Hereditary Angioneurotic Edema: Immunochemical ‘Activity’ without Clinical Expression

1980 ◽  
Vol 61 (3) ◽  
pp. 286-292 ◽  
Author(s):  
Roger E. Spitzer ◽  
Ann E. Stitzel ◽  
Joan R. Urmson
Keyword(s):  
Clinical Expression ◽  
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

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.

Anti-Ro-Positive Lupus and Hereditary Angioneurotic Edema

Dermatology ◽  
1988 ◽  
Vol 177 (6) ◽  
pp. 370-376 ◽  
Author(s):  
G. Guillet ◽  
B. Sassolas ◽  
P. Plantin ◽  
J. Cledes ◽  
P. Youinou ◽  
...  
Keyword(s):  
Angioneurotic Edema ◽  
Hereditary Angioneurotic Edema
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