In congenital factor VII deficiency the clinical picture is related to the levels of factor VII coagulant activity; when factor VII:C levels are very low the bleeding episodes can occur frequently. The most frequent bleedings are menorrhagia and metrorrhagia in females and hemarthrosis in both sexes. There are 3 immunological variants of factor VII deficiency: VII-, VIIR and VII+. Conversely, the genetic variants are 2: one characterized by no discrepancy between VII:C and VII:Ag (found in the heterozygotes of VII- and VIIR variants) and the other, in which a discrepancy between VII:C and VII:Ag is found (heterozygotes from VII+ kindreds). In factor VII deficiency, most commonly the human and ox tissue factors show the highest sensitivity to the coagulation defect, whereas the one extracted from rabbits is definitely less sensitive; the definition of functional variants is based upon a different reactivity to homologous and/or heterologous tissue thromboplastins. In no case was a PIVKA-VII-like protein found and none of the factor VII defective molecules reacted to the generation of important kallikrein activity.

ACTOR VII CONGENITAL DEFICIENCY: CLINICAL PICTURE AND CLASSIFICATION OF THE VARIANTS / G., Mariani; Mazzucconi, Maria Gabriella. - In: HAEMOSTASIS. - ISSN 0301-0147. - 13:(1983), pp. 169-177.

ACTOR VII CONGENITAL DEFICIENCY: CLINICAL PICTURE AND CLASSIFICATION OF THE VARIANTS

MAZZUCCONI, Maria Gabriella
1983

Abstract

In congenital factor VII deficiency the clinical picture is related to the levels of factor VII coagulant activity; when factor VII:C levels are very low the bleeding episodes can occur frequently. The most frequent bleedings are menorrhagia and metrorrhagia in females and hemarthrosis in both sexes. There are 3 immunological variants of factor VII deficiency: VII-, VIIR and VII+. Conversely, the genetic variants are 2: one characterized by no discrepancy between VII:C and VII:Ag (found in the heterozygotes of VII- and VIIR variants) and the other, in which a discrepancy between VII:C and VII:Ag is found (heterozygotes from VII+ kindreds). In factor VII deficiency, most commonly the human and ox tissue factors show the highest sensitivity to the coagulation defect, whereas the one extracted from rabbits is definitely less sensitive; the definition of functional variants is based upon a different reactivity to homologous and/or heterologous tissue thromboplastins. In no case was a PIVKA-VII-like protein found and none of the factor VII defective molecules reacted to the generation of important kallikrein activity.
1983
01 Pubblicazione su rivista::01a Articolo in rivista
ACTOR VII CONGENITAL DEFICIENCY: CLINICAL PICTURE AND CLASSIFICATION OF THE VARIANTS / G., Mariani; Mazzucconi, Maria Gabriella. - In: HAEMOSTASIS. - ISSN 0301-0147. - 13:(1983), pp. 169-177.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/415024
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