New congenital deficiency of high molecular weight kininogen and prekallikrein (Fitzgerald trait). Study of response to DDAVP and venous occlusion.

Abstract

The prolonged partial thromboplastin time observed in the plasma of a 36 year old asymptomatic man was related to the reduced prekallikrein activities (coagulant; antigenic; and amidolytic) and the absence of coagulant and immunologic activities of high molecular weight kininogen (HMWKg). The patient's plasma also exhibited impaired surface-mediated fibrinolysis and impaired generation of kallikrein. The coagulation defect was identified as the "Fitzgerald trait". The levels of CH50, C2, C4 and C-1 inactivator were normal. Venous occlusion in the patient gave rise to a normal release of extrinsic plasminogen activator from the vascular endothelium. The administration of DDAVP led to a FVIII/VWF response which was similar to that obtained in healthy subjects. No alteration could be observed in the contact phase proteins after DDAVP administration.

• Authors: V Vicente, I Alberca, R Gonzalez, A Alegre, C Redondo, J Moro
• Journal: Haematologia (Haematologia (Budap)) Vol. 19 Issue 1 Pg. 41-8 ( 1986) ISSN: 0017-6559 [Print] Netherlands
• PMID: 3636267 (Publication Type: Case Reports, Journal Article)

Chemical References

• Kininogens
• Kinins
• Protein Precursors
• prekininogens
• Prekallikrein
• Kallikreins
• Deamino Arginine Vasopressin

Topics

• Adult
• Blood Coagulation Disorders (blood, congenital, therapy)
• Deamino Arginine Vasopressin (therapeutic use)
• Humans
• Kallikreins (analysis)
• Kininogens (deficiency)
• Kinins
• Male
• Prekallikrein (analysis)
• Protein Precursors (deficiency)
• Syndrome
• Tourniquets