Abnormal Factor X (Factor X Friuli) Coagulation Disorder

Self Cite

In two patients with the abnormal factor X (factor X Friuli) coagulation disorder a multiple tooth extraction was carried out immediately after the transfusion. X of 4 U of factor X concentrate (Bebulin) equivalent to 2,000 ml of normal plasma. No bleeding was noted. The half-life of the exogeneous component was 30 and 28 h, respectively. Two other patients with this peculiar disorder were treated with repeated whole blood, plasma and factor X concentrates for hematuria and posttraumatic fracture of the left zygomatic bone. Factor X levels of 45–50% are hemostatically adequate for tooth extractions, the control of spontaneous hematuria and posttraumatic hematomata. The management of these patients seems similar to that used for patients with classical factor X deficiency.

mentioning

confidence: 99%

Factor X Survival and Therapeutic Factor X Levels in the Abnormal Factor X (Factor X Friuli) Coagulation Disorder

Girolami¹,

Molaro²,

Marco

1974

Self Cite

“…Our patient with classical factor X deficiency had a factor X level of less than 0.1% and no detectable factor X protein was present in immu nological assays. On the contrary, the patient with factor X Friuli abnor mality had a factor X antigen of about 110% of normal and about 7% factor X activity using a tissue whole or partial thromboplastin [5,7]. It may, therefore, be concluded that neither factor X activity nor factor X antigen are necessary for platelet aggregation and adhesiveness.…”

Section: Discussionmentioning

confidence: 93%

“…Factor X deficient plasma was obtained from a patient pre viously reported [8]. Abnormal factor X (factor X Friuli) plasma was obtained from a patient previously reported [5]. The main features of these two patients are sum marized in table I.…”

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

Platelet Aggregation and Adhesiveness in Classical Factor X Deficiency and in the Abnormal Factor X (Factor X Friuli) Coagulation Disorder

Girolami¹,

Fabris²,

Peruffo³

et al. 1976

Platelet aggregation to common inductors and to Ristocetin, Thrombo-fax and Ionophore is normal in congenital factor X deficiency and in factor X Friuli coagulation disorder. Washed normal platelets resuspended in the patient’s plasma and in adsorbed normal plasma showed a normal aggregation. On the contrary, normal platelets resuspended in normal serum failed to aggregate. These studies indicate that factor X plays no role in normal platelet aggregation.

“…So far 11 homozygote patients and several heterozygotes have been reported [10,21,24]. The condition is transmitted as an au tosomal incompletely recessive trait [10,18,21,24], The main feature of the defect consists in the presence of an abnormal factor X which may not or may be activated only very slowly by whole or partial tissue thromboplastin whereas it may still be normally activated by Russell's Viper venom [7][8][9]16]. It was also demonstrated that prothrombin is normal in Friuli patients both as activity and as antigen [13] and that no inhibitor is present [17,22], Immunologically, factor X Friuli behaves as normal factor X both in plasma and is different from coumarin-induced abnormal factor X [15,19,23],…”

mentioning

confidence: 99%

Factor X Friuli Coagulation Disorder

Girolami¹,

Molaro²,

Falomo³

1975

Self Cite

The up-dated case history of the index patient with the factor X Friuli coagulation disorder is reported. The patient, aged 72, died of irreversible shock after progressive jaundice with symptoms of renal, cardiac and hepatic failure. The autopsy showed carcinoma of the head of the pancreas, diffuse petecchial hemorrhages, massive adrenal gland hemorrhage and minimal athero-arteriosclerotic lesions of all arteries. The significance of minimal athero-arteriosclerotic changes in a patient with a congenital coagulation defect is discussed.