Cape Breton Regional Hospital

BACKGROUND: The present study examined the disengage and shift operations of visual attention in young children with autism. METHODS: For this purpose, we used a simple visual orienting task that is thought to engage attention automatically. Once attention was first engaged on a central fixation stimulus, a second stimulus was presented on either side, either simultaneously or successively. Latency to begin an eye movement to the peripheral stimulus served as the main dependent measure. The two stimulus conditions (simultaneous and successive) provided independent measures of disengaging and shifting attention, respectively. Performance of children with autism was compared to that of children with Down syndrome and a normal group. RESULTS: The main finding was that relative to both comparison groups, children with autism had marked difficulty in disengaging attention. Indeed, on 20% of trials they remained fixated on the first of two competing stimuli for the entire 8-second trial duration. Evidence is also provided for a more subtle problem in executing rapid shifts of attention. CONCLUSIONS: Our findings on disengagement in autism parallel those reported in normal 2-month-olds, in whom attention has been described as 'obligatory'. Discussion focuses on the potential role of general versus domain-specific processes in producing some of the core features of autism.

Coxiella burnetii pneumonia developed in all three members of one family 8 to 12 days following exposure to an infected parturient dog. The dog gave birth to four pups; three died shortly after birth, and the fourth died within 24 hours of birth.

BACKGROUND: Sellar masses (SM) are mostly benign growths of pituitary or nonpituitary origin that are increasingly encountered in clinical practice. To date, no comprehensive population-based study has reported the epidemiology of SM from North America. AIM: To determine the epidemiology of SM in the province of Nova Scotia, Canada. METHODS: Data from all pituitary-related referrals within the province were prospectively collected in interlinked computerized registries starting in November 2005. We conducted a retrospective analysis on all patients with SM seen within the province between November 2005 and December 2013. RESULTS: A total of 1107 patients were identified, of which 1005 were alive and residing within the province. The mean age at presentation was 44.6±18 years, with an overall female preponderance (62%) and a population prevalence rate of 0.1%. Of patients with SM, 837 (83%) had pituitary adenomas and 168 (17%) had nonpituitary lesions. The relative prevalence and standardized incidence ratio, respectively, of various SM were: nonfunctioning adenomas (38.4%; 2.34), prolactinomas (34.3%; 2.22), Rathke's cyst (6.5%; 0.5), growth hormone-secreting adenomas (6.5%; 0.3), craniopharyngiomas (4.5%; 0.2), adrenocorticotropic hormone-secreting adenomas (3.8%; 0.2), meningiomas (1.9%), and others (3.9%; 0.21). At presentation, 526 (52.3%) had masses ≥1 cm, 318 (31.6%) at <1 cm, and 11 (1.1%) had functioning pituitary adenomas without discernible tumor, whereas tumor size data were unavailable in 150 (14.9%) patients. The specific pathologies and their most common presenting features were: nonfunctioning adenoma (incidental, headaches, and vision loss), prolactinomas (galactorrhea, menstrual irregularity, and headache), growth hormone-secreting adenomas (enlarging extremities and sweating), adrenocorticotropic hormone-secreting adenoma (easy bruising, muscle wasting, and weight gain) and nonpituitary lesions (incidental, headaches, and vision problems). Secondary hormonal deficiencies were common, ranging from 19.6% to 65.7%; secondary hypogonadism, hypothyroidism, and growth hormone deficiencies constituted the majority of these abnormalities. CONCLUSIONS: This is the largest North American study to date to assess the epidemiology of SM in a large stable population. Given their significant prevalence in the general population, more studies are needed to evaluate the natural history of these masses and to help allocate appropriate resources for their management.

Gold nanomaterials have received great interest for their use in cancer theranostic applications over the past two decades. Many gold nanoparticle-based drug delivery system designs rely on adsorbed ligands such as DNA or cleavable linkers to load therapeutic cargo. The heightened research interest was recently demonstrated in the simple design of nanoparticle-drug conjugates wherein drug molecules are directly adsorbed onto the as-synthesized nanoparticle surface. The potent chemotherapeutic, doxorubicin often serves as a model drug for gold nanoparticle-based delivery platforms; however, the specific interaction facilitating adsorption in this system remains understudied. Here, for the first time, we propose empirical and theoretical evidence suggestive of the main adsorption process where (1) hydrophobic forces drive doxorubicin towards the gold nanoparticle surface before (2) cation-π interactions and gold-carbonyl coordination between the drug molecule and the cations on AuNP surface facilitate DOX adsorption. In addition, biologically relevant compounds, such as serum albumin and glutathione, were shown to enhance desorption of loaded drug molecules from AuNP at physiologically relevant concentrations, providing insight into the drug release and in vivo stability of such drug conjugates.

