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Biomedical subjects

M T Canciani

Publications and source records attributed to M T Canciani.

22 records · Page 2Linked to original sources
PubMed1981

Response of factor VIII/von Willebrand factor to DDAVP in healthy subjects and patients with haemophilia A and von Willebrand's disease.

These studies were designed with the purpose of providing clinico-pharmacological information relevant to the use of DDAVP in the management of mild haemophilia and von Willebrand's disease (VWD). In healthy subjects, intravenous DDAVP produced its maximal response at a dose of 0.3 micrograms/kg. The extent of the increase in factor VIII coagulant activity (VIII:C) and factor VIII related antigen (VIIIR:Ag) induced by this dose was not significantly different from that observed with the same dose in haemophiliacs and VWD patients. In these, the bleeding time was not shortened. DDAVP given intranasally was followed by a two-fold increase of VIII:C. This route of administration might be adopted to provide an emergency aid in bleeding patients and to yield higher VIII:C levels in blood donors. In healthy subjects, the half-disappearance time of autologous VIII:C after increase induced by i.v. DDAVP is similar to that observed in patients with VWD treated in the same conditions, whereas the response appears to be more prolonged in haemophiliacs. This study shows that the consistency of the VIII:C response tends to decrease when repeated doses are given to healthy subjects. Repeatedly-treated haemophiliacs and VWD patients showed varied patterns, ranging from no change of the response to its early abolishment.

Adult↗
PubMed1979

The varied sensitivity of partial thromboplastin and prothrombin time reagents in the demonstration of the lupus-like anticoagulant.

An acquired inhibitor of blood coagulation, similar to that described in patients with Systemic Lupus Erythematosus (SLE), was detected during routine coagulation screening in 10 patients who did not meet the criteria for a diagnosis of SLE. The lupus-like anticoagulant (LLAC) was diagnosed on the basis of prolonged activated partial thromboplastin time (APTT) and/or prothrombin time (PT) which failed to correct when patient plasma was added to normal plasma; an additional criterion was an abnormal tissue thromboplastin inhibition test. No patient had a specific inhibitor directed against factors VIII and IX. Demonstration of LLAC was highly dependent upon the type of reagents adopted in the APTT and PT: the abnormality was detected consistently by one reagent only. One-stage assays of factors VIII and IX were characteristic of the presence of an inhibitor, showing non-parellel dose-response curves or decreased activity at low dilutions which were partially corrected at higher dilutions. Although 7 patients were free of abnormal bleeding, unequivocal signs of haemorrhagic tendency after a surgery were present in the remaining 3 patients. The findings suggest that LLAC is a non-exceptional cause of prolonged coagulation screening tests, and that it may sometimes be associated with impaired haemostasis.

Adolescent↗
PubMedDate not supplied

Inherited deficiency of antithrombin III in an Italian kindred.

Life-long symptoms of venous thromboembolism were present throughout five generations in a large Italian kindred. Eight out of the 22 tested members of the family had reduced plasma levels of antithrombin III (AT III), the main physiological inhibitor of blood coagulation. Three of them, aged 2-28 years, but no thrombotic manifestations. AT III deficiency, which was hitherto thought to be very rare in this country, appeared to be related to a reduced synthesis of the protein without any qualitative abnormality. This view is supported by the concomitant reduction of AT III levels as demonstrated by biological and immunological assays, as well as by a normal pattern of the protein in double-crossed immunoelectrophoresis with and without heparin added to the agarose gel. Long-term treatment with oral anticoagulants appeared to prevent further thromboembolic episodes in two patients, even though plasma AT III levels remained unchanged.

Adolescent↗