[Haemophilia and dental procedures; a complex combination]. / Hemofilie en tandheelkundige behandelingen; een complexe combinatie.

A 28-year-old patient with severe haemophilia A presented to the emergency department with significant and painful swelling of the left cheek, an extensive haematoma extending from the left ear to the anterior thoracic region, an intraoral haematoma over the soft palate with deviation of the uvula to the right, and complaints of shortness of breath when lying down. Three days prior, his dentist had performed a restoration of the 36 molar under local anaesthesia. Due to pain, the general practitioner had administered an intramuscular injection of a non-steroidal anti-inflammatory drug (NSAID) two days post-procedure. The patient was admitted for treatment with coagulation factors and pain management. Dental procedures and local anaesthesia in patients with a severe coagulation disorder require specific preparatory measures, such as administration of coagulation factors. Collaboration and consultation with a patient's haematologist or haemophilia treatment centre are essential requirements for safe dental care.

E-learning improves knowledge and practical skills in haemophilia patients on home treatment: a randomized controlled trial.

Home treatment of haemophilia is currently the standard of care for patients with severe haemophilia. Home treatment increases the responsibility of the patients for their own treatment and care. Therefore, it is of utmost importance to attain a high level of knowledge and practical skills. The aim of our study was to investigate whether or not an educational e-learning program improves knowledge and skills of adult patients with haemophilia on home treatment. Participants treated at the Haemophilia Treatment Center of the Erasmus University Medical Centre completed a questionnaire to test their knowledge of haemophilia, treatment of bleedings and of complications of treatment and were observed during the intravenous injection of clotting factor concentrate, using a standardized scoring list. Afterwards they were randomized to follow an e-learning program or no intervention (control group). After 1 month they completed the same questionnaire again and practical skills were scored once more. At baseline, haemophilia patients (n = 30) scored 24 of 48 questions in the questionnaire correctly. Seventy-five per cent of the items on the practical skills scoring list were performed correctly. One month later, the e-learning group (n = 16; 36; 18-45) showed a higher level of theoretical knowledge compared to the control group (n = 14; 26; 19-32; P < 0.001). Also practical skills were significantly better in the group that followed the e-learning program compared to the control group (respectively P = 0.002). Self-efficacy of 90% vs. 80% the patients with haemophilia was high in all patients. Our study shows that in haemophilia patients with haemophilia, who are on home treatment, knowledge of haemophilia treatment and complications as well as practical skills can be improved by an educational e-learning program.

Stress sensitization following a disaster: a prospective study.

BACKGROUND: According to the stress sensitization hypothesis, prior exposure to extreme stressors may lead to increased responsiveness to subsequent stressors. It is unclear whether disaster exposure is associated with stress sensitization and, if so, whether this effect is lasting or temporary. This study aimed to investigate the occurrence and duration of stress sensitization prospectively following a major disaster. METHOD: Residents affected by a fireworks disaster (n=1083) participated in surveys 2-3 weeks (T1), 18-20 months (T2) and almost 4 years (T3) after the disaster. Participants reported disaster exposure, including direct exposure, injury and damage to their home at T1, and also stressful life events (SLEs) at T2 and T3. Feelings of anxiety and depression, concentration difficulty, hostility, sleep disturbance, and intrusion and avoidance of disaster-related memories were used as indicators of distress. RESULTS: Residents whose home was completely destroyed responded with greater distress to SLEs reported 18-20 months following the disaster than residents whose home was less damaged. There were no differences in stress responsiveness almost 4 years after the disaster. CONCLUSIONS: During the first years after a disaster, stress sensitization may occur in disaster survivors who experienced extreme disaster exposure. Stress sensitization may explain the persistence or progression of distress over time following extreme stressor exposure.

Genetic analysis of autonomic reactivity to psychologically stressful situations.

We present the results of a behavioural genetics study on response profiles of autonomic measures (heart rate, blood pressure, and galvanic skin level), under ecologically valid, stressful conditions. Where response profiles of different physiological variables are the object of study, and when daily life stressors are taken into account (Turner and Hewitt, Annals of Behavioral Medicine, 14 (1992) 12-20), autonomic responsiveness to psychological stressors is thought to be an inherited trait. The participants were 100 female twin pairs, 57 monozygotic and 43 dizygotic twin pairs. Participants watched eight films with a stressful social content while autonomic measures were continuously recorded. Results show that the heritability coefficients of response profiles of autonomic measures are almost twice as high as that of single variables. The results further show that genes exert their influence on physiological behaviour not only directly, but also indirectly, by influencing the idiosyncratic relation between a person and his environment.

4-Methylthiobenzoic acid reduces cisplatin nephrotoxicity in rats without compromising anti-tumour activity.

Administration of 4-methylthiobenzoic acid (MTBA) (100 mg/kg) strongly reduced cisplatin nephrotoxicity (7.5 mg/kg, 20 min after MTBA) in rats as determined by histopathology and blood urea nitrogen. Anti-tumour activity against a colonic adenocarcinoma, CC 531, that was implanted in rats, was unaffected by MTBA pretreatment. Studies with isolated renal proximal tubular cells (PTC) demonstrated that preincubation of the cells with MTBA diminished cisplatin nephrotoxicity in vitro as it did in vivo. Preincubation of the PTC with probenecid completely abolished the protective effect of MTBA against cisplatin toxicity. These data indicate that MTBA is actively transported into the PTC. The mechanism of action of MTBA was investigated by NMR studies which showed that cisplatin and cis-diamminediaquaplatinum(II), its hydrolysis product, reacted with the methylthio-sulphur. We suggest that MTBA after selective accumulation in the kidney inactivates cisplatin intracellularly by nucleophilic attack of the methylthio-sulphur to the Pt-moiety. Since MTBA shows no acute toxicity in the rat, even if administered at very high doses, it may be useful to suppress the nephrotoxic side effects of cisplatin anti-tumour therapy.