The histopathological changes of herpes simplex, herpes zoster, and varicella are considered to be indistinguishable from one another. Evaluation of the clinical setting, with adjunctive studies if necessary, generally clarifies the specific diagnosis. Vesicular lesions in all three conditions can involve epidermal and adnexal epithelium with characteristic cytopathic features. We describe three patients with non-vesicular eruptions on the head and neck whose biopsies revealed exclusive folliculosebaceous involvement by herpes. All three patients developed typical herpes zoster within days of the biopsy. There is compelling scientific evidence in the literature indicating that, in herpes zoster, the virus is transported from dorsal root or trigeminal ganglia via myelinated nerves to the skin. These terminate at the isthmus of hair follicles and primary infection of follicular and sebaceous epithelium occurs. Spread of infection to the epidermis follows. In contrast, data pertaining to recurrent herpes simplex indicates that axonal transport of the virus from sensory ganglia to the skin is directed primarily to the epidermis, via terminal non-myelinated nerve twigs. The clinical evolution of our three cases and scientific data in the literature indicate that exclusive folliculosebaceous involvement by herpes, in the setting of a non-vesicular eruption, represents early herpes zoster.

BACKGROUND: Techniques for repairing the meniscus include both open and arthroscopic techniques using sutures and bioabsorbable implants. The purpose of this study was to compare the effectiveness of inside-out suturing and bioabsorbable arrows for repair of vertical meniscal lesions. STUDY DESIGN: Randomized controlled clinical trial; Level of evidence, 1. METHODS: One hundred consecutive patients were randomly assigned to arrows (n = 51) or sutures (n = 49). Sixty-five percent of patients (31 sutures, 34 arrows) underwent a concomitant anterior cruciate ligament reconstruction. A blinded research associate conducted assessments at 6 weeks and 3, 6, 12, and 24 months postoperatively. The primary outcome was retear rate. Secondary outcomes included the Western Ontario Meniscal Evaluation Tool, Anterior Cruciate Ligament Quality of Life Outcome Measure, and side-to-side comparisons of flexion and extension. RESULTS: At baseline, groups were similar in age, gender, time from injury to surgery, and length and location of tear. Mean follow-up was 28.0 +/- 8.4 months. There were 22 failed meniscal repairs (11 in each group), which did not represent a significant difference in the rate of failure between groups (P = .92). The mean quality of life scores and side-to-side differences in extension and flexion measurements were not significantly different between groups. Two patients from the arrow group crossed over into the suture group at the time of surgery because of technical difficulties with the device, and in 3 instances, a single suture was needed to keep the tear reduced while arrows were introduced. Two patients required reoperation for removal of a prominent, subcutaneous arrow, and 1 patient in the suture group suffered a transient peroneal nerve palsy during revision suturing. CONCLUSION: At intermediate follow-up, there were no statistically significant differences in measured outcomes between meniscal suturing and arrows. Longer term follow-up is necessary to identify differences between these 2 treatments, particularly to estimate the incidence of articular surface damage in patients whose meniscal tear was repaired using arrows.

OBJECTIVES: Pituitary incidentalomas (PI) are frequently found on brain imaging. Despite their high prevalence, little is known about their long-term natural history and there are limited guidelines on how to monitor them. METHODS: We conducted a retrospective study to compare epidemiological characteristics at presentation and the natural history of PI in population-based vs referral-based registries from two tertiary-care referral centers in Canada. RESULTS: A total of 328 patients with PI were included, of whom 73% had pituitary adenomas (PA) and 27% had non-pituitary sellar masses. The commonest indications for imaging were headache (28%), dizziness (12%) and stroke/transient ischemic attack (TIA) (9%). There was a slight female preponderance (52%) with a median age of 55 years at diagnosis; 71% presented as macroadenomas (>10mm). Of PA, 25% were functioning tumors and at presentation 36% of patients had evidence of secondary hormonal deficiency (SHD). Of the total cohort, 68% were treated medically or conservatively whereas 32% required surgery. Most tumors (87% in non-surgery and 68% in post-surgery group) remained stable during follow-up. Similarly, 84% of patients in the non-surgery and 73% in the surgery group did not develop additional SHD during follow-up. The diagnosis of non-functioning adenoma was a risk factor for tumor enlargement and a change in SHD status was associated with a change in tumor size. CONCLUSIONS: Our data suggest that most PI seen in tertiary-care referral centers present as macroadenomas and may frequently be functional, often requiring medical or surgical intervention.

