Prekallikrein Deficiency

07/01/1986 - "Studies of plasma prekallikrein in a family with prekallikrein deficiency were made. "
10/15/1981 - "These studies demonstrate the molecular heterogeneity of human prekallikrein deficiency and show that persons with the CRM+ variant have a nonfunctional form of prekallikrein in their plasma."
05/10/1976 - "Therefore, these studies suggest that severe prekallikrein (Fletcher factor) deficiency in man is not associated with any clinically significant impairment in hemostasis, fibrinolysis, inflammatory responses or leukocyte function."
05/10/1976 - "Clinical and physiologic studies of two siblings with prekallikrein (Fletcher factor) deficiency."
01/01/2020 - "Background: Hereditary prekallikrein (Fletcher factor) deficiency is a rare condition characterized by a prolonged activated partial thromboplastin time. "

12/31/1967 - "Distinction from Hageman factor and "Fletcher factor" deficiency; family study; and problems of diagnosis."
01/01/2021 - "Severe Prekallikrein Deficiency Associated with Low Level of Factor XII: A Case Report."
03/15/2002 - "Prekallikrein deficiency: the characteristic normalization of the severely prolonged aPTT following increased preincubation time is due to autoactivation of factor XII."
05/01/1982 - "Severe Fletcher factor (plasma prekallikrein) deficiency with partial deficiency of Hageman factor (factor XII): report of a case with observation on in vivo and in vitro leukocyte chemotaxis."
10/01/1973 - "This chemotactic activity can be distinguished from that generated by Hageman factor activation of prekallikrein and is demonstrable in plasma that is genetically deficient in prekallikrein (Fletcher factor deficiency). "

06/01/2011 - "The mutant allele is predicted to encode a truncated protein lacking half of the catalytic domain of prekallikrein, suggesting that the truncated protein causes prekallikrein deficiency in the patient."
01/01/1979 - "Studies of plasmas from individuals with Hageman trait (factor XII deficiency), plasma thromboplastin antecedent (PTA, factor XI) deficiency, Fletcher trait (plasma prekallikrein deficiency) and Fitzgerald trait (high molecular weight-kininogen deficiency) have revealed the importance of these proteins in blood coagulation. "

01/01/1979 - "Studies of plasmas from individuals with Hageman trait (factor XII deficiency), plasma thromboplastin antecedent (PTA, factor XI) deficiency, Fletcher trait (plasma prekallikrein deficiency) and Fitzgerald trait (high molecular weight-kininogen deficiency) have revealed the importance of these proteins in blood coagulation. "
03/01/1979 - "Collagen activation of platelet-associated Factor XI has been proposed as a mechanism for initiating intrinsic clotting independent of Factor XII. Since this could explain the lack of bleeding in patients with hereditary Factor XII deficiency, prekallikrein deficiency and high molecular weight kininogen deficiency, we subjected the hypothesis to rigorous testing. "

08/01/1985 - "Despite markedly prolonged activated partial thromboplastin times (APTT) patients with Fletcher factor (prekallikrein) deficiency do not clinically bleed. "
05/10/1976 - "One subject's leukocytes showed normal tissue factor production on stimulation by endotoxin, although prekallikrein deficiency did impair the endotoxin-stimulated generation of serum procoagulant activity. "
01/01/2016 - "However, in arterial thrombosis models where reactive oxygen species are generated, prekallikrein deficiency results in downregulation of vessel wall tissue factor generation with reduced thrombin generation. "

01/01/2020 - "The majority of the reports did not indicate any association between prekallikrein deficiency and comorbidities; however, additional observation is required to confirm the long-term safety of plasma kallikrein inhibition."
09/17/2019 - "The majority of the reports did not indicate any association between prekallikrein deficiency and comorbidities; however, additional observation is required to confirm the long-term safety of plasma kallikrein inhibition."

06/01/2011 - "Severe prekallikrein deficiency due to a homozygous Trp499Stop nonsense mutation."
10/01/2004 - "Severe prekallikrein deficiency associated with homozygosity for an Arg94Stop nonsense mutation."

05/01/2009 - "The prekallikrein deficiency results from two mutations in the KLKB 1-gene: first, an insertion of 1 bp in codon 149 in exon 5 and, second, a base exchange Cys 548 (TGC) > Tyr (TAC) in exon 14. "
12/01/2003 - "Severe prekallikrein (Fletcher factor) deficiency due to a compound heterozygosis (383Trp stop codon and Cys529Tyr)."

11/01/2020 - "Complete inhibition of attacks may well require sustained blood levels of C1 inhibitor that exceed 85% of normal, and it may be possible to delete the prekallikrein gene (analogous to familial prekallikrein deficiency), which is the one factor that might alleviate bradykinin formation, even by factor XII-independent initiating mechanisms, with the possible exception of Mannose Associated Serine Protease 1 (MASP-1) cleavage of high molecular weight kininogen (HK). "

11/01/2020 - "Complete inhibition of attacks may well require sustained blood levels of C1 inhibitor that exceed 85% of normal, and it may be possible to delete the prekallikrein gene (analogous to familial prekallikrein deficiency), which is the one factor that might alleviate bradykinin formation, even by factor XII-independent initiating mechanisms, with the possible exception of Mannose Associated Serine Protease 1 (MASP-1) cleavage of high molecular weight kininogen (HK). "

08/01/1992 - "Replacement therapy in a patient with congenital prekallikrein deficiency undergoing surgery."

07/01/2009 - "Fletcher factor deficiency in a woman requiring emergency caesarean section."

08/01/2006 - "Management of anticoagulation for coronary artery bypass surgery in a patient with severe prekallikrein deficiency."

12/01/1996 - "Spinal anesthesia in a patient with prekallikrein deficiency."