» Articles » PMID: 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)

Overview
Journal J Clin Invest
Specialty General Medicine
Date 1975 May 1
PMID 16695963
Citations 35
Authors
Affiliations
Soon will be listed here.
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.

Citing Articles

The complex role of kininogens in hereditary angioedema.

Kaplan A, Joseph K, Ghebrehiwet B Front Allergy. 2022; 3:952753.

PMID: 35991308 PMC: 9382879. DOI: 10.3389/falgy.2022.952753.


Anti-HK antibody reveals critical roles of a 20-residue HK region for Aβ-induced plasma contact system activation.

Chen Z, Kumar Singh P, Horn K, Strickland S, Norris E Blood Adv. 2022; 6(10):3090-3101.

PMID: 35147669 PMC: 9131899. DOI: 10.1182/bloodadvances.2021006612.


The contact activation system and vascular factors as alternative targets for Alzheimer's disease therapy.

Singh P, Badimon A, Chen Z, Strickland S, Norris E Res Pract Thromb Haemost. 2021; 5(4):e12504.

PMID: 33977208 PMC: 8105157. DOI: 10.1002/rth2.12504.


Hereditary angioneurotic oedema: characterization of plasma kinin and vascular permeability-enhancing activities.

Shoemaker L, Schurman S, DONALDSON V, Davis 3rd A Clin Exp Immunol. 1994; 95(1):22-8.

PMID: 8287604 PMC: 1534616. DOI: 10.1111/j.1365-2249.1994.tb06009.x.


Function and immunochemistry of prekallikrein-high molecular weight kininogen complex in plasma.

Scott C, Colman R J Clin Invest. 1980; 65(2):413-21.

PMID: 7356688 PMC: 371379. DOI: 10.1172/JCI109684.


References
1.
Eisen V . Kinin formation and fibrinolysis in human plasma. J Physiol. 1963; 166(3):514-29. PMC: 1359350. DOI: 10.1113/jphysiol.1963.sp007120. View

2.
Ratnoff O, MILES A . THE INDUCTION OF PERMEABILITY-INCREASING ACTIVITY IN HUMAN PLASMA BY ACTIVATED HAGEMAN FACTOR. Br J Exp Pathol. 1964; 45:328-45. PMC: 2093057. View

3.
SCHIFFMAN S, Lee P . 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. Br J Haematol. 1974; 27(1):101-14. DOI: 10.1111/j.1365-2141.1974.tb06778.x. View

4.
Ogston D, OGSTON C, Ratnoff O, Forbes C . Studies on a complex mechanism for the activation of plasminogen by kaolin and by chloroform: the participation of Hageman factor and additional cofactors. J Clin Invest. 1969; 48(10):1786-801. PMC: 322415. DOI: 10.1172/JCI106145. View

5.
Davies G, Holman G, Johnston T, Lowe J . Studies on kallikrein: failure of some anti-inflammatory drugs to affect release of kinin. Br J Pharmacol Chemother. 1966; 28(2):212-7. PMC: 1510866. DOI: 10.1111/j.1476-5381.1966.tb01887.x. View