In Vivo Function of $$C\bar 1$$ -Inhibitor and Pathophysiology of Edema Attack in Patients with Hereditary Angioneurotic Edema

1989 ◽  
pp. 79-86
Author(s):  
J. Kodama ◽  
K. Uchida ◽  
T. Sakata ◽  
F. Funakoshi
Keyword(s):  
Angioneurotic Edema ◽  
Hereditary Angioneurotic Edema

FAMILIAL PAROXYSMAL DYSFUNCTION OF THE AUTONOMIC NERVOUS SYSTEM (A PERIODIC DISEASE), OFTEN PRECIPITATED BY EMOTIONAL STRESS

PEDIATRICS ◽  
1957 ◽  
Vol 20 (5) ◽  
pp. 782-793
Author(s):  
Douglas C. Heiner ◽  
John R. Blitzer
Keyword(s):  
Nervous System ◽  
Autonomic Nervous System ◽  
Angioneurotic Edema ◽  
Dominant Type ◽  
Autonomic Nervous ◽  
Emotional Factors ◽  
System A ◽  
Hereditary Angioneurotic Edema ◽  
Paroxysmal Disorders ◽  
Periodic Disease

Two children and their father, currently under investigation, have displayed paroxysmal symptoms which include a rash, angioneurotic edema, severe symptoms from the gastrointestinal and urinary tracts and other evidences of dysfunction of the autonomic nervous system. Attempts at medicinal therapy failed to ameliorate the attacks. A striking hereditary pattern is present suggesting a simple dominant type of inheritance. Emotional factors have been found to be capable of precipitating these paroxysms which have many features in common with paroxysmal disorders described in the literature under the terms periodic peritonitis, cyclic vomiting, hereditary angioneurotic edema and "periodic disease." It is important that careful evaluation of hereditary and emotional factors be made in all patients exhibiting recurrent symptoms of unexplained etiology. Psychiatric study and psychotherapy may offer a means to alleviate the symptoms in some of these patients.

scholarly journals Hereditary angioneurotic edema … a disease has been described

2017 ◽  
Vol 38 (6) ◽  
pp. 399-400
Author(s):  
Joseph A. Bellanti ◽  
Russell A. Settipane
Keyword(s):  
Angioneurotic Edema ◽  
Hereditary Angioneurotic Edema

Hereditary angioneurotic edema: Clinical and laboratory findings in 58 subjects

1989 ◽  
Vol 19 (1) ◽  
pp. 51-58
Author(s):  
Luigi Fontana ◽  
Roberto Perricone ◽  
Caterina De Carolis ◽  
John G. Pizzolo ◽  
Carlo U. Casciani
Keyword(s):  
Angioneurotic Edema ◽  
Laboratory Findings ◽  
Hereditary Angioneurotic Edema

Clinical Aspects and Therapy of Hereditary Angioneurotic Edema

1981 ◽  
pp. 272-278 ◽  
Author(s):  
W. Opferkuch ◽  
P. M. Kövary ◽  
U. Jaeger ◽  
K. Echternacht-Happle ◽  
W. Gronemeyer ◽  
...  
Keyword(s):  
Clinical Aspects ◽  
Angioneurotic Edema ◽  
Hereditary Angioneurotic Edema

INTERACTIONS OF DYSFUNCTIONAL Cl-INHIBITORS FROM PATIENTS WITH TYPE II HEREDITARY ANGIONEUROTIC EDEMA (HANE) WITH ACTIVATED HAGEMAN FACTOR (FACTOR XIIa).

1987 ◽  
Author(s):  
V H Donaldson ◽  
M D B H Mitchell
Keyword(s):  
Normal Serum ◽  
Type Ii ◽  
Inhibitor Protein ◽  
Amidolytic Activity ◽  
Hageman Factor ◽  
Hereditary Angioneurotic Edema ◽  
Coagulant Activity ◽  
Antigenic Properties ◽  
Factor Xiia

Type II HANE is characterized by a deficiency of Cl-inhibitor (Cl-INH) activity in serum which is associated with a dysfunctional inhibitor protein having a normal or increased quantity o|_the antigenic properties of normal serum Cl-inhibitor. Dysfunctional Cl-INH proteins were purified from members_of eight different kindred with Type II HANE and compared to normal Cl-inhibitor with respect to their inhibitory activity directed against the amidolytic and clot-promoting properties of purified activated Hageman factor. All but one dysfunctional Cl-inhibitor blocked the amidolytic activity of ellagic acid-activated Hageman factor; all eight blocked the clot-promoting activity of Hageman factor activated in solutions of sulfatides and BSA. The inhibition _of amidolytic activity was equal to or greater than that of normal Cl-INH (Donaldson, et al., 3. Clin. Invest. 75:124,1985). The impairment of the specific Hageman factor coagulant activity of activated Hageman factor by six^f the eight dysfunctional inhibitors was less than that of the normal Cl-inhibitor, although readily measured. Dysfunctional Cl-inhibitor proteins were also heterogeneous with respect to their formation of stable complexes and their susceptibility to cleavage by Hageman factor activated with BSA-sulfatides when analyzed in SDS-gel electrophoresis. Although these observatons cannot be directly applied to in vivo pathophysiologic changes in plasma, dysfunctional Cl-inhibitors do have the potential of regulating activated Hageman factor.

scholarly journals Interactions of plasma kallikrein and C1-s with normal and dysfunctional C1(-)-inhibitor proteins from patients with hereditary angioneurotic edema: analytic gel studies

Blood ◽  
1987 ◽  
Vol 69 (4) ◽  
pp. 1096-1101 ◽  
Author(s):  
VH Donaldson ◽  
CJ Wagner ◽  
B Tsuei ◽  
G Kindness ◽  
DH Bing ◽  
...  
Keyword(s):  
Complex Formation ◽  
Gel Electrophoresis ◽  
Sodium Dodecyl ◽  
Original Position ◽  
Stable Complex ◽  
Plasma Kallikrein ◽  
Type Ii ◽  
C1 Inhibitor ◽  
Angioneurotic Edema ◽  
Hereditary Angioneurotic Edema

Abstract Purified preparations of normal C1(-)-inhibitor (C1(-)-INH) formed high mol wt complexes with plasma kallikrein that were stable during sodium dodecyl sulfate (SDS)-gel electrophoresis, but most of the dysfunctional C1(-)-INH proteins isolated from plasma of patients with type II hereditary angioneurotic edema (HANE) did not. Two of eight dysfunctional C1(-)-INH proteins were cleaved to lower mol wt forms that were not seen following the reaction of normal C1(-)-INH with equimolar amounts, or less, of plasma kallikrein. Only the higher mol wt component of normal C1(-)-INH (106,000 mol wt) appeared to form a stable complex with the plasma kallikrein, whereas both the 106,000 and 96,000 mol wt forms made stable complexes with C1-s. When a preparation of normal C1(-)-INH containing a homogeneous single band of C1(-)-INH was exposed to C1-s or kallikrein, a “doublet” form evolved in which the heaviest band was in the original position of native C1(-)-INH; C1- s cleavage provided a second band of 96,000; and cleavage by kallikrein, a second band of 94,000 mol wt. We conclude that dysfunctional C1(-)-INH proteins from plasma of persons with type II hereditary angioneurotic edema have impaired interactions with plasma kallikrein and are heterogeneous with respect to these interactions. Moreover, the requirements for the formation of stable complexes between normal C1(-)-INH and plasma kallikrein differed from those for stable complex formation with C1-s. The doublet form of C1(-)-INH, which purified preparations frequently demonstrate, may be due to prior cleavage by C1-s or kallikrein.

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