We examined PFA-100® results in a large paediatric patient population diagnosed specifically with δ-PSPD, and determined the relationship between PFA-100®

and platelet electron microscopy (the gold standard for diagnosis). This study is a retrospective review of patients <19 years of age diagnosed with δ-PSPD at Nationwide Children’s Hospital from 2008 to 2010. To examine the correlation between PFA-100® and average number of granules per platelet we used Spearman’s Rho as a non-parametric measure of dependence. A total of 105 patients diagnosed with δ-PSPD were included, of which 99 patients underwent PFA-100® testing. Of those tested 46% had at least one abnormal closure time, whereas 16% had abnormal results for both cartridges. We found no statistical correlation between C-EPI closure time and average number PD-0332991 mouse of granules per platelet (ρ= −0.0095, P-value = 0.9328), nor between C-ADP closure time and the average number of granules (ρ = 0.0315, P-value = 0.7798). The PFA-100®, a widely used screening test for suspected bleeding disorders, did not correlate with presence or severity of δ-PSPD as determined by platelet electron microscopy. When evaluating patients with suspected bleeding disorders, PFA-100® alone cannot be used to rule out the presence of a δ-PSPD. “
“Summary.  There is a potential for significant paradigm shift in

the assessment of haemostasis from the conventional AZD5363 cell line plasma recalcification times, such as prothrombin time (PT) and activated partial thromboplastin time (APTT), which correspond to artificially created compartments of haemostasis to tests that assess the entire process in a more physiological and holistic manner. These include the thrombin generation test, thromboelastogram and the clot wave form analysis. While these tests have been described many years ago, there is renewed interest in their use with modified technology for assessing normal haemostasis and its disorders. Although early data suggest that they can provide much greater information

regarding the overall haemostasis process and its disorders, many challenges remain. Some of them are possible only on instruments that are proprietary technology, expensive and are not widely available. 上海皓元 Furthermore, these tests need to be standardized with regard to their reagents, methodology and interpretation, and finally, much more data need to be collected regarding clinical correlations with the parameters measured. Haemostasis and its abnormalities have been traditionally assessed by plasma clotting times, such as the prothrombin, activated partial thromboplastin and thrombin times[1]. These times depend on the thrombin dependent conversion of fibrinogen to fibrin, but note only the initiation of this process and not its speed or total extent. Factor assays based on these tests have defined the different coagulation disorders including haemophilia[2,3].