Determinants of scholarly project completion in a paediatric resident program in Canada.

BACKGROUND: The primary aims of this study were to: 1) assess barriers and facilitators of completing scholarly projects from residents and faculty mentor perspectives, 2) determine the perceived value of new initiatives designed to support resident scholarly projects and 3) determine if these initiatives led to changes in resident publications. DESIGN AND METHODS: Between June and September 2014, we surveyed 18 paediatric residents and 41 faculty mentors regarding barriers to resident scholarly project completion and the value of new initiatives to support scholarly activity. We also tracked scientific publications by residents before and after implementation of these initiatives. RESULTS: The primary perceived barriers to research for residents and faculty were lack of protected time (64.3% versus 68.6%, respectively), lack of resident interest in scholarly activity (50.0% versus 60.0%, respectively) and lack of mentor motivation. Mentors and residents did not agree that lack of proper training in research (29% versus 54%, respectively) and faculty motivation (29% versus 17%, respectively) were barriers to completing a project. A dedicated research coordinator (71.4% versus 70.6%, respectively), a revised research curriculum (71.4% versus 41.2%, respectively) and works in progress sessions (50.0% versus 61.8%, respectively) were perceived as valuable initiatives to the program. These initiatives were not associated with changes in annual resident publication rates. CONCLUSIONS: Lack of time and competing clinical training are primary barriers to scholarly project completion for residents in addition to a lack of motivation on the part of faculty members. Improving program support was perceived as positive changes to address these barriers but did not increase resident publication rates. The information provided here could be used to tailor future resident research programs and highlight the value of gathering input from resident and faculty when designing initiatives to enhance resident research productivity.

Relative Risk and Incidence for Developmental Dysplasia of the Hip.

OBJECTIVE: To determine the incidence and associated risk factors of developmental dysplasia of the hip (DDH) in a modern population without universal screening. STUDY DESIGN: Children with DDH were identified from the Manitoba Centre for Health Policy's Data Repository by the use of International Classification of Diseases diagnosis codes as well as physician billing tariffs for surgical procedures for DDH for all children born between 1995 and 2012. To identify the outpatient-treated patients, ultrasound scans and radiographic imaging for DDH were reviewed for 2004-2012. Overall incidence was calculated on the basis of birth rate for the province per year. Relative risks of sex, first born, breech position, clubfoot deformity, multiple gestations, as well as regional health areas were analyzed with χ2 tests. RESULTS: We identified 1716 cases of DDH of 258 499 newborns. The incidence of DDH was calculated at 6.6/1000 newborns. Late-presenting DDH was detected in 2.2/1000 newborns. Female first-born children, clubfoot deformity, and breech position were associated significantly with an increased risk. Children with DDH born in rural areas of the Northern and Central part of Manitoba presented at a later age than those who are born in the urban areas (P < .0001) CONCLUSION: This study shows the need for improved early detection and awareness at well-baby clinics of risk factors and regional differences for DDH.

Survival of children with medulloblastoma in Canada diagnosed between 1990 and 2009 inclusive.

The treatment of medulloblastoma, the most common malignant brain tumor in children, has evolved over the last few decades. The objectives of this paper were to determine the survival of pediatric medulloblastoma in Canada, to determine if there has been an improvement in the survival rates between the years of 1990 and 2009, inclusive, and to determine prognostic factors for survival. All patients under the age of 18 years diagnosed with medulloblastoma from 1990 to 2009, inclusive, in Canada were included. Data collected included date of diagnosis, age at diagnosis, gender, stage, pathology, treatment, recurrence and current status. From these, survival rates were determined. Data were obtained on 628 eligible patients. The overall 5-year survival rate for the study time period was 69.2 ± 3.3 %. The survival rate increased during the interval of 1996-2000, then remained stable; 1990-1994: 60.2 ± 4.3 %; 1995-1999: 73.2 ± 3.5 %; 2000-2004: 68.8 ± 3.7 %; and 2005-2009: 72.1 ± 4.9 %, p = 0.05. Children over 14 years of age had a significantly better overall survival than those age 5-14 and those under 5 (85.7 ± 5.5 % vs 76.1 ± 2.7 % and 60.8 ± 3 % respectively, p = 0.001). Histologic medulloblastoma subtype and M stage of disease did not result in significant differences in survival. Despite changes in approaches to therapy, we demonstrate a steady survival rate for children with medulloblastoma after 1996. In our analyses, age over 14 years was associated with a higher survival rate.

Incidence of medulloblastoma in Canadian children.

Medulloblastoma is the most common malignant brain tumor in children. There was a perception of pediatric neuro-oncologists that the incidence had declined in Canada. An epidemiological survey was undertaken to determine the incidence of this tumor in Canada and if a change had indeed occurred. All patients 14 years and under diagnosed with medulloblastoma from 1990 to 2009 inclusive in Canada were included. Data collected included date of diagnosis, age at diagnosis, gender, stage, pathology, treatment, recurrence and current status. Data were analysed for change in incidence over time. Data were obtained on 574 eligible patients. The mean overall incidence per 1,000,000 persons was 4.82 (95 % CI 4.28-5.35) for the study time period. The mean age at diagnosis was 5.8 years, and there was a male predominance. Although there was an increase in incidence over the first three time periods (24 % for 1990-1994, 27.5 % for 1995-1999, 27.7 % for 2000-2004), the most recent time period (2005-2009) showed a decrease (21 %). This was true for male children while the incidence was stable for females. The mean incidence rate was double for children under the age of 5 years (7.92 per million) compared to those over 5 years (3.64 per million).This study showed that from 1990 to 2009 the incidence of medulloblastoma was relatively stable, with a slight decrease in the last five-year time period.

Haploinsufficiency of an RB-E2F1-Condensin II complex leads to aberrant replication and aneuploidy.

UNLABELLED: Genome instability is a characteristic of malignant cells; however, evidence for its contribution to tumorigenesis has been enigmatic. In this study, we demonstrate that the retinoblastoma protein, E2F1, and Condensin II localize to discrete genomic locations including major satellite repeats at pericentromeres. In the absence of this complex, aberrant replication ensues followed by defective chromosome segregation in mitosis. Surprisingly, loss of even one copy of the retinoblastoma gene reduced recruitment of Condensin II to pericentromeres and caused this phenotype. Using cancer genome data and gene-targeted mice, we demonstrate that mutation of one copy of RB1 is associated with chromosome copy-number variation in cancer. Our study connects DNA replication and chromosome structure defects with aneuploidy through a dosage-sensitive complex at pericentromeric repeats. SIGNIFICANCE: Genome instability is inherent to most cancers and is the basis for selective killing of cancer cells by genotoxic therapeutics. In this report, we demonstrate that instability can be caused by loss of a single allele of the retinoblastoma gene that prevents proper replication and condensation of pericentromeric chromosomal regions, leading to elevated levels of aneuploidy in cancer.