Congenital Spca Deficiency: A Hitherto Unrecognized Coagulation Defect With Hemorrhage Rectified by Serum and Serum Fractions 1

Alexander, Benjamin; Goldstein, Robert H.; Landwehr, Greta; Cook, Charlotte; Addelson, Eunice; Wilson, Claire

doi:10.1172/jci102477

Cited by 272 publications

(110 citation statements)

References 14 publications

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“…and 720 volts. Under these conditions the thermocouple readings on the curtain during electrophoresis were 180 C. Before starting electrophoresis it was important to allow the instrument to stabilize for at least (11,12) and Stuart factor (13). D PAPER STRIP "Glass factor" is a term used to express the ability of certain fractions or of plasma treated with glass to shorten the recalcification time and improve prothrombin current applied.…”

Section: Methodsmentioning

confidence: 99%

Application of Continuous Flow Electrophoresis to the Study of the Blood Coagulation Proteins and the Fibrinolytic Enzyme System. I. Normal Human Materials1

Lewis¹,

Walters²,

Didisheim³

et al. 1958

J. Clin. Invest.

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Continuous flow filter paper electrophoresis, as developed by Svensson and Brattsen (1), Grassmann and Hannig (2-4) and Durrum (5), offers a method of plasma fractionation in which protein denaturation appears minimal. This study involves the testing of such fractions for biological activity in various coagulation systems. With the exception of fibrinogen, the coagulation proteins are present in plasma in minute amounts which, at the present time, can be assayed only by their activities in specific clotting systems. Many of these coagulation factors are readily denatured; thus, rigid control with particular attention to temperature is necessary to obtain good recovery of biological activity.Previous electrophoretic studies concerning distribution of plasma coagulation proteins have employed paper electrophoresis with elutions from cut strips. Owen and McKenzie (6) found prothrombin to coincide with a2 globulin, proconvertin (stable conversion factor) with ft globulin, proaccelerin (labile conversion factor) in a variable area between y and f8 peaks, antithrombin between /3 and a2, and fibrinogen remaining at the point of application. Using the same technique, Frick and Hagen (7) found Hageman factor activity in a fraction located between ft and -y globulin. On the other hand, the electrophoretic mobility of purified prothrombin was described by Seegers, McClaughry, and Fahey (8) as approximately equivalent to that of a, globulin. The

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Section: Methodsmentioning

confidence: 99%

Application of Continuous Flow Electrophoresis to the Study of the Blood Coagulation Proteins and the Fibrinolytic Enzyme System. I. Normal Human Materials1

Lewis¹,

Walters²,

Didisheim³

et al. 1958

J. Clin. Invest.

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“…(17,18) During the first half of the 20th century, the remaining coagulation enzymes and co-factors were identified. (19)(20)(21)(22)(23)(24)(25) It has also become clear that coagulation proteins are primarily produced by the liver. Factors II, VII, IX, and X are vitamin K-dependent proteins, which require gamma-glutamyl carboxylation to become biologically active.…”

Section: Introductionmentioning

confidence: 99%

The blood coagulation system as a molecular machine

2003

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The human blood coagulation system comprises a series of linked glycoproteins that upon activation induce the generation of downstream enzymes ultimately forming fibrin. This process is primarily important to arrest bleeding (hemostasis). Hemostasis is a typical example of a molecular machine, where the assembly of substrates, enzymes, protein cofactors and calcium ions on a phospholipid surface markedly accelerates the rate of coagulation. Excess, pathological, coagulation activity occurs in “thrombosis”, the formation of an intravascular clot, which in the most dramatic form precipitates in the microvasculature as disseminated intravascular coagulation. Thrombosis occurs according to a biochemical machine model in the case of atherothrombosis on a ruptured atherosclerotic plaque, but may develop at a slower rate in venous thrombosis, illustrating that the coagulation machinery can act at different velocities. The separate coagulation enzymes are also important in other biological processes, including inflammation for which the rapid conversion of one coagulation factor by the other is not a prerequisite. The latter role of coagulation enzymes may be related to the old and probably maintained function of the coagulation machine in innate immunity. BioEssays 25:1220–1228, 2003. © 2003 Wiley Perioicals, Inc.

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“…In their original description of congenital proconvertin 1 deficiency, Alexander, Goldstein, Landwehr and Cook (6) indicated that the corrective effect of normal plasma or serum transfusion was lost within 24 hours. After transfusion of whole blood to an infant with severe congenital hypoproconvertinemia, Hitzig and Zollinger (7) found the proconvertin half-life to be 115 to 135 minutes.…”

mentioning

confidence: 99%

Disappearance Rate of Concentrated Proconvertin Extracts in Congenital and Acquired Hypoproconvertinemia*

Hoag¹,

Aggeler²,

Fowell³

1960

J. Clin. Invest.

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While developing methods for extraction and concentration of various adsorbable blood clotting factors (1-3), we found that an extract made by Al(OH)3 adsorption of citrated plasma contained large amounts of proconvertin and only small quantities of prothrombin, Stuart factor (4), and plasma thromboplastin component (PTC) (5). The present report is concerned with the disappearance rate of proconvertin following the rapid intravenous administration of this extract to nine patients with congenital or acquired hypoproconvertinemia.In their original description of congenital proconvertin 1 deficiency, Alexander, Goldstein, Landwehr and Cook (6) indicated that the corrective effect of normal plasma or serum transfusion was lost within 24 hours. After transfusion of whole blood to an infant with severe congenital hypoproconvertinemia, Hitzig and Zollinger (7) found the proconvertin half-life to be 115 to 135 minutes. The half-life was 57 minutes following injection of 20 ml of an extract having a proconvertin potency 30 times that of normal plasma.Frick (8) stated that the half-life of proconvertin was 5.5 to 6 hours following plasma transfusion in normal subjects made hypoproconvertinemic by the intravenous administration of Warfarin sodium.2 Didisheim, Loeb, Blatrix and Soulier (9) stated that the half-life of proconvertin was 50 to 55 hours, following intravenous admin-*

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Congenital Spca Deficiency: A Hitherto Unrecognized Coagulation Defect With Hemorrhage Rectified by Serum and Serum Fractions 1

Cited by 272 publications

References 14 publications

Application of Continuous Flow Electrophoresis to the Study of the Blood Coagulation Proteins and the Fibrinolytic Enzyme System. I. Normal Human Materials1

Application of Continuous Flow Electrophoresis to the Study of the Blood Coagulation Proteins and the Fibrinolytic Enzyme System. I. Normal Human Materials1

The blood coagulation system as a molecular machine

Disappearance Rate of Concentrated Proconvertin Extracts in Congenital and Acquired Hypoproconvertinemia*

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