AIM: The purpose of the present study was to determine the accuracy of nurses' self-reports of absence by examining: (1) the correlation, intra-class correlation, and Cronbach's alpha for self-reported absence and absence as reported in organizational records, (2) difference in central tendency for the two measures of absence and (3) the percentage of nurses who underestimate their absence. BACKGROUND: Research on nurses' absenteeism has often relied on self-reports of absence. However, nurses may not be aware of their actual absenteeism, or they may underestimate it. METHOD: Self-reported absence from questionnaires completed by 215 Canadian nurses was compared with their absence from organizational records. RESULTS: There is a strong positive correlation, a strong intra-class correlation and Cronbach's alpha for the two measures of absence. However, there is a difference in central tendency that is related to the majority of nurses in this study (51.1%) underestimating their days absent from work. CONCLUSIONS: Research examining the predictors of absence may consider measuring absence with self-reports. Nevertheless, nurses demonstrated a bias to underestimate their absence. IMPLICATIONS FOR NURSING MANAGEMENT: Feedback interventions to reduce absenteeism can be developed to include providing nurses with accurate information about their absence.

Carers often feel disempowered and engage in behaviours that inadvertently enable their loved one's ED symptoms and yet little is known regarding these processes. This study examined the relationships among fear, self-blame, self-efficacy, and accommodating and enabling behaviours in 137 carers of adolescents and adults with ED. The results revealed that fear and self-blame predicted low carer self-efficacy in supporting their loved one's recovery as well as the extent to which carers reported engaging in recovery-interfering behaviours. The relevance of these findings are discussed in the context of family-oriented ED therapies and highlight the importance for clinicians to attend to and help to process strong emotions in carers, in order to improve their supportive efforts and, ultimately, ED outcomes.

A process model was tested whereby parental fear and self-blame were targeted in order to enhance parental self-efficacy and supportive efforts in the context of emotion-focused family therapy (EFFT) for eating disorders (ED). A 2-day EFFT group intervention was delivered to parents of adolescent and adult children with ED. Data were collected from eight treatment sites (N = 124). Data were analyzed using t-tests, regression analyses and structural equation modeling. The findings supported the proposed process model. Through the processing of parents’ maladaptive fear and self-blame, parents felt more empowered to support their child’s recovery. This increase in self-efficacy led to an increase in parents’ intentions to engage in recovery-focused behaviors. This study is the first to test a method for clinicians to increase supportive efforts by targeting and enhancing caregiver self-efficacy via the processing of emotion.

Merkel cell carcinoma (MCC) is a rare, frequently lethal, primary neuroendocrine carcinoma of the skin. Histopathologically, it appears as a dermal nodule of small, undifferentiated malignant cells. Historically, MCC was considered to be an eccrine carcinoma. Recognition of its neuroendocrine features later led to the hypothesis that it arose from Merkel cells in the skin, although recent evidence revisits the question of an epithelial origin. We present a case of MCC arising on the temple of a 78-year-old male, in association with an actinic keratosis. Three years later, a local tumor recurrence showed a mixed malignancy comprising small cell neuroendocrine and large cell squamoid components. A further recurrence at the site two years later, after local radiotherapy, revealed a bizarre pleomorphic large cell morphology with retention of immunohistochemical features of a neuroendocrine carcinoma. Evolution to a bizarre pleomorphic large cell neoplasm has been recorded in malignant tumors treated by radiotherapy, but is unique for MCC. The association of this MCC with an actinic keratosis and the development of squamoid differentiation in a local recurrence support the link between MCC and epithelial neoplasia. In addition, its evolution to an atypical fibroxanthoma-like morphology is of interest, because some view atypical fibroxanthomas as bizarre variants of squamous cell carcinoma.

Mycosis fungoides, a cutaneous T-cell lymphoma, typically presents as indolent, progressive, and persistent erythematous patches or plaques with mild scaling and over time can evolve into tumor stage with tumor nodules. Other presentations include eczematous, psoriasiform, poikilodermatous, and hypopigmented patches. We report Mycosis fungoides in a 14-year-old boy presenting as pigmented purpuric dermatitis and review the relevant literature. This is a rare presentation of a condition that is uncommon in the pediatric population. In our patient, histologic features were typical of Mycosis fungoides presenting as pigmented purpuric dermatitis. The clinical features, pathology, molecular biology, and the relationship between these two entities are discussed.

