Detection and IgG subclass analysis of antibodies to factor VIII in multitransfused haemophiliacs and healthy individuals.

Using a binding assay to immobilized factor VIII (F VIII) (ELISA) we measured the amount of IgG with binding capacity to FVIII, in the plasma of patients with an inhibitor to F VIII, in multitransfused haemophiliacs without inhibitor and in a control group of blood donors. It was shown that the amount of IgG bound to VIII was elevated in patients with an inhibitor although a weak correlation could be established between the inhibitor titre (BU) and the amount of bound IgG. In all haemophiliacs without inhibitor, IgG bound to F VIII were present.

Biological effects of a S/D-treated, very high purity, von Willebrand factor concentrate in five patients with severe von Willebrand disease.

A highly purified von Willebrand factor (vWf) concentrate with low factor VIII (FVIII) content was tested to evaluate its biological effects in five patients with severe von Willebrand disease (vWd). The patients were infused with a single dose of this product corresponding to approximately 65 IU vWf:RCo and approximately 11 IU FVIII:C/kg and were followed up for 48 h. The plasma levels of FVIII and vWf and the multimeric pattern of vWf were determined before and 1, 3, 6, 12, 24 and 48 h after infusion.

Behçet syndrome associated with protein S deficiency.

Behçet syndrome is a multisystem disorder characterized by ocular, mucocutaneous, articular, gastrointestinal and neurologic abnormalities. We report here an unusual case of Behçet syndrome, characterized by the importance of the thrombotic events (7 phlebitis of both legs and resection of two toes). Additional manifestations of the Behçet syndrome occurred only 10 years after the first thrombotic episode.

Investigation of factor VIII:C gene restriction fragment length polymorphisms and search for deletions in hemophiliac subjects in Algeria.

The frequency of alleles for intragenic (intron 17 and intron 25) and extragenic (DXS15 and DXS52) F8C RFLPs was investigated in the Algerian population. Altogether 287 X chromosomes (97 males and 95 females) were studied. The allele frequencies found with the two intragenic F8C RFLPs were not substantially different from those reported in a Mediterranean population.

A new case of 'type II' inherited protein S deficiency.

Protein S inherited deficiency is associated with high risk of recurrent venous thrombotic disease (Broekmans et al, 1985a, b). Protein S exists as two forms in plasma, either free and functionally active or complexed with C4b-binding protein (C4b BP) and inactive (Dahlbäck & Stenflo, 1981). We report here the case of a 26-year-old woman and her brother, 28 years old, both suffering from recurrent venous thrombosis since the age of 20, diagnosed as severe protein S deficiency according to the following data: free protein S: 2.

[Platelet aggregation in diabetes mellitus in children].

Platelets-endothelial cells interaction appears to cause vascular damage in diabetes. Platelet aggregation was studied in 24 diabetic children and adolescents (mean age: 12.2 years, mean duration of diabetes: 3.

Antithrombin III Alger: a new homozygous AT III variant.

A qualitative abnormality of antithrombin III (AT III) was found in the plasma of a 41-year old patient. The plasmatic AT III antigen concentration was 130% and the progressive anti-F IIa and anti-F Xa activities were normal (105% and 137%). The plasma heparin cofactor activity was less than 10%, when measured by F IIa or F Xa inhibition.