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151.
152.
Summary. During the haemostatic response, the formation of a primary platelet plug limits bleeding and provides a surface for clotting factors to assemble and become activated. The initial platelet plug is stabilized by fibrin monomers, covalently cross-linked by FXIII, forming a platelets-fibrin thrombus. Defects in platelets as well as inherited deficiencies of coagulation factors including fibrinogen, FII, FV, FV + FVIII, FVII, FX, FXI and FXIII deficiencies, generally lead to lifelong bleeding disorders, whose severity of bleeding symptoms is heterogeneous in platelets abnormalities but generally inversely proportional to the degree of the factor deficiency in rare bleeding disorders (RBDs). The prevalence of platelet defects among the general population has not been established, whereas for RBDs it ranges from approximately 1 in 2 million to 1 in 500 000, being higher in countries where consanguineous marriages are diffused. As a consequence of the rarity of these deficiencies, the type and severity of bleeding symptoms, the underlying molecular defects, and the actual management of bleeding episodes are not well established. In this review the main features, diagnosis, available treatment options and treatment complications of the platelet disorders, caused by abnormalities in platelet receptors for adhesive proteins, platelet receptors for soluble agonists, platelet granules, signal transduction pathways, or procoagulant phospholipids will be discussed by Dr Cattaneo, whereas fibrinogen deficiency and FXIII deficiency will be described by Dr Inbal and Dr de Moerloose, respectively. Finally, the update of the Rare Bleeding Disorders Database will be presented by Dr Spreafico. 相似文献
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154.
K. BEETON P. DE KLEIJN P. HILLIARD S. FUNK N. ZOURIKIAN B.-M. BERGSTROM R. H. H. ENGELBERT J. J. VAN DER NET M. J. MANCO-JOHNSON P. PETRINI M. VAN DEN BERG A. ABAD B. M. FELDMAN A. S. DORIA B. LUNDIN P. M. POONNOOSE J. A. JOHN M. L. KAVITHA S. M. PADANKATTI M. DEVADARASINI D. PAZANI A. SRIVASTAVA F. R. VAN GENDEREN R. VACHALATHITI 《Haemophilia》2006,12(S3):102-107
Summary. Assessment of impairment and function is essential in order to monitor joint status and evaluate therapeutic interventions in patients with haemophilia. The improvements in the treatment of haemophilia have required the development of more sensitive tools to detect the more minor dysfunctions that may now be apparent. This paper outlines some of the recent developments in this field. The Haemophilia Joint Health Score (HJHS) provides a systematic and robust measure of joint impairment. The MRI Scoring System has been designed to provide a comprehensive scoring system combining both progressive and additive scales. The Functional Independence Score for Haemophilia (FISH) has been developed to assess performance of functional activities and can be used in conjunction with the Haemophilia Activities List (HAL) which provides a self report measure of function. It is recommended that both measures are evaluated as these tools measure different constructs. Further refinement and testing of the psychometric properties of all of these tools is in progress. More widespread use of these tools will enable the sharing of data across the world so promoting best practice and ultimately enhancing patient care. 相似文献
155.
MORTALITY OF JAMAICAN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS 总被引:2,自引:0,他引:2
HARRIS E. N.; WILLIAMS E.; SHAH D. J.; DE CEULAER K. 《Rheumatology (Oxford, England)》1989,28(2):113-117
A retrospective study of all patients with systemic lupus erythematosus(SLE) who died at the University Hospital of the West Indiesover a 14-year period is presented. The major cause of deathwas infection followed by renal failure. Gram-negative organismswere the major microbiological agents causing infections. Side-effectsof therapy were common, in particular bone marrow depressionand haemorrhage related to anticoagulants. It appears that controllingsevere lupus activity without increasing the risk of life-threateningcomplications remains an important goal in the treatment ofSLE. KEY WORDS: Systemic lupus erythematosus, Mortality, Infection, Anticoagulants, Jamaica 相似文献
156.
