Literature DB >> 16695963

Fitzgerald Trait: Deficiency of a Hitherto Unrecognized Agent, Fitzgerald Factor, Participating in Surface-Mediated Reactions of Clotting, Fibrinolysis, Generation of Kinins, and the Property of Diluted Plasma Enhancing Vascular Permeability (PF/Dil).

H Saito1, O D Ratnoff, R Waldmann, J P Abraham.   

Abstract

The prolonged partial thromboplastin time observed in the plasma of a 71-yr-old asymptomatic man was related to the deficiency of a hitherto unrecognized agent. The patient's plasma also exhibited impaired surface-mediated fibrinolysis and esterolytic activity and impaired generation of kinins and of the property enhancing vascular permeability designated PF/Dil. The patient's plasma contained normal amounts of all known clotting factors except Fletcher factor (a plasma prekallikrein) which was present at a concentration of 10-15% of pooled normal plasma. Fletcher trait plasma, however, contained normal amounts of the agent missing from the patient's plasma and corrected the defects in clotting, fibrinolysis, and vascular permeability. Fletcher trait plasma was less effective in correcting generation of kinins and esterolytic activity, presumably because of the patient's partial deficiency of prekallikrein. The site of action of the factor deficient in the patient's plasma appeared to be subsequent to the activation of Hageman factor and plasma prekallikrein. A fraction of normal plasma, devoid of other clotting factors, corrected the defect in clotting in the patient's plasma; a similar fraction of the patient's plasma did not correct this abnormality. No evidence yet exists pointing to the familial nature of the patient's defect. Tentatively, the patient's disorder may be referred to by his surname as Fitzgerald trait, and the agent apparently deficient in his plasma as Fitzgerald factor.

Entities:  

Year:  1975        PMID: 16695963      PMCID: PMC301855          DOI: 10.1172/JCI108009

Source DB:  PubMed          Journal:  J Clin Invest        ISSN: 0021-9738            Impact factor:   14.808


  20 in total

1.  Assay and properties of serum inhibitor of C'l-esterase.

Authors:  L R LEVY; I H LEPOW
Journal:  Proc Soc Exp Biol Med       Date:  1959 Aug-Sep

2.  Defective esterase and kinin-forming activity in human Fletcher trait plasma. A fraction rich in kallikreinlike activity.

Authors:  V H Donaldson; H Saito; O D Ratnoff
Journal:  Circ Res       Date:  1974-05       Impact factor: 17.367

3.  Preparation, characterization, and activation of a highly purified factor XI: evidence that a hitherto unrecognized plasma activity participates in the interaction of factors XI and XII.

Authors:  S Schiffman; P Lee
Journal:  Br J Haematol       Date:  1974-05       Impact factor: 6.998

4.  Studies on a complex mechanism for the activation of plasminogen by kaolin and by chloroform: the participation of Hageman factor and additional cofactors.

Authors:  D Ogston; C M Ogston; O D Ratnoff; C D Forbes
Journal:  J Clin Invest       Date:  1969-10       Impact factor: 14.808

5.  Defective activation of clotting, fibrinolytic, and permeability-enhancing systems in human Fletcher trait plasma.

Authors:  H Saito; O D Ratnoff; V H Donaldson
Journal:  Circ Res       Date:  1974-05       Impact factor: 17.367

6.  The kallikreinogen-kallikrein enzyme system of human plasma. Assay of components and observations in disease states.

Authors:  R W Colman; J W Mason; S Sherry
Journal:  Ann Intern Med       Date:  1969-10       Impact factor: 25.391

7.  Studies on kallikrein: failure of some anti-inflammatory drugs to affect release of kinin.

Authors:  G E Davies; G Holman; T P Johnston; J S Lowe
Journal:  Br J Pharmacol Chemother       Date:  1966-11

8.  Fletcher factor activity in plasmas of various species.

Authors:  H Saito; G Goldsmith; O D Ratnoff
Journal:  Proc Soc Exp Biol Med       Date:  1974-11

9.  Partial purification of plasma thromboplastin antecedent (factor XI) and its activation by trypsin.

Authors:  H Saito; O D Ratnoff; J S Marshall; J Pensky
Journal:  J Clin Invest       Date:  1973-04       Impact factor: 14.808

10.  The fibrinolytic pathway of human plasma. Isolation and characterization of the plasminogen proactivator.

Authors:  A P Kaplan; K F Austen
Journal:  J Exp Med       Date:  1972-12-01       Impact factor: 14.307

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  35 in total

1.  Partial purification and characterization of contact activation cofactor.

Authors:  S Schiffman; P Lee
Journal:  J Clin Invest       Date:  1975-11       Impact factor: 14.808

2.  Purification of high molecular weight kininogen and the role of this agent in blood coagulation.

Authors:  H Saito
Journal:  J Clin Invest       Date:  1977-09       Impact factor: 14.808

3.  Activation and function of human Hageman factor. The role of high molecular weight kininogen and prekallikrein.

Authors:  H L Meier; J V Pierce; R W Colman; A P Kaplan
Journal:  J Clin Invest       Date:  1977-07       Impact factor: 14.808

4.  Flaujeac factor deficiency. Reconstitution with highly purified bovine high molecular weight-kininogen and delineation of a new permeability-enhancing peptide released by plasma kallikrein from bovine high molecular weight-kininogen.

Authors:  R T Matheson; D R Miller; M J Lacombe; Y N Han; S Iwanaga; H Kato; K D wuepper
Journal:  J Clin Invest       Date:  1976-12       Impact factor: 14.808

5.  Prekallikrein deficiency in a kindred with kininogen deficiency and Fitzgerald trait clotting defect. Evidence that high molecular weight kininogen and prekallikrein exist as a complex in normal human plasma.

Authors:  V H Donaldson; J Kleniewski; H Saito; J K Sayed
Journal:  J Clin Invest       Date:  1977-09       Impact factor: 14.808

6.  Potentiation of the function of Hageman factor fragments by high molecular weight kininogen.

Authors:  C Y Liu; C F Scott; A Bagdasarian; J V Pierce; A P Kaplan; R W Colman
Journal:  J Clin Invest       Date:  1977-07       Impact factor: 14.808

7.  Association of factor XI and high molecular weight kininogen in human plasma.

Authors:  R E Thompson; R Mandle; A P Kaplan
Journal:  J Clin Invest       Date:  1977-12       Impact factor: 14.808

8.  Kinetic analysis of the interaction of human tissue kallikrein with single-chain human high and low molecular weight kininogens.

Authors:  M Maier; K F Austen; J Spragg
Journal:  Proc Natl Acad Sci U S A       Date:  1983-07       Impact factor: 11.205

9.  Synthesis and secretion of alpha 2-plasmin inhibitor by established human liver cell lines.

Authors:  H Saito; L T Goodnough; B B Knowles; D P Aden
Journal:  Proc Natl Acad Sci U S A       Date:  1982-09       Impact factor: 11.205

10.  Synthesis and release of Hageman factor (Factor XII) by the isolated perfused rat liver.

Authors:  H Saito; S M Hamilton; A S Tavill; L T Goodnough; L Louis; A Angell
Journal:  J Clin Invest       Date:  1983-09       Impact factor: 14.808

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