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Biomedical subjects

Y Sultan

Publications and source records attributed to Y Sultan.

At least 163 records · Page 9Linked to original sources
PubMed1976

Bernard-Soulier syndrome: a new platelet glycoprotein abnormality. Its relationship with platelet adhesion to subendothelium and with the factor VIII von Willebrand protein.

A decreased platelet adhesion to rabbit aorta subendothelium (Baumgartner technique) is confirmed in the Bernard Soulier (giant platelet) syndrome. Electron microscope techniques using a purified antibody against Factor VIII/von Willebrand protein, revealed an apparently normal presence of the Factor VIII/von Willebrand protein on the Bernard Soulier platelets. Electrophoretic characterization of the major protein and glycoprotein components of the Bernard Soulier platelets following sodium dodecyl sulfate solubilization indicated a relatively normal protein content but suggested a reduced content of the 155,000 molecular weight major platelet glycoprotein. This was confirmed by a reduced release of high molecular weight acidic glycopeptides following incubation of washed Bernard Soulier platelets with trypsin. It is proposed that this abnormality may be related to the previously reported reduced sialic acid content and the reduced electrophoretic mobility of the Bernard Soulier platelets and that a glycoprotein reduced or abnormal in the Bernard Soulier platelets is necessary for the normal adhesion of platelets to subendothelium.

Adult↗
PubMed1975

[Treatment of hemophilia with immunologic inhibitor of factor VIII by using "activated" coagulant fractions].

Bleeding episodes in hemophiliacs with inhibitor to factor VIII are often dramatic situations. High level inhibitors do not respond to massive amount of factor VIII and are often refractory to immunosuppressive drugs such as cyclophosphamide. In this situation it has been proposed to use "activated prothrombin complex" to control bleeding in hemophilic patients with inhibitors. A plasmatic fraction "Auto IX" has been used successfully for the treatment of an intestinal hemorrhage in a patient with an inhibitor to F. VIII. Possible mechanisms of action as well as potential thrombotic danger of such concentrate are discussed.

Adult↗
PubMed1975

Detection of heterozygotes in both parents of homozygous patients with Von Willebrand's disease.

Three patients with severe Von Willebrand's disease are shown to be homozygotes. They were born from unaffected parents. New techniques using a factor-VIII-related antigen assay by the Laurell method and a ristocetin-induced platelet aggregation assay demonstrated abnormalities in these two tests in both parents of the probands. Factor-VIII-related of heterogotes could not be differentiated from normal factor-VIII-related antigen by the immunodiffusion technique, crossed immunoelectrophoresis, and filtration on a sepharose 4b column.

Adolescent↗
PubMed1975

Studies of the human factor VIII/von Willebrand factor protein. III. Qualitative defects in von Willebrand's disease.

The Factor VIII/von Willebrand factor protein was characterized in two unrelated patients with von Willebrand's disease in whom procoagulant and Factor VIII/von Willebrand factor antigen levels were normal. In both patients evidence of an abnormal protein was observed on crossed antigen-antibody electrophoresis. In one patient the Factor VIII/von Willebrand factor protein eluted from Sepharose 4B in a position and distribution identical to normal with normal levels of procoagulant activity and antigen. However, the partially purified Factor VIII/von Willebrand factor protein had markedly reduced von Willebrand factor activity in a ristocetin assay. In the second patient the peak of Factor VIII/von Willebrand factor protein, antigen, and procoagulant activity eluted from a Sepharose 4B column with an estimated molecular weight of approximately half that of normal. This protein had no von Willebrand factor activity. In both patients the reduced Factor VIII/von Willebrand factor protein subunit was indistinguishable from normal on polyacrylamide gel electrophoresis. These studies indicate that in some patients with von Willebrand's disease there is a qualitative defect of the Factor VII/von Willebrand factor protein; the total amount of protein, antigen, and procoagulant activity are normal while the von Willebrand factor activity is deficient.

Antibodies↗
PubMed1975

The suppression of factor VIII antibodies in haemophilia.

Three severely affected haemophiliacs who had developed antibodies to factor VIII are described in whom the administration of cyclophosphamide was thought to have prevented reappearance of the antibody after factor VIII infusion. Some of the possible causes which might have led to the favourable outcome in these and three other, previously reported, patients in whom the antibody, which was raised at the time of IS therapy, dropped to zero and failed to reappear, are discussed. It is suggested that immunosuppressive therapy was effective in these patients as it was given during or soon after the primary immune response. As the antibody response becomes increasingly well established, so the chances of successful immunosuppressive therapy recede.

Antibody Formation↗
PubMed1975

[Medical treatment of hemophilic hemarthrosis and surgical prospects].

The treatment of acute hemarthrosis as utilized by eighteen hemophilic centers in different towns from France was presented. The amount of F.VIII infused, the use of cortisone and analgesic drugs, immobilisation and rehabilitation were discussed by each center. Discrepancies from one center to another appeared except for one point: the amount of F.VIII to be infused and the interest of early infusion. The interest of orthopedic surgery in the evolution of hemophilic arthropathies was developed.

Acute Disease↗