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Costea I.,Public Health Agency of Canada | MacK D.R.,Childrens Hospital of Eastern Ontario | Lemaitre R.N.,University of Washington | Israel D.,British Columbias Childrens Hospital | And 3 more authors.
Gastroenterology | Year: 2014

Increased dietary ratios of ω6/ω3 polyunsaturated fatty acids have been implicated in the pathogenesis of Crohn's disease (CD), but epidemiologic data are limited. We investigated whether variants of genes that control polyunsaturated fatty acid metabolism (CYP4F3, FADS1, and FADS2), along with the dietary ratio of ω6/ω3, confers susceptibility to CD. Based on data from 182 children newly diagnosed with CD and 250 controls, we found that children who consumed a higher dietary ratio of ω6/ω3 were susceptible for CD if they were also carriers of specific variants of CYP4F3 and FADS2 genes. Our findings implicate diet-gene interactions in the pathogenesis of CD. © 2014 by the AGA Institute.


Fryer C.,British Columbias Childrens Hospital
Pediatric Blood and Cancer | Year: 2011

Most publications report the adverse (negative) health issues in childhood cancer survivors. Presenting information to the newly diagnosed patient in a positive manner is advocated, while noting that recurrence is the most likely adverse event. Re-analysis of population-based studies on life-threatening toxicities from Nordic, Dutch, United Kingdom, French, Italian, and N. American publications shows that 5-year survivors have a near normal life expectancy, 75% have no severe or life-threatening treatment related toxicity and 87% remain free of a second malignancy. Children who received radiation or anthracycline >250-300mg/m 2 are at greatest risk for treatment related life-threatening toxicities. © 2011 Wiley Periodicals, Inc.


Chanoine J.-P.,University of British Columbia | Chanoine J.-P.,British Columbias Childrens Hospital | Richard M.,University of British Columbia
International Journal of Pediatric Obesity | Year: 2011

Background. Pharmacotherapy, associated with a comprehensive weight loss intervention, has emerged as a promising therapeutic approach in adolescents. Identification of subjects who best respond to a pharmacological intervention remains difficult. Objective. To compare the value of early weight loss after 12 weeks of treatment with placebo or orlistat (120 mg three times a day) in predicting treatment outcome after 52 weeks. Methods. Secondary analysis of a randomized control trial in 182 placebo-treated and 357 orlistat-treated obese adolescents (Body mass index [BMI] ≥2 kg/m2 above the 95 th percentile). Results. Percent weight change at 12 weeks was positively correlated with percent change in weight (r2 ≥ 0.41), BMI (r2 ≥ 0.33) and waist circumference (r2 ≥ 0.20) at 52 weeks in both the placebo and orlistat groups (P < 0.001). A weight loss ≥5% of baseline weight at 12 weeks was associated with a mean weight loss of 8.1% (95% CI: 6.4 to 9.7) at the study end that was independent of treatment. Subjects in the orlistat group were 2.44 times (95% CI: 1.34 to 4.46) more likely to experience a weight loss ≥5% after 12 weeks than subjects in the placebo group (P = 0.0028). Conclusions. Early weight loss predicts a favourable outcome in both placebo-treated and orlistat-treated subjects but is more than 2 times more likely to occur in the orlistat group. Addition of orlistat should be considered as part of a weight loss intervention but reevaluated after 3 months of treatment. © 2011 Informa Healthcare.


Heran M.K.S.,British Columbias Childrens Hospital | Hockley A.,British Columbias Childrens Hospital
Pediatric Cardiology | Year: 2011

We present the case of a 5-year-old boy diagnosed with Kawasaki disease at 10 months of age who developed fusiform aneurysms of the coronary arteries as well as a previously undescribed pattern of mirror-image peripheral arterial aneurysms. These aneurysms were diagnosed on conventional angiography of the aortoiliac circulation and the bilateral upper extremity circulation. © Springer Science+Business Media, LLC 2011.


Morishita K.,British Columbias Childrens Hospital | Petty R.E.,British Columbias Childrens Hospital
Rheumatology | Year: 2011

The mucopolysaccharidoses (MPSs) are a heterogeneous group of inherited metabolic disorders caused by enzyme deficiencies that lead to progressive lysosomal storage of glycosaminoglycans. Musculoskeletal manifestations are common across all forms of MPS and are often apparent early in the disease course. Diagnostic delays occur frequently in these patients, especially those with more attenuated forms of disease. Treatments for many types of MPS are now available; however, they are most effective if started early before the development of irreversible damage. Some manifestations such as stiffness and joint contractures may mimic other conditions such as inflammatory arthritis, which may cause further delays. Rheumatologists and other specialists should be aware of the musculoskeletal manifestations of MPS so that diagnostic delays can be avoided and appropriate management initiated. © The Author 2011. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.


Krempien J.L.,University of British Columbia | Krempien J.L.,British Columbias Childrens Hospital | Barr S.I.,University of British Columbia
Eating Behaviors | Year: 2012

Athletes with a spinal cord injury (SCI) appear to have relatively modest energy requirements despite demanding training regimes. Virtually nothing is known about the factors which influence the energy intake of those with a SCI including food related attitudes and behaviours. Using a cross-sectional observational design, three aspects of eating attitudes were measured using the Three-Factor Eating Questionnaire (TFEQ) along with six days of self-reported dietary intake and anthropometrics. Between March 2007 and May 2009, a total of 32 Canadian athletes with a SCI (n = 24 men, n = 8 women) completed the study. The TFEQ scales showed a cognitive dietary restraint score of 10.8 ± 4.7, disinhibition score of 2.8 ± 1.8 and hunger score of 3.1 ± 2.2. When the group was split into high and low restraint groups using a median of 11.5, no differences were detected in any of the absolute parameters of reported dietary intake although the higher restraint group had protein intakes account for a greater proportion of total energy. Those with higher restraint scores also had a relatively higher disinhibition score. While the cognitive dietary restraint scores for the women were similar to other able-bodied populations, the scores for men were higher than population norms from other studies. The scores for disinhibition and hunger were lower than reported ranges from able-bodied subjects. These athletes may be actively monitoring or limiting dietary intake to avoid the high prevalence of obesity associated with a SCI or perhaps to maintain an ideal body composition for their sport performance. © 2011 Elsevier Ltd.


