Surgery in patients with haemophilia and high responding inhibitors: Izmir experience

Summary. This report evaluates the haemostatic efficacy of recombinant factor VIIa (rFVIIa) and activated prothrombin complex concentrate (APCC) in patients with haemophilia and high responding inhibitors who underwent major and minor surgery. Data pertaining to surgeries from 2001 to 2009 at a single centre were retrospectively analysed. During this period, 53 surgical procedures were performed in 30 haemophiliacs with high responding inhibitors. Mean age was 16.2 ± 9.4 years. Eleven major surgeries in 4 patients, 41 radioisotope synovectomies (RS) and one circumcision classified as minor surgery in 28 patients were performed. Among the major surgery procedures, four were treated with rFVIIa, five with APCC and two with sequential use of APCC and rFVIIa. We used rFVIIa at the dosage of 80–120 μg kg^?1 every 2 h and APCC 100 IU kg^?1 every 12 h for the major surgery. When performing RS, we used rFVIIa in 18 patients with 26 target joints and APCC in 9 patients with 15 target joints. Three consecutive doses of rFVIIa (90 μg kg^?1) were used at 2‐h intervals followed by additional three doses at 6‐h intervals. The initial dose of APCC was 75 IU kg^?1 followed by a second and third dose of 50 IU kg^?1 at 12‐h intervals. APCC and rFVIIa demonstrated excellent efficacy in our major and minor surgical interventions [100% (22/22) and 94% (31/33), respectively]. We had only two bleeding complications with rFVIIa. There were no thromboembolic complications. APCC and rFVIIa provide an effective and safe first line haemostatic therapy for inhibitor‐positive haemophiliacs, allowing both major and minor surgery to be successfully performed.     

Microparticle‐associated tissue factor activity,venous thromboembolism and mortality in pancreatic,gastric, colorectal and brain cancer patients

Summary. Background: Tissue factor (TF) expression by tumors contributes to tumor growth. Release of TF‐positive microparticles (MPs) may contribute to venous thromboembolism (VTE). Objectives: To conduct a prospective cohort study to determine whether elevated MP‐associated TF (MP‐TF) activity is predictive of VTE and mortality in four cancer types. Patients/Methods: We determined MP‐TF activity in pancreatic, gastric, colorectal and brain cancer patients. We used a chromogenic endpoint assay for all patients and also a chromogenic kinetic assay for patients with pancreatic and brain cancer. Results: During follow‐up, 12/60 (20%) pancreatic, 6/43 (14%) gastric, 12/126 (10%) colorectal and 19/119 (16%) brain cancer patients developed VTE; 46/60 (77%), 30/43 (70%), 47/126 (37%) and 67/119 (56%), respectively, died. MP‐TF activity levels were highest in pancreatic cancer. We did not find a statistically significant association of MP‐TF activity with the risk of VTE in any of the four cancer types by using two statistical methods. An association of MP‐TF activity with the risk of mortality was detected in pancreatic cancer with the endpoint assay (hazard ratio [HR] 1.8 and 95% confidence interval [CI] 1.4–2.3 per doubling of activity, P < 0.001) and the kinetic assay (HR 1.2, 95% CI 1.1–1.4, P < 0.001); adjustment for type of treatment was not performed. In pancreatic cancer, MP‐TF activity correlated with D‐dimer level (endpoint assay, r = 0.51; chromogenic assay, r = 0.48), and a correlation between assays (r = 0.61) was found. Conclusion: MP‐TF activity was not associated with future VTE in pancreatic, gastric, colorectal and brain cancer. However, we found a strong association of MP‐TF activity with mortality in pancreatic cancer. MP‐TF activity might be reflective of an aggressive pancreatic cancer phenotype.     

