Continuous infusion of recombinant factor VIII formulated with sucrose in surgery: non-interventional, observational study in patients with severe haemophilia A.

In haemophilia A, continuous infusion (CI) of FVIII perioperatively provides a more constant FVIII level than conventional bolus injections, avoiding low trough levels that could increase bleeding risk. Due to the low number of surgical cases in clinical trials, especially in haemophilia, more information on the clinical practice of CI from observational studies is helpful. We aimed to evaluate the effectiveness and safety of CI with recombinant factor VIII formulated with sucrose (rFVIII-FS) in a typical surgery practice setting. This was a non-interventional study in 12 centres. Patients with severe haemophilia A who received rFVIII-FS by CI during and after surgery were included in this study if they had more than 150 exposure days (EDs) to any FVIII product and had no history of inhibitors before CI. Patients were observed during the entire course of CI, with monitoring up to 3 months thereafter. Twenty-five patients with 28 surgeries were included in the analysis. Median age was 51.7 (range 10-75). Most (75%; 21/25) patients underwent orthopaedic surgeries. The median dose of rFVIII-FS consumed during CI was 376 IU kg(-1) (range 157.9-3605.6 IU kg(-1)) with a greater median dose for orthopaedic surgeries (424.0 IU kg(-1)) compared to non-orthopaedic surgeries (278.5 IU kg(-1)). 95% of all FVIII measurements (214/224) were on target. Efficacy and tolerability were rated as good/excellent in 89.3% (25/28) of surgeries. No inhibitors were observed during or after surgery. This study demonstrates the effectiveness of CI with rFVIII-FS during surgery in patients with severe haemophilia A in a clinical practice setting.

[Management of pregnancy and childbirth in carriers of haemophilia]. / Begeleiding van de zwangerschap en de partus bij draagsters van hemofilie.

3 pregnant women, aged 27, 33 and 31 years respectively, were carriers of haemophilia A. The first patient had a caesarean section without prior measurement or substitution of factor VIII. She gave birth to a healthy boy, but developed severe diffuse abdominal bleeding after a few hours. The second patient had a normal level of factor VIII, and lived 100 km away from the nearest haemophilia treatment centre. Ultrasound investigation revealed a female foetus. She gave birth in the local hospital. The third patient was pregnant with a male foetus, but refused further prenatal investigation. Contrary to medical advice she gave birth at home. For carriers of haemophilia, there are several options for prenatal diagnosis and managing labour and delivery. Early referral is advised and the need for adequate counselling is explained. It is important to have an experienced haemophilia treatment centre nearby, where haematologists, gynaecologists, geneticists and paediatricians cooperate in caring for pregnant carriers of haemophilia.

Recurrent severe bleeding from gastrointestinal angiodysplasia in a patient with von Willebrand's disease, controlled with recombinant factor VIIa.

A patient with von Willebrand's disease had recurrent gastrointestinal bleeding from angiodysplasia, with inadequate response to von Willebrand factor substitution, medical and endoscopic treatment, and resection of affected bowel. Frequent blood transfusions were required. She started home treatment with recombinant activated factor VII (rFVIIa) at the onset of bleeding, in addition to her standard therapy. From then on, bleeds could be controlled rapidly and no more blood transfusions were needed. We conclude that rFVIIa is effective in this case of angiodysplasia and might be a therapeutic option in similar patients.

Infection control in patients undergoing mechanical ventilation: traditional approach versus a new development--selective decontamination of the digestive tract.

Nosocomial infections are major causes of morbidity and mortality in critically ill patients. Traditional infection control practices focus on preventing infection by controlling patient exposure to microorganisms within the patient's environment. We discuss these practices, along with the factors and organisms responsible for nosocomial infection in the patient undergoing mechanical ventilation. Despite traditional infection control measures, nosocomial infection continues to be a major problem. In recent years evidence has accumulated that points to bacterial colonization of the oropharynx and gastrointestinal tract as a major source of infection. A new technique, selective decontamination of the digestive tract, is being studied extensively for its ability to control colonization of the oral cavity and the gastrointestinal tract. In the technique nonabsorbable topical antibiotics are applied to the oropharynx and instilled into the stomach, and a short course of an intravenous cephalosporin is included. The technique appears a worthwhile addition to traditional infection control measures.