McPherson G.,University of British Columbia | Jefferson R.,British Columbias Childrens Hospital | Kissoon N.,British Columbias Childrens Hospital | Kwong L.,British Columbias Childrens Hospital | Rasmussen K.,British Columbias Childrens Hospital
Pediatric Critical Care Medicine | Year: 2011

Background: Inclusion of parents on interprofessional patient rounds is increasingly recognized as a parental right and as a marker of quality care in pediatric intensive care units. Creating policies and practices that welcome parents and their contributions into patient rounds has proven challenging in many settings. Objective: To develop a detailed understanding of the physical, professional and interpersonal contexts of one pediatric intensive care unit in order to develop a feasible, relevant and sustainable approach to parental inclusion on rounds. Design: Prospective qualitative and descriptive exploratory survey. Setting: A pediatric Intensive care unit at a tertiary care children's hospital. Subjects: Physicians, nurses and other health care professionals working in our pediatric intensive care unit and to all parents who had children admitted to the unit during a one month period. Interventions: None. Measurement and Main Results: Surveys were distributed to physicians, nurses and other health care professionals working in our pediatric intensive care unit and to all parents who had children admitted to the unit during a 1-month period. In addition in-depth interviews were conducted with a subset of 13 healthcare providers. Descriptive statistics were used to report survey results. Transcripts of the interviews and the qualitative comments provided on the surveys were analyzed according to principles of interpretive description. Parents indicated a strong desire to participate in pediatric intensive care unit rounds, while healthcare providers varied in the extent to which they believed parents' participation would achieve the intended goals. Key considerations that need to be addressed for successful practice change to incorporate parents on rounds include: working on consistent and reliable communication, addressing the issues of confidentiality, time constraints, and teaching during rounds, and attention to the role of parents during rounds and health care professionals' facilitation of that role. Conclusion: The participants in this study believed that parents' participation on rounds is an important consideration. For inclusion of parents to be effective and sustainable, policy and practice change in this direction requires measures to recognize parents as important contributors to pediatric intensive care unit rounds while accounting for the complex responsibility of healthcare providers in the physical and social space of the pediatric intensive care unit. © 2011 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.


Lauder G.R.,British Columbias Childrens Hospital
Paediatric Anaesthesia | Year: 2015

Inhalational anesthesia has dominated the practice of pediatric anesthesia. However, as the introduction of agents such as propofol, short-acting opioids, midazolam, and dexmedetomidine a monumental change has occurred. With increasing use, the overwhelming advantages of total intravenous anesthesia (TIVA) have emerged and driven change in practice. These advantages, outlined in this review, will justify why TIVA will supercede inhalational anesthesia in future pediatric anesthetic practice. © 2014 John Wiley & Sons Ltd.


Breugem C.C.,University Utrecht | Courtemanche D.J.,British Columbias Childrens Hospital
Cleft Palate-Craniofacial Journal | Year: 2010

Objective: To gather evidence surrounding the confusion in the classification of Robin sequence and inform those who have the power to make the changes in defining this symptom complex. Method: A questionnaire was sent to all participating cleft palate teams (N = 204) of the American Cleft Palate-Craniofacial Association. The questionnaire identified the precise, different characteristics for diagnosing Robin sequence and evaluated whether the difference between a retrognathia and micrognathia influenced the diagnosis process. We subsequently also investigated whether the cleft type (i.e., U-shaped versus V-shaped) had any influence in the decisionmaking process. A PubMed literature review of the 50 most recent manuscripts about Robin sequence was evaluated also. Results: Seventy-three questionnaires were received. This 35% response rate revealed 14 different definitions of Robin sequence. A PubMed literature review of 50 consecutive manuscripts revealed 15 different descriptions. Conclusion: This study confirms that nosologic confusion is widespread with regard to defining Robin sequence. This has implications for evaluating Robin sequence, giving advice about the prognosis and genetic counseling, and refining treatment options.


McDougall C.M.,British Columbias Childrens Hospital | Adderley R.J.,British Columbias Childrens Hospital | Wensley D.F.,British Columbias Childrens Hospital | Seear M.D.,British Columbias Childrens Hospital
Archives of Disease in Childhood | Year: 2013

Background Cross-sectional studies have suggested a rapid expansion in paediatric long-term ventilation (LTV) over the last 20 years but information on longitudinal trends is limited. Methods Data were collected prospectively on all patients receiving LTV over a 15-year period (1.1.95-31.12.09) in a single regional referral centre. Results 144 children commenced LTV during the 15- year period. The incidence of LTV increased significantly over time, with an accompanying 10-fold increase in prevalence due to a significant increase in institution of non-invasive ventilation (NIV). There was no significant increase in invasive ventilation. 5-year survival was 94% overall and was significantly higher for patients on NIV (97%) than invasively ventilated patients (84%). 10-year survival was 91% overall. Although some children were able to discontinue respiratory support (21% at 5 years and 42% at 10 years), the number of patients transitioned to adult services increased significantly over time (26% of total cohort). Patients with neuromuscular disease were less likely to discontinue support than other patients. Conclusions The paediatric LTV population has expanded significantly over 15 years. Future planning of paediatric hospital and community services, as well as adult services, must take into account the needs of this growing population.

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