Congenital deficiency of factor VII (hypoconvertinemia)

Owen, Charles A.; Amundsen, Melvin A.; Thompson, John H.; Spittell, John A.; Bowie, E. J. W.; Stilwell, George G.; Hewlett, James S.; Mills, Stephen D.; Sauer, William G.; Gage, Robert P.

doi:10.1016/0002-9343(64)90213-x

Cited by 40 publications

(9 citation statements)

References 95 publications

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“…This observation is in agreement with in vitro data which indicate that these patients lack inhibitors in their plasma [6]. It is interesting to note that similar results have been obtained by us in another abnor mality of the prothrombin complex factors, namely factor X Friuli disorder [4], The survival times observed are well in line with those reported in the literature [1,2,[8][9][10][11][12]. However, it is worth mentioning that some of the studies carried out dealt with the infu sion of large quantities of plasma which made all but impossible to calculate a satis factory half-life.…”

Section: Discussionsupporting

confidence: 90%

Factor VII Survival Studies in Factor VII Padua Abnormality

Girolami¹,

Toffanin²,

Gazzetta³

1980

Acta Haematol

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Two patients with factor VII Padua abnormality were studied during prophylactic administration of factor VII concentrates for tooth extractions. The amounts administered were 15.8 and 17.2 units/kg body weight in 30 and 20 min, respectively. The end infusion factor VII activity levels were 55 and 64%, respectively. Survival times of the exogenous components were 6.5 and 6 h, respectively. Satisfactory hemostasis was obtained in both instances.

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Section: Discussionsupporting

confidence: 90%

Factor VII Survival Studies in Factor VII Padua Abnormality

Girolami¹,

Toffanin²,

Gazzetta³

1980

Acta Haematol

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“…The lack of correc- tion of the prothrombin time after addition of the plasmas of 3 known cases of factor VII deficiency establishes the diagnosis unequivocally. The slight abnormality observed in the thromboplastin generation sys tem found in one of our cases is not surprising since this has already been seen in other proved factor VII deficient cases [6,21]. This could indicate that factor VII plays a minor role even in the thromboplastin generation.…”

Section: Discussionsupporting

confidence: 81%

“…The severity of bleeding is variable. Epistaxis, bleeding from the gums, easy bruising and menorrhagias are the commonest bleeding manifestations [21]. Hemarthroses are common but are often posttraumatic.…”

Section: Discussionmentioning

confidence: 99%

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Congenital Hypoproconvertinemia (Factor VII Deficiency)

Girolami¹,

Scorza²,

Brunetti³

et al. 1973

Acta Haematol

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Two new cases of congenital factor VII deficiency are reported. They are two 7-year-old boys belonging to two different kindreds. The main clinical features were: bleeding from the gums, epitaxis (one patient), easy bruising, melena, posttraumatic and spontaneous hemarthrosis. The main coagulation features were: prolonged prothrombin time, normal Stypvencephalin clotting time, normal first stage coagulation tests, normal platelet and vascular tests. The prothrombin time was corrected by the addition of normal serum, factor II or factor V or factor X-deficient plasmas and of the abnormal factor X (factor X Friuli) plasma. On the contrary, the addition of adsorbed normal plasma and of three plasmas with known factor VII deficiency failed to correct the abnormality. The factor VII levels were found to be 2.3 and 2.7% of normal, respectively. The parents of the patients were not consanguineous. But 3 of them, together with other members of the 2 families, were found to have slightly decreased factor VII levels and were considered to be heterozygotes for the defect. These findings are consistent with an incompletely recessive pattern of autosomal inheritance

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“…The final product of the cascade is the formation of a fibrin clot. 5,6 Several of these factors are part of the common pathway of coagulation and are determined both by PT and aPTT. Because factor VII activity is observed primarily in the extrinsic pathway, an isolated deficiency of this factor manifests as T Tsuda, Y Okamoto, R Sakaguchi et al…”

Section: Discussionmentioning

confidence: 99%

Isolated Factor VII Deficiency Diagnosed after a Life-Threatening Brain Haemorrhage

Tsuda¹,

Okamoto²,

Sakaguchi³

et al. 2000

J Int Med Res

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A 65-year-old man was admitted to another hospital with a life-threatening brain haemorrhage, and laboratory examinations on admission revealed prolonged prothrombin time with normal activated partial thromboplastin time. To establish the cause of his abnormal coagulation, he was referred to our clinic. Neither the patient nor his family had any previous history of bleeding symptoms. His liver function was within normal limits but coagulation tests showed increased plasma activities of factors II, VIII, IX, X, with reduced activities of factors V and VII. The activity of factor VII was less than 2% but no inhibitor of factor VII was detected in the plasma. We concluded that the patient had a rare congenital isolated factor VII deficiency although he had not shown earlier bleeding problems, presumably because of compensation for the factor VII deficiency by enhanced activities of components of the extrinsic coagulation pathway, factors II, VIII, IX and X.

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Congenital deficiency of factor VII (hypoconvertinemia)

Cited by 40 publications

References 95 publications

Factor VII Survival Studies in Factor VII Padua Abnormality

Factor VII Survival Studies in Factor VII Padua Abnormality

Congenital Hypoproconvertinemia (Factor VII Deficiency)

Isolated Factor VII Deficiency Diagnosed after a Life-Threatening Brain Haemorrhage

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