Patient resources in the therapeutic education of haemophiliacs in France: their skills and roles as defined by consensus of a working group

Summary. The activities of ‘expert patients’ or ‘patient tutors’, who help educate their peers, are gaining recognition in the health care system. This study investigates the role played by such patients in therapeutic education programmes organized by caregivers to validate the role of patients in implementing the therapeutic education of haemophilic patients and to define the skills required for such activities. This study employs the consensus methodology recommended by France’s National Authority for Health. The working group includes seven caregivers from Hemophiliac Treatment Centers (HTCs) and three patients from the French Association of Hemophiliacs (FAH). The role of patients in haemophilia education is recognized. Patients participating in the education of their peers are referred to as ‘patient resources’. A patient resource should be an adult, a volunteer and live in the same region as his peers. Candidates are chosen by the FAH and the HTCs to serve based on their motivation to facilitate the education of other patients as well as on their psychological and pedagogical aptitudes. A patient resource participates in the conception and administration of therapeutic education programmes. He also mediates between the caregivers and the patients. He ensures that the patients understand the material and are able to apply their knowledge in daily life. His activities are governed by professional ethics. Seven categories of skills were defined, permitting the group to determine precisely which skills are required to function as a patient resource. Supervision of the patients is planned to reinforce reflexive practices in the patients. Evolution of the health care system has led patients to become involved in therapeutic education. This phenomenon calls for a framework to be developed and an evaluation of its eventual effects.     

Post-COVID syndrome: pulmonary complications

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has infected millions of people worlwide and caused a pandemic that is still ongoing. The virus can cause a disease named as COVID-19, which is composed of multi systemic manifestations with a pulmonary system predominance. As the time passes, we are dealing more and more with a wide variety of effects and complications of the disease in survivors as far as with concerns about the clinical outcome and the timeline of symptoms in different patients. Since the lungs are the most involved organs and the post-COVID prolonged and persistent effects are mainly related to the pulmonary system, it is crucial to define and predict the outcome and to determine the individuals that can progress to fibrosis and loss of function of lungs. This review summarizes the current literature regarding the pulmonary complications in post-COVID syndrome and the management of these conditions.     

Measurement of family impact of skin disease: further validation of the Family Dermatology Life Quality Index (FDLQI)

Aim To adapt the Impact on Family scale (IOF), originally developed for families of children with chronic illnesses, for use in families of adult patients and to examine the convergent validity of the Family Dermatology Life Quality Index (FDLQI) by comparing it with the adapted version of the IOF scale. Methods Adult family members of patients with different skin diseases attending the out‐patients clinic of a secondary referral centre completed the FDLQI and the adapted version of the IOF scale, whereas patients completed the Dermatology Life Quality Index (DLQI). Results A total of 50 patients and 50 family members/partners participated in the study. The mean scores for the three instruments were as follows: FDLQI = 6.0 (SD = 6.4, median = 4); IOF scale = 27.9 (SD = 8.2, median = 29); DLQI = 5.4 (SD = 5.6, median = 3). There was no difference between male and female participants in the mean scores of any of the three instruments. The FDLQI demonstrated high internal consistency (Cronbach's α = 0.91). Factor analysis revealed the presence of one factor structure underlying the items of the FDLQI, which explained 55.8% of the total variance. The IOF scale also showed a high internal consistency (Cronbach's α = 0.89). The results of factor analysis revealed one dominant factor and three weaker factors, which together explained 72% of the total variance. The convergent validity of the FDLQI was demonstrated by a highly significant positive correlation between the mean FDLQI and the IOF scale scores (r_s = 0.61, P < 0.0001). All the items of the FDLQI also had significant correlations with the mean total IOF scale scores (P < 0.05). Conclusions The results of this study have demonstrated that with minor modifications IOF scale, which was originally developed for families of children suffering from chronic illnesses, can also be used in families of adult dermatology patients. The study has also provided further evidence of the validity of the newly developed FDLQI.     

THE ‘VASOVAGAL EFFECT’ OF NITRATES: AN UNDER-RECOGNISED COMPLICATION OF NITRATE USE

SUMMARY Nitrates are useful drugs in cardiovascular medicine. They have a presumed vasovagal effect, which is a rare but often under-recognised complication that is partly caused by central inhibition of the sympathetic nervous system. However, no consistent variable associated with the presumed vasovagal effects following nitrate administration can be identified, and there are only limited reports of the phenomenon in the literature. In fact, the true prevalence of such a complication is unknown, because no data from observational studies exist, although it would seem likely to be a rare event. A greater awareness and recognition of this unusual effect of nitrate use is necessary in view of the increasing use of nitrates. Fortunately, recovery is generally the rule with supportive management.