Summary. A North American registry for rare bleeding disorders [factor (F)II, factor (F)VII, factor (F)X, factor (F)V, factor (F)XIII, fibrinogen deficiencies and dysfibrinogenemias] was established to gather information about disease prevalence, genotyping frequency, diagnostic events, clinical manifestations, treatment and prophylaxis strategies, as well as disease‐ and treatment‐related complications. Questionnaires were sent to 225 hemophilia treatment centers in the USA and Canada. Among 26% of responding centers, 294 individuals [4.4% of the registered children (200/4583) and 2.4% of adults (94/3809)] were diagnosed with one or more of the rare bleeding disorders (RBDs) included in this survey. The ethnic distribution for each disorder paralleled that of the general US population with the exception of the disproportionately large number of Latinos with FII deficiency. Only 5.4% of affected individuals were genotyped. An abnormal preoperative bleeding screen most often led to diagnosis. The most common coagulopathy was FVII deficiency; however, 40% of homozygous patients were asymptomatic. FX and FXIII deficiencies caused the most severe bleeding manifestations. Among all RBDs, the most common sites of bleeding were skin and mucus membranes. Multiple products were used to treat hemorrhage; however, half of the bleeding episodes required no therapy. The majority of patients suffered no long‐term complications from hemorrhage. Treatment‐related complications included viral seroconversion, anemia, allergic reactions and venous access device‐related events. This registry provides the most comprehensive information to date about North American individuals with RBDs and could serve as an important resource for both basic scientist and clinician.
This review highlights the clinical significance of coagulation and fibrinolytic responses, and adaptations in healthy individuals and patients with cardiovascular disease (CVD). Much of the review focuses on indicators of the potential for coagulation and fibrinolysis. The terms 'coagulation potential' and 'fibrinolytic potential' are used frequently, as much of the literature in the area of exercise haemostasis evaluates factors that reflect an increased potential for coagulation, while coagulation per se, may or may not be occurring. Similarly, fibrinolysis is definitively the lysis of inappropriate or excessive blood clot, which may or may not be occurring when the enzymes that stimulate fibrinolysis are activated. Nevertheless, markers of coagulation and fibrinolytic potential are associated with CVD, ischaemic events, and cardiovascular mortality. Additionally, fibrinolytic potential is associated with other established CVD risk factors. Ischaemic events triggered by physical exertion are more likely to occur due to an occlusive thrombus, suggesting the exercise-induced responses related to haemostasis are of clinical significance. The magnitude of increase in coagulation potential, platelet aggregation and fibrinolysis appears to be primarily determined by exercise intensity. Patients with CVD may also have a larger increase in coagulation potential during acute exercise than healthy individuals. Additionally, the magnitude of the fibrinolytic response is largely related to the resting fibrinolytic profile of the individual. In particular, high resting plasminogen activator inhibitor-1 may diminish the magnitude of tissue plasminogen activator response during acute exercise. Therefore, acute responses to exercise may increase the risk of ischaemic event. However, chronic aerobic exercise training may decrease coagulation potential and increase fibrinolytic potential in both healthy individuals and CVD patients. Due to the aforementioned importance of resting fibrinolysis on the fibrinolytic response to exercise, chronic aerobic exercise training may cause favourable adaptations that could contribute to decreased risk for ischaemic event, both at rest and during physical exertion.
There is a growing trend for runners to use compression stockings (CS) to improve performance. The purpose of this study was to determine the effect of CS on physiological variables associated with running performance. Participants were 10 NCAA division III cross-country runners. The study used a randomized, crossover design with 2 conditions (with CS and without CS). Both conditions consisted of a maximal treadmill test that involved 3-minute stages of increasing speed and incline, separated by a minute and one-half walking recovery stage. Seven days later, the participants repeated the maximal test but switched CS condition. Heart rate, blood lactate (BLa), blood lactate threshold, maximal oxygen consumption (VO2max), respiratory exchange ratio, rating of perceived exertion, and time to fatigue were measured. Before and during the maximal treadmill tests, the variables showed no significant difference (p ≤ 0.05) between the CS conditions. Blood lactate was lower while wearing CS when measured during recovery at the 1-minute (CS = 13.3 ± 2.9 mmol · L(-1), non-CS = 14.8 ± 2.8 mmol · L(-1), p = 0.03) and the 5-minute (CS = 11.0 ± 2.7 mmol · L(-1), non-CS = 12.8 ± 2.8 mmol · L(-1), p = 0.02) periods. Time to fatigue was longer without CS (CS = 23.570 ± 2.39 minutes, non-CS = 23.93 ± 2.49 minutes, p = 0.04). These findings suggest that CS may not improve running performance, but could lend credence to certain manufacturers' claims of improved recovery through lower BLa values after exercise.
Apply It! Gain a better understanding of exertional rhabdomyolysis (ER). Understand how ER can affect your clients/athletes, how to identify the signs and symptoms, and most importantly, learn how you can work to prevent it from developing.
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