDNA (13). In this paper, we report the construction of a human liver cDNA library and the identification and sequence of a full-length human antithrombin III cDNA clone. MATERIALS AND METHODSConstruction of a Human Liver cDNA Library. A human liver biopsy specimen was kindly provided by Bill Williams (University of Texas Medical School in Houston). Total nucleic acid was extracted from the frozen tissue with phenol (14), and poly(A)-containing RNA was prepared by oligo(dT)-cellulose column chromatography (15). The RNA preparation was used for cDNA synthesis under conditions that favored full-length cDNA production (16). The cDNA preparation was sedimented through an alkaline sucrose gradient (16) and only fractions containing cDNA species of 1,000 nucleotides or more were pooled. The single-stranded cDNA was subsequently made double-stranded with reverse transcriptase (17). After nuclease S1 treatment, tracts of poly(dC) were added to the 3' termini of the DNA molecules by using terminal transferase (18). The enzymatically synthesized cDNA was rehybridized with Pst I-linearized pBR322 DNA that had been tailed with poly(dG) and the DNA was used for transformation of Escherichia coli RR1 (18). Bacterial transformants we...

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“…In this deficiency, thrombosis may occur spontaneously or in association with pregnancy, trauma, or surgery, although arterial thrombosis is generally rare [3]. In reports describing arterial thrombus due to antithrombin deficiency, thrombus formation occurred in some aortic arteries, including the renal artery [4,5,6]. We describe a rare pediatric case of renal hypertension caused by renal artery obstruction due to thrombus formation.…”

Section: Introductionmentioning

confidence: 90%

Renovascular hypertension due to antithrombin deficiency in childhood

Miura

Takahashi

et al. 2004

Pediatr Nephrol

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Inherited antithrombin deficiency generally causes a predisposition toward vascular thrombus above the age of 15 years. A 1-year-old boy developed renal hypertension caused by renal artery obstruction due to thrombus formation. This thrombus formation was attributed to antithrombin deficiency caused by a novel SERPINCI gene mutation (AT III Akita, M352R). This suggests that antithrombin deficiency can cause renal artery obstruction, inducing renal hypertension through vascular thrombosis even in children.

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Acute Aortic Thrombosis in Antithrombin III Deficiency

Cited by 52 publications

References 7 publications

Brain Embolism Caused by a Mobile Aortic Thrombus with Iron Deficiency Anemia

Brain Embolism Caused by a Mobile Aortic Thrombus with Iron Deficiency Anemia

Isolation and sequence characterization of a cDNA clone of human antithrombin III.

Renovascular hypertension due to antithrombin deficiency in childhood

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