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Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype. Broad approval of therapy necessitates close follow-up of potential rare adverse events and effectiveness in the larger real-world population.
The Canadian Neuromuscular Disease Registry (CNDR) undertook an iterative multi-stakeholder process to expand the existing SMA dataset to capture items relevant to patient outcomes in a post-marketing environment. The CNDR SMA expanded registry is a longitudinal, prospective, observational study of patients with SMA in Canada designed to evaluate the safety and effectiveness of novel therapies and provide practical information unattainable in trials.
The consensus expanded dataset includes items that address therapy effectiveness and safety and is collected in a multicenter, prospective, observational study, including SMA patients regardless of therapeutic status. The expanded dataset is aligned with global datasets to facilitate collaboration. Additionally, consensus dataset development aimed to standardize appropriate outcome measures across the network and broader Canadian community. Prospective outcome studies, data use, and analyses are independent of the funding partner.
Prospective outcome data collected will provide results on safety and effectiveness in a post-therapy approval era. These data are essential to inform improvements in care and access to therapy for all SMA patients.
Enterococcus causes clinically significant bloodstream infections (BSIs). In centers with a higher prevalence of vancomycin resistant enterococcus (VRE) colonization, a common clinical question is whether empiric treatment directed against VRE should be initiated in the setting of a suspected enterococcal BSI. Unfortunately, VRE treatment options are limited, and relatively expensive, and subject patients to the risk of adverse reactions. We hypothesized that the results of VRE colonization screening could predict vancomycin resistance in enterococcal BSI.
We reviewed 370 consecutive cases of enterococcal BSI over a 7-year period at 2 tertiary-care hospitals to determine whether vancomycin-resistant BSIs could be predicted based on known colonization status (ie, patients with swabs performed within 30 days, more remotely, or never tested). We calculated sensitivity and specificity, and we plotted negative predictives values (NPVs) and positive predictive values (PPVs) as a function of prevalence.
A negative screening swab within 30 days of infection yielded NPVs of 90% and 95% in settings where <27.0% and 15.0% of enterococcal BSI are resistant to vancomycin, respectively. In patients with known VRE colonization, the PPV for VRE in enterococcal BSI was >50% at any prevalence exceeding 25%.
The results of a negative VRE screening test result performed within 30 days can help eliminate unnecessary empiric therapy in patients with suspected enterococcal BSI. Conversely, patients with positive VRE screening swabs require careful consideration of empiric VRE-directed therapy when enterococcal BSI appears likely.
Exclusive breast-feeding (EBF) provides optimal nutrition for infants and mothers. The practice of EBF while adhering to antiretroviral medication decreases the risk of mother-to-child transmission of HIV from approximately 25 % to less than 5 %. Thus the WHO recommends EBF for the first 6 months among HIV-infected women living in resource-limited settings; however, EBF rates remain low. In the present study our aim was to design and implement a pilot intervention promoting EBF among HIV-infected women.
The Information–Motivation–Behavioural Skills (IMB) model was applied in a brief motivational interviewing counselling session that was tested in a small randomized controlled trial.
Pietermaritzburg, South Africa, at two comparable rural public health service clinics.
Sixty-eight HIV-infected women in their third trimester were enrolled and completed baseline interviews between June and August 2014. Those randomized to the intervention arm received the IMB-based pilot intervention directly following baseline interviews. Follow-up interviews occurred at 6 weeks postpartum.
While not significantly different between trial arms, high rates of intention and practice of EBF at 6-week follow-up were reported. Findings showed high levels of self-efficacy being significantly predictive of breast-feeding initiation and duration regardless of intervention arm.
Future research must account for breast-feeding self-efficacy on sustaining breast-feeding behaviour and leverage strategies to enhance self-efficacy in supportive interventions. Supporting breast-feeding behaviour through programmes that include both individual-level and multi-systems components targeting the role of health-care providers, family and community may create environments that value and support EBF behaviour.
The attachment system is responsible for emotional-motivational bonding with others and is associated with individual emotion regulation strategies (avoidance-disengagement; anxiety-hypervigilance); however, little is known how these individual differences in emotion regulation strategies influence partners’ interpersonal emotional experiences. Prior research examining the link between individual differences in attachment avoidance and anxiety and emotional connectedness in couples has interestingly shown counter-intuitive effects of individual attachment styles on couples’ shared emotions, such that attachment anxiety was associated with the lowest levels of emotional synchrony (Butner, Diamond, & Hicks, 2007). These results beg for additional research on whether and how individual differences in attachment styles moderate the transmission of emotion between partners. Using daily diaries and second-by-second measures of emotional experience from 30 couples, it was hypothesised that couples high in attachment avoidance (disengagement) would show lower levels, whereas couples high in attachment anxiety (hypervigilance) would show higher levels of emotion transmission. Results were counter to our predictions; attachment avoidance increased — and attachment anxiety decreased — emotion transmission between partners. Findings suggest attachment dynamics may not have the same effect on couples’ joint emotional functioning in a dyadic context as they do on individuals’ emotional functioning.
Girls receiving lower quality paternal investment tend to engage in more risky sexual behavior (RSB) than peers. Whereas paternal investment theory posits that this effect is causal, it could arise from environmental or genetic confounds. To distinguish between these competing explanations, the current authors employed a genetically and environmentally controlled sibling design (N = 101 sister pairs; ages 18–36), which retrospectively examined the effects of differential sibling exposure to family disruption/father absence and quality of fathering. Consistent with a causal explanation, differences between older and younger sisters in the effects of quality of fathering on RSB were greatest in biologically disrupted families when there was a large age gap between the sisters (thus maximizing differential exposure to fathers), with greater exposure within families to higher quality fathering serving as a protective factor against RSB. Further, variation around the lower end of fathering quality appeared to have the most influence on RSB. In contrast, differential sibling exposure to family disruption/father absence (irrespective of quality of fathering) was not associated with RSB. The differential sibling-exposure design affords a new quasi-experimental method for evaluating the causal effects of fathers within families.
There is currently little information on the genetic epidemiology of Alzheimer disease (AD) among North American Aboriginal populations. No cases of familialAD (FAD) in these populations have been published to date.
Here, we describe a large North American Aboriginal kindred with early onset FAD (EOFAD) in which genetic testing was done.
Results and Conclusions:
A novel Presenilin 1 (PS1) gene mutation (L250F) has been identified. In contrast to the three previously reported families with PS1 codon 250 mutations, affected members of this kindred demonstrate neither myoclonus nor seizures. Furthermore, the identification of a PS1 mutation in a North American Aboriginal kindred presents several unique challenges with respect to knowledge transfer and continuity of care in a geographically remote and culturally distinct community.
We demonstrate the facile fabrication of crack-free nanostructured crystalline titania into microsystems. Titania layers were formed by reacting Ti thin films, deposited by evaporation and sputtering, with aqueous H202. Cracks were observed in titania layers formed on blanket Ti films but absent on arrays of patterned Ti pads below a threshold dimension. Nanostructured titania formed from sputtered and evaporated Ti films consists of aligned fibrous and sponge-like nanoporous morphologies, respectively. Rat fibroblasts L-cells cultured on these titania fibers remain viable up to 3 days. These observations demonstrate the feasibility of this technique to integrate nanostructured titania into Nano|Micro-Electromechanical systems (N|MEMS) devices.
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