Acquired haemophilia is a rare bleeding disorder caused by autoimmune antibodies interacting with factor VIII (FVIII) or factor IX. Anticipating a high degree of heterogeneity amongst cases, we recently initiated systematic recording of whole blood (WB) coagulation dynamic profiles using our recently developed thrombelastographic method employing very small amounts of tissue factor for activation. Six newly diagnosed patients with acquired haemophilia A in our University Hospital were investigated with the purpose to characterize the WB clotting phenotypes in each patient, as well as inspecting the ex vivo and in vivo response to supplementation with various haemostatic agents. Our results show a striking heterogeneity in patients WB clotting profiles, each patient having a particular pattern and an individual type of response to bypassing agents. Profiles in some of patients resembled severe haemophilia A, even if there was a measurable residual FVIII:C activity while others were more similar moderate-to-mild haemophilia. In one case the profile was very close to normal. Each patient seemed to respond individually to bypassing agents. WB clotting profiles assisted us in selecting an optimal treatment modality in each case and whenever possible, we compared the clinical effects of the treatment selected with the appearance of the WB clotting pattern. In one patient, the ex vivo response to FVIII looked promising, and a approximately 200 IU kg-1 per 24 h high-dose programme nearly normalized the clotting profile in 2-week time. Our preliminary small series of data should be concluded with caution. However, it seems that WB clotting profile studies at baseline, with ex vivo addition of haemostasis promoting agents, and during treatment may hold the potential to predict the success of treatment.

We describe the fatal course of a patient with initial symptoms of vomiting and nausea who developed symptoms of dystonia, encephalopathy, and coma. The cause of death was poisoning with 3-nitropropionic acid from coconut water spoiled with the fungus Arthrinium saccharicola . We present the clinical findings and forensic analysis.

Aims/hypothesis Hypertriacylglycerolaemia is a hallmark of diabetic dyslipidaemia with increased concentrations of triacylglycerol (TG)-rich VLDL 1 particles. However, whether VLDL 1 secretion or removal is abnormal in type 2 diabetes remains unclear. The aim of this study was to compare basal and insulin-mediated VLDL 1 -and VLDL 2 -TG kinetics in men with type 2 diabetes and healthy men using a novel direct VLDL 1 -and VLDL 2 -TG labelling method. Methods Twelve men with type 2 diabetes and 12 healthy men matched for age and BMI were recruited. VLDL 1 -and VLDL 2 -TG turnover were measured during a 4 h basal period and a 3.5 h hyperinsulinaemic clamp period using a primed-constant infusion of ex vivo labelled VLDL 1 -TG and VLDL 2 -TG. Results Basal VLDL 1 -TG and VLDL 2 -TG secretion rates were similar in men with diabetes and healthy men. During hyperinsulinaemia, VLDL 1 -TG secretion rates were suppressed significantly in both groups, whereas no suppression of VLDL 2 -TG secretion rate was observed. VLDL 1 -TG to VLDL 2 -TG transfer rate was not significantly different from zero in both groups, while VLDL 1 -TG fatty acid oxidation rate was substantial, with a contribution to total energy expenditure of approximately 15% during postabsorptive conditions. VLDL 1 and VLDL 2 particle size (TG/apolipoprotein B [apoB] ratio) and apoB-100 concentration were unaltered by hyperinsulinaemia in men with type 2 diabetes, but significantly reduced in healthy men. Conclusions/interpretation Insulin inhibits VLDL 1 -TG secretion rate similarly in age-and BMI-matched men with type 2 diabetes and healthy men, while VLDL 2 -TG secretion is unaltered by hyperinsulinaemia. However, VLDL 1 -and VLDL 2 -apoB levels are not lowered by hyperinsulinaemia in men with type 2 diabetes, which is indicative of a diminished hepatic response to insulin. Trial registration ClinicalTrials.gov NCT01564550

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