Primary prophylaxis for children with severe congenital factor VII deficiency - Clinical and laboratory assessment.

Severe congenital factor VII (FVII) deficiency is a rare bleeding disorder. Prophylaxis with replacement therapy has been suggested to patients, yet the most beneficial dosing regimens and therapy intervals are still to be defined. Due to the lack of evidence-based data, we hereby present our experience with long-term administration and monitoring primary prophylaxis in children with severe FVII deficiency and an extremely high bleeding risk. Four children with familial FVII deficiency, treated by prophylactic recombinant activated factor VII (rFVIIa), 15-30µg/kg/dose, given 2-3 times weekly since infancy, are discussed. Clinical follow up and monitoring laboratory assays, including thrombin generation, measured at various time points after prophylactic rFVIIa administration are presented. Among our treated patients neither FVII activity nor thrombin generation parameters (both already declined 24h post rFVIIa administration) were able to predict the impact of prophylaxis, and could not be used as surrogate markers in order to assess the most beneficial treatment frequency. However, the long clinical follow-up and comprehensive laboratory assessment performed, have shown that early primary prophylaxis as administered in our cohort was safe and effective.

Fatigue in medical residents--lessons to be learned.

BACKGROUND: Fatigue among medical residents is a well-known and widely discussed phenomenon that has generated much debate. OBJECTIVE: We wanted to evaluate self-reported fatigue and sleep deprivation, as well as some of the major consequences that have been identified in the period after the medical residents'strike in 2000. DESIGN: A cross sectional study. PARTICIPANTS: Seventy-six medical residents at Soroka university medical centre, who were asked to answer a questionnaire about their personal lives and fatigue level. DATA AND RESULTS: The average work-week was 68.1 +/- 12.4 hours. Residents reported 6.0 +/- 1.3 hours of sleep per night on a regular day and an average of 1.1 +/- 0.5 hours during a 24-hour on-call shift. The ESS score was 11.5 +/- 5.4 points. The number of hours worked per week correlated significantly with the ESS score. Of the residents who drove after a night shift, 29.3% reported falling asleep at least once while driving, and 13.9% of drivers reported that they were involved in a motor vehicle accident. CONCLUSIONS: In view of these results, we express a deep concern for the future of Israeli medical residents and their patients. We call upon the authorities to develop appropriate working conditions that will ensure the safety of patients and residents.