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Recommendations for factor replacement therapy for postoperative haemostasis in haemophilia are often empirical and based on the physiological understanding of haemostatic requirements. This report describes the haemostatic management of patients with severe haemophilia undergoing major surgery using lower than usually recommended levels of factor replacement therapy. Eighteen adults (11 with FVIII and seven with FIX deficiency) with an average weight of 52 kg (range: 27-69) underwent 20 major surgical procedures. Factor concentrates were administered by intermittent bolus infusions. The dose of FVIII infused before surgery was 76 mu kg-1 (range: 51-113) and that of FIX was 77 mu kg-1 (range: 50-104). The preoperative levels achieved were 107% (range: 80-180) and 73% (range: 60-90), respectively. Between days 1 and 3 after surgery, an average of 29 mu kg-1 day-1 (range: 20-46) of FVIII and 23 mu kg-1 day-1 (range: 12-42) of FIX was used resulting in mean trough levels of 36% (range: 12-62) and 34% (range: 11-52), respectively. After day 4, an average of 19 mu kg-1 day-1 (range: 15-25) of FVIII and 18 mu kg-1 day-1 (range: 10-37) of FIX was administered until wound healing. The average duration of factor replacement was 11 days (range: 8-16). The mean dose of factor concentrate per patient was 260 mu kg-1 (range: 179-338) of FVIII and 300 mu kg-1 (range: 183-524) of FIX. The total amount of factor used per patient ranged from 12,380 to 19,980 units of FVIII and 8000 to 23,600 units of FIX. Only one patient had post-operative bleeding which was due to a surgical cause. It is concluded that it may be possible to use much lower than recommended levels of factor replacement therapy for postoperative haemostasis in severe haemophilia.

Objective In the era of organ shortage, redo liver transplantation (reLT) is frequently discussed in terms of expected poor outcome, high cost and therefore wasteful resources. However, there is a lack of benchmark data to reliably assess outcomes after reLT. The aim of this study was to define the ideal reLT case, and to establish clinically relevant benchmark values for best achievable outcome in reLT. Methods We collected data on reLT between January 2010 and December 2018 from 22 high volume transplant centers on three continents. Benchmark cases were defined as recipients with model of end-stage liver disease score <=25, absence of portal vein thrombosis, no mechanical ventilation before surgery, receiving a graft from a donor after brain death. In addition, early reLT including those for primary non-function (PNF) were excluded. Clinically relevant endpoints covering intra- and postoperative course were selected and complications were graded by severity using the Clavien-Dindo classification and the comprehensive complication index (CCI). The benchmark cutoff for each outcome was derived from the 75th percentile of the median values of all benchmark centers, indicating the “best achievable” result. To assess the utility of the newly established benchmark values, we analyzed patients who received reLT for PNF (non-benchmark patients). Results Out of 1110 reLT 413 (37.2%) qualified as benchmark cases. Benchmark values included: Length of intensive care unit and hospital stay: <=6 and <=24 days, respectively; Clavien-Dindo grade >=3a complications and the CCI at 1 year: <=76% and <=72.2, respectively; in-hospital and 1-year mortality rates: <=14.0% and <=14.3%, respectively. The cutoffs for transplant-specific complications such as biliary complications at 1 year, outflow problems at 1 year and hepatic artery thrombosis at discharge were <=27.3%, <=2.5% and <=4.8%, respectively. Patients receiving a reLT for PNF showed mean outcome values all outside the reLT benchmark values. In-hospital mortality rate was 34.4% and the mean CCI at discharge 68.8. Conclusion ReLT remains associated with high morbidity and mortality. The availability of benchmark values for outcome parameters of reLT may serve for comparison in any future analyses of individuals, patient groups, or centers, but also in the evaluation of new therapeutic strategies and principles.

This experiment suggests that butyrate has a significant role in increasing the mechanical strength of colonic anastomoses in rats.