Factor replacement therapy for the treatment of moderate to severe haemophilia A and B can be complicated by the production of inhibitory alloantibodies to factor VIII (FVIII) or factor IX. Treatment with the nanofiltered anti-inhibitor coagulant complex, Factor Eight Inhibitor Bypassing Activity (FEIBA NF), is a key therapeutic option for controlling acute haemorrhages in patients with high-titre inhibitors or low-titre inhibitors refractory to replacement therapy. Given the high risk for morbidity and mortality in haemophilia patients with inhibitors to FVIII or FIX, we conducted this Phase 3 prospective study to evaluate whether prophylaxis with FEIBA NF is a safe and effective treatment option. Over a 1-year period, 17 subjects were treated prophylactically (85 ± 15 U kg−1 every other day) while 19 subjects were treated on demand. The median (IQR) annualized bleeding rate (ABR) during prophylaxis was 7.9 (8.1), compared to 28.7 (32.3) during on-demand treatment, which amounts to a 72.5% reduction and a statistically significant difference in ABRs between arms (P = 0.0003). Three (17.6%) subjects (ITT) on prophylaxis experienced no bleeding episodes, whereas none treated on demand were bleeding episode-free. Total utilization of FEIBA NF for the treatment of bleeding episodes was significantly higher during on-demand therapy than prophylaxis (P = 0.0067). There were no differences in the rates of related adverse events between arms. This study demonstrates that FEIBA prophylaxis significantly reduces all types of bleeding compared with on-demand treatment, and the safety of prophylaxis is comparable to that of on-demand treatment.

Intracranial haemorrhage (ICH) is a common cause of morbidity and mortality in haemophilic patients. The overall incidence of ICH has been reported to range from 2.2% to 7.5% in patients with haemophilia. From 1987 to 2001, 401 haemophilic patients from the Serviço de Hemofilia, Disciplina de Hematologia e Hemoterapia, Universidade Federal de São Paulo were evaluated. The episodes of ICH were documented by CT scan and the anatomic location, clinical presentation, relationship to trauma and clinical factors, including the presence of HIV infection and the presence of inhibitor, were reviewed. Among 401 haemophilic patients, 45 ICH episodes in 35 (8.7%) patients with age ranging from 4 days to 49 years (mean 10.6 years) were observed. A history of recent trauma was documented in 24 (53.3%) cases. Seventeen (37.8%) episodes occurred in more than one site of bleeding, 12 (26.7%) were subdural, seven (15.5%) subarachnoid, four (8.9%) epidural, two (4.4%) intracerebral and one (2.2%) intraventricular. The most frequent symptoms were headache and drowsiness. All patients were submitted to replacement therapy and neurosurgical intervention was performed in eight (17.8%) patients. Despite the treatment, three (8.6%) haemophilia A patients died due to the ICH event and three presented late sequelae. The most important aspect of ICH management is the early replacement therapy in haemophilic patients. This prompt treatment will increase the chances of a better prognosis. Another impact measure consists in the administration of the deficient coagulation factor after every head trauma, even when considered minor.

Health literacy, a more complex concept than knowledge, is a required capacity to obtain, understand, integrate and act on health information [1], in order to enhance individual and community health, which is defined by different levels, according to the autonomy and personal capacitation in decision making [2]. Medium levels of Health literacy in an adolescent population were found in a study conducted in 2013/2014, being higher in sexual and reproductive health and lower in substance use. It was also noticed that the higher levels of health literacy were in the area adolescents refer to have receipt more health information. The health literacy competence with higher scores was communication skills, and the lower scores were in the capacity to analyze factors that influence health. Higher levels were also found in younger teenagers, but in a higher school level, confirming the importance of health education in these age and development stage. Adolescents seek more information in health professionals and parents, being friends more valued as a source information in older adolescents, which enhance the importance of peer education mainly in older adolescents [3]. As a set of competences based on knowledge, health literacy should be developed through education interventions, encompassing the cultural and social context of individuals, since the society, culture and education system where the individual is inserted can define the way the development and enforcement of the health literacy competences [4]. The valued sources of information should be taken into account, as well as needs of information in some topics referred by adolescents in an efficient health education. Schizophrenia is a serious and chronic mental illness which has a profound effect on the health and well-being related with the well-known nature of psychotic symptoms. The exercise has the potential to improve the life of people with schizophrenia improving physical health and alleviating psychiatric symptoms. However, most people with schizophrenia remains sedentary and lack of access to exercise programs are barriers to achieve health benefits. The aim of this study is to evaluate the effect of exercise on I) the type of intervention in mental health, II) in salivary levels of alpha-amylase and cortisol and serum levels of S100B and BDNF, and on III) the quality of life and selfperception of the physical domain of people with schizophrenia. The sample consisted of 31 females in long-term institutions in the Casa de Saúde Rainha Santa Isabel, with age between 25 and 63, and with diagnosis of schizophrenia according to the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR). Physical fitness was assessed by the six-minute walk distance test (6MWD). Biological variables were determined by ELISA (Enzyme-Linked Immunosorbent Assay). Psychological variables were assessed using SF-36, PSPP-SCV, RSES and SWLS tests. Walking exercise has a positive impact on physical fitness (6MWD -p = 0.001) and physical components of the psychological test...

Our findings suggest that increasing access to clotting factor concentrates for young boys with severe haemophilia is a global imperative.

The Pro-FEIBA study reported health-related quality of life (HRQoL) improved following 6-month of Factor Eight Inhibitor Bypassing Activity (FEIBA) prophylaxis. This study investigates whether 12-month of FEIBA prophylaxis improved HRQoL in haemophilia patients with inhibitors. Thirty-six subjects in a 1-year prospective, randomized, open-label, parallel-design study were randomized to prophylaxis (85 ± 15 U kg(-1) every other day) or on-demand treatment. HRQoL was assessed at screening, 6 and 12-month termination using the EQ-5D, Haem-A-QoL, Haemo-QoL and a general pain visual analog scale (VAS). To evaluate changes, paired t-tests and criteria for minimally important differences were applied. Repeated measures regression tested the association between annualized bleeding rate (ABR) and physical HRQoL. At 6 and 12 months, prophylaxis subjects reported clinically meaningful improvement in EQ-5D index (mean improvement, 0.10 and 0.08, respectively) and both clinically meaningful and statistically significant improvements in EQ-VAS scores (16.9 and 15.7, respectively; P < 0.05) vs. baseline. General pain was significantly reduced during prophylaxis at each follow-up (mean improvement, 20.3 and 23.2, respectively; both P <0.05). At 12 months, prophylaxis subjects achieved significant improvements in Haem-A-QoL Total Score and in four domains: Physical Health, Feeling, View, and Work and School (all P < 0.05). No statistically significant changes, except for Haem-A-QoL Physical Health at 6 months, were observed with on-demand treatment. ABR was decreased by 72.5% with prophylaxis vs. on-demand treatment (P = 0.0003) and reduced ABR was associated with better physical HRQoL (P < 0.05). FEIBA prophylaxis significantly reduced ABR and improved HRQoL in inhibitor patients. Subjects with lower ABR reported better physical HRQoL.