Treatment of prosthetic tricuspid valve thrombosis with recombinant tissue-type plasminogen activator 总被引:1,自引:0,他引:1
CAMBIER P.; MOMBAERTS P.; DE GEEST H.; COLLEN D.; VAN DE WERF F. 《European heart journal》1987,8(7):906-909
Recombinant tissue-type plasminogen activator (rt-PA) was admministeredintravenously to a 64-years old female with thrombotic malfunctionof a BjörkShiley prosthetic tricuspid valve. 150mg of single-chain rt-PA was infused over 8 hours followed byan additional dose of 50 mg over the next 8 hours. At the endof the first infusion, restoration of normal valve functionwas demonstrated by fluoroscopic and echo-Doppler examinations.Mild systemic activation of the fibrinolytic system occurred,with a decrease of fibrinogen and 2-antiplasmin to 53% and 33%,respectively, of the preinfusion value at the nadir. 相似文献
157.
JACQUES M.T. DE BARKER Ph.D. RICHARD N.W. HAUER M.D. PATRICIA F.A. BAKKER M.D. ANTON E. BECKER M.D. MICHIEL J. JANSE M.D. ETIENNE O. ROBLES DE MEDINA M.D. 《Journal of cardiovascular electrophysiology》1994,5(4):335-344
Abnormal Automaticity in Human Atrium, introduction: A 32-year-old woman was operated upon because of drug refractory atrial tachycardia.
Methods and Results: Electrophysiologic study was performed prior to operation. During surgery, epicardial mapping of the electrical activity of the left atrium was performed. The left atrial appendage was resected and studied in a tissue bath. Thereafter, histologic examination was performed. Polarity of the P wave in the surface ECG suggested that the tachycardia originated high in the left atrium. Epicardial mapping disclosed earliest activation in the apex of the left atrial appendage. Intracellular recordings from surgical specimen made at the site of origin, which was marked during surgery, revealed cells with phase 4 depolarization at cycle lengths ranging from 360 to 540 msec. Exit block prevented spread of activation from the spontaneously firing cells to surrounding tissue. Histology showed that spontaneous activity arose in an area with abnormal cells-characterized by an amorphous, pale eosinophilic staining cytoplasm and absence of nuclei-surrounded by normal myocytes.
Conclusion: The observations indicate that the mechanism of the atrial tachycardia was based on abnormal automaticity in an area consisting of a conglomeration of normal and abnormal myocytes. 相似文献
Methods and Results: Electrophysiologic study was performed prior to operation. During surgery, epicardial mapping of the electrical activity of the left atrium was performed. The left atrial appendage was resected and studied in a tissue bath. Thereafter, histologic examination was performed. Polarity of the P wave in the surface ECG suggested that the tachycardia originated high in the left atrium. Epicardial mapping disclosed earliest activation in the apex of the left atrial appendage. Intracellular recordings from surgical specimen made at the site of origin, which was marked during surgery, revealed cells with phase 4 depolarization at cycle lengths ranging from 360 to 540 msec. Exit block prevented spread of activation from the spontaneously firing cells to surrounding tissue. Histology showed that spontaneous activity arose in an area with abnormal cells-characterized by an amorphous, pale eosinophilic staining cytoplasm and absence of nuclei-surrounded by normal myocytes.
Conclusion: The observations indicate that the mechanism of the atrial tachycardia was based on abnormal automaticity in an area consisting of a conglomeration of normal and abnormal myocytes. 相似文献
158.
Long‐term efficacy of a 0.07% cetylpyridinium chloride mouth rinse in relation to plaque and gingivitis: a 6‐month randomized,vehicle‐controlled clinical trial 下载免费PDF全文
159.
GIULIO CONTE M.D. CARLO DE ASMUNDIS M.D. Ph.D. JUAN SIEIRA M.D. MOISES LEVINSTEIN M.D. GIAN‐BATTISTA CHIERCHIA M.D. GIACOMO DI GIOVANNI M.D. GIANNIS BALTOGIANNIS M.D. GIUSEPPE CICONTE M.D. YUKIO SAITOH M.D. RUBEN CASADO‐ARROYO M.D. GUDRUN PAPPAERT R.N. PEDRO BRUGADA M.D. Ph.D. 《Journal of cardiovascular electrophysiology》2014,25(5):514-519
160.
Camila Santos PEREIRA Marcos Vinícius Macedo de OLIVEIRA Lucas Oliveira BARROS Gabriela Alencar BANDEIRA Sérgio Henrique Sousa SANTOS John R. BASILE André Luiz Sena GUIMAR?ES Alfredo Maurício Batista DE PAULA 《Journal of applied oral science : revista FOB》2013,21(5):416-421