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Protease inhibitor deficiencies in patients with angio‐oedema
Author(s) -
BLEUMINK E.,
DOEGLAS H.M.G.
Publication year - 1986
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.1986.tb00459.x
Subject(s) - kallikrein , protease inhibitor (pharmacology) , protease , medicine , c1 inhibitor , enzyme inhibitor , gastroenterology , trypsin , endocrinology , alpha (finance) , enzyme , angioedema , chemistry , immunology , biochemistry , surgery , construct validity , human immunodeficiency virus (hiv) , antiretroviral therapy , viral load , patient satisfaction
Summary In 41 patients with recurrent angio‐oedema, studies were undertaken of the plasma protease inhibitor levels, Alpha‐i‐antitrypsin was measured by a Mancini method and the inhibitor levels for C1‐esterase, trypsin, chymotrypsin, urinary kallikrein and plasma kallikrein were determined by enzymatic assays with synthetic esters and chromogenic tripeptides as substrates. The control group consisted of 40 healthy individuals (medical students and department employees). Statistically significant differences between the patient group and controls, of the inhibitor levels for urinary kallikrein and plasma kallikrein, were found ( P = 0.001). Further analysis revealed that the angio‐oedema group could be divided into several subgroups of patients in which protease inhibitor deficiencies were found to be present:1 One patient had a C1‐csterase inhibitor deficiency. This patient also showed an alpha‐1‐antitrypsin deficiency (PI type MZ). 2 Six patients had an alpha‐i‐antitrypsin deficiency; in five of them a Z genotype was found to be present (4 × MZ, i × ZZ), and in one patient an MS PI type could be detected. 3 In seven patients a plasma kallikrein inhibitor deficiency was found to be a prominent feature.It is concluded that besides the C1‐esterase inhibitor other protease inhibitor deficiencies may play a part in the aetiology of angio‐oedema.