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Fibrinolytic study in a homozygous protein C deficient patient
Authors:J Aznar  A Dasi  F Espa?a  A Estellés
Institution:1. Department of Clinical Pathology, Valencia, Spain;2. Department of Pediatrics, Valencia, Spain;3. Research Centre. Hospital “La Fe” 46009 Valencia, Spain;1. Institute of Sound and Vibration Research, University of Southampton, Southampton, SO17 1BJ, UK;2. School of Urban Railway Transportation, Shanghai University of Engineering Science, Shanghai, 201620, China;1. College of Chemistry & Chemical Engineering, Shaoxing University, Shaoxing 312000, PR China;2. Mathematic Information College, Shaoxing University, Shaoxing 312000, PR China;3. School of Chemistry Biology and Material Engineering, Suzhou University of Science and Technology, Suzhou 215009, PR China;1. Severance Biomedical Science Institute, Graduate School of Medical Science, Brain Korea 21 Project, Yonsei University College of Medicine, Republic of Korea;2. Severance Biomedical Science Institute, Yonsei Biomedical Research Institute, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea;1. Cell Biology, Department of Biology, Faculty of Science, Utrecht University, Padualaan 8, Utrecht, CH 3584 The Netherlands
Abstract:The fibrinolytic system was evaluated in a patient with homozygous protein C deficiency as well as in several members of his family with a partial deficiency of this protein. Before anticoagulant therapy the patient showed skin lesions which quickly disappeared after administration of fresh plasma. After anticoagulant treatment, the propositus suffered two clinical episodes of "ecchymotic" lesions, which were controlled with fresh plasma. The patient has remained free of new lesions and other clinical episodes up to the present date. The fibrinolytic activity of both the propositus and his family was normal. The patient's father showed adequate release of tissue plasminogen activator after controlled physical exercise. According to clinical and analytical data from our patient and his family, it is suggested that, in spite of the preservation of the fibrinolytic system in this case, a localized deficiency in fibrinolysis could exist in view of the clinical behaviour of the skin lesions described.
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