Multimerin is a massive soluble, multimeric protein found in platelets and endothelial cells. Recent studies identified multimerin as a specific coagulation factor V binding protein, complexed with platelet, but not plasma, factor V. These findings led us to investigate individuals with inherited factor V deficiencies for possible multimerin abnormalities. Platelet proteins were evaluated using immunoassays, sodium dodecyl sulfate-polyacrylamide gel electrophoresis, immunoblotting, immunoprecipitation, and direct binding studies. Patients with factor V Quebec, a disorder with abnormal platelet factor V, had a quantitative deficiency in multimerin (n = 11 tested; mean, 12.5%; range, 5% to 27% of the normal pool; normal range, 45% to 214%) with a normal multimer pattern. Quantitative and qualitative abnormalities were detected in their platelet factor V. An unrelated patient who was deficient in platelet and plasma factor V had normal platelet multimerin. The levels of platelet beta- thromboglobulin, von Willebrand factor, thrombospondin, and fibrinogen antigen were normal in the factor V Quebec patients. However, proteins with abnormal mobility were detected in their platelet lysate and releasate, and their platelet thrombospondin, von Willebrand factor, and fibrinogen showed evidence of proteolytic degradation. Platelet counts of the factor V Quebec patients ranged from mildly thrombocytopenic to low normal (mean, 159 x 10(9)/L; range, 104 to 198 x 10(9)/L). In addition, their platelets failed to aggregate in response to 6 to 10 micromol/L epinephrine despite normal numbers of platelet alpha 2-adrenergic receptors. These data indicate that patients with factor V Quebec have an inherited bleeding disorder distinct from other platelet disorders and associated with multiple abnormalities, including multimerin deficiency, abnormal platelet factor V, thrombospondin, von Willebrand factor, and fibrinogen, and an epinephrine aggregation defect.
Effective communication is central to a successful physician-patient relationship. Communication is usually enhanced when the linguistic and cultural attributes of patients are incorporated in health care delivery. With this purpose in mind, the Faculty of Medicine at the University of Ottawa has developed a French-language stream to train future physicians for the francophone minority population of Ontario. As part of this project, a communication skills laboratory was created for francophone students in 1996, since all three tertiary care teaching hospitals operated in English only. The laboratory consists of a controlled environment where francophone students conduct interviews in French while being observed by clinicians trained in observation and feedback techniques. It makes use of simulated patients trained to play specific roles and to give feedback to students. Laboratory sessions take place throughout the first and second years and expose students to 15 scenarios covering different themes in each year. Each scenario includes a communication problem. Facilities are in place for filming the encounters for review by students. The project has had favorable outcomes. Both students and clinician-supervisors find that the laboratory offers an excellent learning environment and describe the cases as realistic and instructive. Clerkship preceptors are pleased with the students' communication skills. Because of the success of the laboratory, faculty authorities plan to translate the scenarios and offer similar sessions to students in the English-language stream. The teaching methods used in the communication skills laboratory may be of interest to other medical schools that serve linguistic minority populations.
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