https://mmbr.asm.org/content/mmbr/27/2/117.full.pdf

The inflammatory response

Hereditary angioneurotic edema. I. Case reports and review of the literature.

Flaujeac trait plasma resembled Hageman trait or Fletcher trait, in that the intrinsic coagulation pathway, plasma fibinolytic pathway, kinin-forming system, permeability factor of dilution (PF/dil) phenomenon were abnormal. The defect in each assay was reconstituted by afactor separable from Hageman factor or Fletcher factor. This substance was an alpha-globulin with an approximate mol wt of 170,000. Flaujeac plasma did not release a kinin upon incubation with kallikrein and was deficient in total kininogen antigen. Antiserum to kininogen inhibited the activity of the factor in solution. Flaufeac factor was identified as a kininogen of high molecular weight (HMW-kininogen). The mean total kininogen antigen in four children of the proposita was 51% (range 34-62%) of normal. A functional coagulation assay of HMW-kininogen in the children was 34% (range 23-55%). The results were consistent with autosomal recessive inheritance. The plasma pathways of intrinsic coagulation, fibrinolysis, kinin formation, and PF/dil generation are dependent upon HMW-kininogen. We believe this is the first demonstration of biological function for a kininogen apart from its role as a substrate for kallikreins.

/pdf/flaujeac-trait-deficiency-of-human-plasma-kininogen-3lxviessss.pdf

Flaujeac trait. Deficiency of human plasma kininogen.

It has long been established that the blood vessels, in inflammatory foci of almost any variety, becorne abnorrnally permeable. During the past few years, structural and functional studies have brought out the fact that there are severa1 types of pathologic vascular leakage. At the ‘time of this writing, we can list four, each of which can be studied experimentally in a relatively “pure” forrn: ( 1 ) the histurnine-type leakage; (2 ) the leakage of direct vascular injury; ( 3 ) the leakage of the delnyed-prolonpecl responsr; and last (4 ) the leakage of regenerating capillaries-eharacteristic of granulation tissue-which is described i n this monograph by G. I. Schoefl.] The purpose of this presentation is to describe and compare, in surnmary forrn, the first three types of vascular leakage mentioned abovc.

A light and electron microscopic analysis of vascular injury.

Christie, Graham-Stewart & Ingram (1960) described an abdominoperineal excision of the rectum in a haemophilic of moderate severity who received animal antihaemophilic globulin (AHG) to cover the operation and post-operative period. Responses to this material were measured by assaying the AHG activity in the patient's plasma before and after each dose; and on two occasions the responses were strikingly high. These peaks corresponded to episodes of acute haemorrhage requiring immediate blood transfusion; and there was also an intermediate peak coinciding with a lesser haemorrhage insufficient to necessitate transfusion. It was not clear whether the high responses correlated with the severity of the bleeding or with blood transfusion; but in another haemophilic an unexpectedly high AHG level was associated with haematemesis while the patient was under observation only. It seemed therefore that haemorrhage was the correlated factor; and in considering a possible mechanism, the extensive previous work on the acceleration of blood coagulation after the administration of adrenaline was recalled: this was reviewed by Forwell (1955), and the problem was re-examined by Forwell & Ingram (1957), who thought at that time that the effect depended on an increase in the activity of Factor V. A possible explanation of the observations in the haemophilics was that haemorrhage had led to a release of adrenaline (Greever & Watts, 1959), and that this had in turn brought about an increase in the activity ofAHG in the patients' plasmas. Forwell & Ingram (1957) had not examined AHG in their experimental subjects who had received adrenaline; it therefore seemed of interest to extend the earlier work to include AHG. The results of these experiments are now presented.

https://physoc.onlinelibrary.wiley.com/doi/pdf/10.1113/jphysiol.1961.sp006670

Increase in antihaemophilic globulin activity following infusion of adrenaline

Susceptibility of the Guinea Pig to Pharmacological Factors from its Own Serum

Enzyme-like globulins from serum reproducing the vascular phenomena of inflammation. IV. Activable permeability factor and its inhibitor in the serum of the rat and the rabbit.

The kininogens of plasma kallikrein and plasmin were separable as distinct substrates by molecular sieve chromatography through Sephadex G-200 from a human kininogen. Both substrates were α-globulins and the separation shows that plasmin forms kinin by direct action on a substrate.

The separation of the substrates for plasma kallikrein and plasmin from a human kininogen.

Enzyme-like globulins from serum reproducing the vascular phenomena of inflammation. II. Isolation and properties of the permeability factor and its inhibitor.

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Susceptibility of the Guinea Pig to Pharmacological Factors from its Own Serum

Enzyme-like globulins from serum reproducing the vascular phenomena of inflammation. IV. Activable permeability factor and its inhibitor in the serum of the rat and the rabbit.

The separation of the substrates for plasma kallikrein and plasmin from a human kininogen.

Enzyme-like globulins from serum reproducing the vascular phenomena of inflammation. II. Isolation and properties of the permeability factor and its inhibitor.