OBJECTIVE: To determine the proportion of patients with rheumatoid arthritis (RA) under rheumatologic care treated with disease-modifying antirheumatic drugs (DMARD) within 6 months from symptom onset and the components of time to treatment and its predictors. METHODS: A historical inception cohort of 339 patients with RA randomly selected from 18 rheumatology practices was audited. The proportion that initiated DMARD treatment within 6 months from symptom onset was estimated using Kaplan-Meier analysis. Time to each component of the care pathway was estimated. Multivariable modeling was used to determine predictors of early treatment using 12 preselected variables available in the clinical charts. Bootstrapping was used to validate the model. RESULTS: Within 6 months from symptom onset, 41% (95% CI 36%-46%) of patients were treated with DMARD. The median time to treatment was 8.4 (interquartile range 3.8-24) months. Events preceding rheumatology referral accounted for 78.1% of the time to treatment. The most prominent predictor of increased time to treatment was a concomitant musculoskeletal condition, such as osteoarthritis or fibromyalgia. The significance of other variables was less consistent across the models investigated. Included variables accounted for 0.69 ± 0.03 of the variability in the model. CONCLUSION: Fewer than 50% of patients with RA are treated with DMARD within 6 months from symptom onset. Time to referral to rheumatology represents the greatest component delay to treatment. Concomitant musculoskeletal condition was the most prominent predictor of delayed initiation of DMARD. Implications of these and other findings warrant further investigation.

The medical management of end-of-life symptoms, and the psychosocial care of the dying and their families have not been a specific part of the curriculum for undergraduate medical students or residency training programs. The purpose of our research was to assess family medicine residents' knowledge of and attitudes toward care of the dying. All entering (PGY1) and exiting (PGY2) residents of the Dalhousie University Family Medicine Residency Program were given a 50-item survey on end-of-life care. They survey contains two 25-item subscales concerning attitudes/opinions toward end-of-life care, and knowledge about care. Thirty-one of the 33 entering PGY1s 94%) and 26 of the 30 exiting PGY2s (86%) completed the surveys. Overall attitude scores were felt to be high among both groups, with little difference between them. Areas of concern regarding the adequacy of knowledge were found in relation to managing opioid drugs and the symptom of dyspnea. Interventions are now in development to address these issues in the residency program. In an era of subspecialties, the challenge of integrating these areas into the curriculum without creating rotations in specialist palliative care is an issue faced by most family medicine residency programs.

Expressed emotion (EE) was measured in the parental primary carer (carer) of 92 adolescents with intellectual impairment to examine its associated characteristics. High EE was mainly a result of high levels of emotional overinvolvement. High EE was associated with psychological illhealth, poor-quality marriage and poor practical social support of the carer, and psychiatric disorder in the adolescent with intellectual impairment. This suggests that EE may be a useful indicator of coping difficulties in these families. The subgroup of high EE emotional overinvolvement was associated with a carer with more psychological illhealth, a worse-quality marriage, less practical social support, greater professional support and an insecure style of respite care usage for an adolescent of greater intellectual impairment. The subgroup of criticism have an adolescent of less severe intellectual impairment, more behavioural disturbance and yet the carer has less professional support. Appreciation of the quality of the relationship of the carer with their dependent family member may enable greater understanding of how to improve the quality of life for both the carer and the cared for.

BACKGROUND: Arteriosclerosis and emphysema develop in individuals with Schimke immuno-osseous dysplasia (SIOD), a multisystem disorder caused by biallelic mutations in SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1). However, the mechanism by which the vascular and pulmonary disease arises in SIOD remains unknown. METHODS: We reviewed the records of 65 patients with SMARCAL1 mutations. Molecular and immunohistochemical analyses were conducted on autopsy tissue from 4 SIOD patients. RESULTS: Thirty-two of 63 patients had signs of arteriosclerosis and 3 of 51 had signs of emphysema. The arteriosclerosis was characterized by intimal and medial hyperplasia, smooth muscle cell hyperplasia and fragmented and disorganized elastin fibers, and the pulmonary disease was characterized by panlobular enlargement of air spaces. Consistent with a cell autonomous disorder, SMARCAL1 was expressed in arterial and lung tissue, and both the aorta and lung of SIOD patients had reduced expression of elastin and alterations in the expression of regulators of elastin gene expression. CONCLUSIONS: This first comprehensive study of the vascular and pulmonary complications of SIOD shows that these commonly cause morbidity and mortality and might arise from impaired elastogenesis. Additionally, the effect of SMARCAL1 deficiency on elastin expression provides a model for understanding other features of SIOD.

