A new case of total kininogen deficiency.
Thromb Res
; 60(6): 457-67, 1990 Dec 15.
Article
em En
| MEDLINE
| ID: mdl-2127962
ABSTRACT
Six families with total kininogen deficiency have been described in the literature. We report herein an additional case in a Pakistanese woman. The defect was discovered accidentally due to lack of normal clot formation in a preoperative routine blood sample. She had a borderline prolonged bleeding time, and reported occasional hematuria, but was otherwise without symptoms. Absence of both kininogen species was proven by functional and immunological methods, and by lack of kinin formation both by plasma kallikrein and hog pancreas kallikrein. Prekallikrein was reduced, probably because the main part circulates complexed to high molecular kininogen. Activation of intrinsic fibrinolysis was grossly hampered, and cold activation of coagulation absent with epsilonaminocaproic acid and greatly retarded by dextran sulfate, kaolin and ellagic acid. Together with other evidence the findings indicate the following order of importance for contact activation in plasma--F.XII, high molecular weight kininogen, prekallikrein.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Cininogênios
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
Thromb Res
Ano de publicação:
1990
Tipo de documento:
Article
País de afiliação:
Noruega