OBJECTIVE: The Aim: To evaluate risk factors for dysmenorrhea in women of reproductive age and to review its characteristics. PATIENTS AND METHODS: Material and Methods: A questionnaire was conducted among 354 women followed by a statistical analysis of the gathered data. RESULTS: Results: Among the interviewed women 84.2 % suffer from painful menstruations. Women under 25 years of age are more likely to experience pain, which reduces with age. Dysmenorrhea is more common among women whose menstrual cycles are irregular (92.5%) with a number of bleeding days 4-7 (90.7 %), though still occurring within the normal 21-35 days interval (87.6 %). Nulliparous women are more likely to experience menstrual pain (89.9 %). CONCLUSION: Conclusion: Dysmenorrhea depends on women's age, length of menstrual bleeding, length of menstrual cycle and its regularity, age at menarche and parity, but does not depend on body mass index and smoking status.

Little is known about longitudinal trends in objectively measured physical activity (PA) during and after pulmonary rehabilitation (PR) for individuals with Chronic Obstructive Pulmonary Disease (COPD). The purpose of this study was to examine the PA trajectories of patients with COPD during and after PR and whether demographic, clinical, or program characteristics differed across these trajectories. The study was approved by Research Ethics Boards at all participating institutions, and written informed consent was obtained from each participant prior to study inclusion. COPD patients (N = 190) completed a questionnaire and wore a pedometer for 7 days at baseline, end of PR, and 3 and 9 months after completing PR. Latent class growth analyses showed that two distinct PA trajectories emerged. Active Maintainers averaged 9177 steps/day at baseline, and maintained this level throughout the assessment and post rehabilitation period. In contrast, Inactive Maintainers averaged 3133 steps/day at baseline, which also remained stable during and after PR. Follow-up analyses showed the Inactive Maintainers were more likely to be retired from work and have lower baseline scores for their stress tests and 6-minute walk tests compared to Active Maintainers (all p < 0.05). These results suggest that two distinct steps/day trajectories exist for COPD patients during and after completing PR that are partially explained by specific demographic and clinical characteristics.

The annual Eastern Canadian Colorectal Cancer Consensus Conference was held in Montreal, Quebec, October 22-24, 2009. Health care professionals involved in the care of patients with colorectal cancer participated in presentation and discussion sessions for the purposes of developing the recommendations presented here. This consensus statement addresses current issues in the management colorectal cancer, such as the management of hepatic and pulmonary metastases, the role of monoclonal antibodies to the epidermal growth factor receptor, and the benefits and safety of chemotherapy in elderly patients. The management of gastrointestinal neuroendocrine tumours and gastric cancer are also discussed.

BACKGROUND AND OBJECTIVES: In 2014, Nova Scotia released a provincial palliative care strategy and implementation working groups were established. The Capacity Building and Practice Change Working Group, comprised of health professionals, public advisors, academics, educators, and a volunteer supervisor, was asked to select palliative care education programs for health professionals and volunteers. The first step in achieving this mandate was to establish competencies for health professionals and volunteers caring for patients with life-limiting illness and their families and those specializing in palliative care. METHODS: In 2015, a literature search for palliative care competencies and an environmental scan of related education programs were conducted. The Irish Palliative Care Competence Framework serves as the foundation of the Nova Scotia Palliative Care Competency Framework. Additional disciplines and competencies were added and any competencies not specific to palliative care were removed. To highlight interprofessional practice, the framework illustrates shared and discipline-specific competencies. Stakeholders were asked to validate the framework and map the competencies to educational programs. Numerous rounds of review refined the framework. RESULTS: The framework includes competencies for 22 disciplines, 9 nursing specialties, and 4 physician specialties. CONCLUSIONS: The framework, released in 2017, and the selection and implementation of education programs were a significant undertaking. The framework will support the implementation of the Nova Scotia Integrated Palliative Care Strategy, enhance the interprofessional nature of palliative care, and guide the further implementation of education programs. Other jurisdictions have expressed considerable interest in the framework.