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Keene D.,Childrens Hospital of Eastern Ontario | Carret A.-S.,Hospital Ste Justine | Fryer C.,BC Childrens Hospital | Brossard J.,Université de Sherbrooke | And 9 more authors.
Child's Nervous System | Year: 2011

Background: Choroid plexus tumors (CPT) are rare pediatric tumors. A population-based study on choroid plexus carcinoma (CPC) and choroid plexus papilloma (CPP) was carried out to describe the incidence, demographic, and outcome data and to identify potential prognostic factors. Methods: The CPT population from the Canadian databank of CNS tumor in children ≤36 months diagnosed between 1990 and 2005 was reviewed Results: Out of the 579 reported cases of CNS tumors, 37 were CPT. The annual age-adjusted incidence rate was 0.22 + 0.12 (95% CI 0.16-0.28)/100,000 children <3 years. There were 21 (56.7%) CPP and 16 (43.3.5%) CPC. Twenty patients (54%) were males. Median age at diagnosis was 7 months(range 0-30). Ten patients(62.5%) with CPC and one with CPP were metastatic at diagnosis. Twenty patients with CPP (95%) had a complete resection, whereas 6/16 CPC (37.5%) achieved a resection >90%. Fourteen CPC patients received adjuvant chemotherapy. None of the 37 patients received adjuvant radiation. At completion of survey, all CPP and five CPC were alive. Median survival time for CPC patients was 15 months (0-120). One death was related to intraoperative hemorrhage, another to chemotherapy-induced toxicity, and one to secondary AML. Age at diagnosis, degree of resection and metastatic status were not significant prognostic factors for CPC. Conclusion: By contrast to CPC, CPP have an excellent prognosis following surgery alone. Survival of CPC remains poor. However, these data may suggest adjuvant chemotherapy can alter the aggressive natural history of CPC. As with other rare CNS tumors, international collaboration is required to identify optimal therapy. © 2010 Springer-Verlag.

Chitlur M.,Wayne State University | Sorensen B.,St Thomas Hospital | Rivard G.E.,Hospital Ste Justine | Young G.,Childrens Hospital Los Angeles | And 6 more authors.
Haemophilia | Year: 2011

Laboratory evaluation of bleeding disorders has been performed with the standard clotting assays such as the PT and PTT for several decades. Our improved understanding of the process of blood coagulation has now revealed the important role played by the cellular elements such as platelets, monocytes and red blood cells. The need for a test that can assess clotting in a more 'global' manner, beyond the initiation of clot formation, has led to greater interest in assays such as thrombin generation and thromboelastography. Even though there are several publications using thromboelastography it remains a research tool as the methodology is not standardized. In an attempt to show reproducibility and consistency using thromboelastography, a group of investigators from different countries joined hands to form the TEG-ROTEM Working Group. Two studies were performed using PRP and FVIII deficient plasma and an intrinsic pathway activator. This article summarizes the results of the first international effort at standardization of thromboelastography. Both of the instruments using this technology (TEG® and ROTEM®) were used. Nine laboratories from countries around the globe participated in this effort. The results showed a significant inter-laboratory variance with CV's greater than 10%. Although these results were not satisfactory, this has been the first effort to standardize this methodology and significant work remains to be done to improve reliability and reproducibility. These studies were performed on PRP and the results may be more reliable when preformed on whole blood samples. We believe that it is important to continue this work so that we may investigate the usefulness and potential applications of thromboelastography in the evaluation of bleeding and thrombosis. © 2011 Blackwell Publishing Ltd.

Burjonrappa S.,New York Medical College | Thiboutot E.,Hospital Ste Justine | Castilloux J.,Hospital Ste Justine | St-Vil D.,Hospital Ste Justine
Journal of Pediatric Surgery | Year: 2010

Purpose: The purpose of was to study the short- and long-term outcomes in the management of isolated esophageal atresia with different operative strategies. Methods: All patients undergoing type A atresia repair over a 15-year period were included. Demographic data, birth weight, gestational age, incidence of associated anomalies, management, and long-term outcomes were studied. Results: Fifteen patients with type A atresia (9 male) were treated in the study period. The mean gestational age was 35.5 weeks (range, 27-39 weeks), and the mean birth weight was 2179 g (range, 670-3520 g). Eight babies had associated anomalies. Thirteen patients underwent gastrostomy as the initial procedure, and 2 underwent the Foker procedure. In the delayed management group, 9 patients underwent primary anastomosis, with 2 patients needing proximal pouch myotomy. Two patients underwent a Collis gastroplasty. Two patients underwent a cervical esophagostomy and a gastric tube replacement at 4 months and 1 year, respectively. Eight patients (60%) in this group had anastomotic leaks. All patients are currently on prokinetics and proton pump inhibitors. Seven required antireflux surgery. The median length of hospital admission was 4 months (range, 3-19 months). The native esophagus was preserved in 13 (85%) of 15 babies. All patients are alive, and 14 of 15 are capable of feeding orally. Conclusions: Type A esophageal atresia continues to be associated with significant morbidity despite advances in surgical technique and intensive care. © 2010 Elsevier Inc. All rights reserved.

Rae C.,McMaster University | Furlong W.,McMaster University | Furlong W.,Health Utilities Inc | Jankovic M.,Azienda Ospedaliera | And 8 more authors.
European Journal of Cancer Care | Year: 2014

Berlin-Frankfurt-Munster (BFM) and Dana-Farber Cancer Institute (DFCI) consortia's treatment strategies for acute lymphoblastic leukaemia (ALL) in children are widely used. We compared the health effects and monetary costs of hospital treatments for these two strategies. Parents of children treated at seven centres in Canada, Italy and the USA completed health-related quality of life (HRQL) assessments during four active treatment phases and at 2 years after treatment. Mean HRQL scores were used to calculate quality-adjusted life years (QALYs) for a period of 5 years following diagnosis. Total costs of treatment were determined from variables in administrative databases in a universally accessible and publicly funded healthcare system. Valid HRQL assessments (n=1200) were collected for 307 BFM and 317 DFCI patients, with costs measured for 66 BFM and 28 DFCI patients. QALYs per patient were <1.0% greater for BFM than DFCI. Median HRQL scores revealed no difference in QALYs. The difference in mean total costs for BFM (US$88480) and DFCI (US$93026) was not significant (P=0.600). This study provides no evidence of superiority for one treatment strategy over the other. Current BFM or DFCI strategies should represent conventional management for the next economic evaluation of treatments for ALL in childhood. © 2014 John Wiley & Sons Ltd.

Athale U.,Hamilton Health Sciences | Moghrabi A.,Hospital Ste Justine | Nayiager T.,Hamilton Health Sciences | Delva Y.-L.,Hospital Ste Justine | And 4 more authors.
Pediatric Blood and Cancer | Year: 2010

Background. The pathogenesis and the impact of therapy on thrombin activation in children with acute lymphoblastic leukemia (ALL) are unknown. Steroids may contribute to ALL-associated thrombosis. We explored the hemostatic effects of methylprednisolone monotherapy (MpMT) (32 mg/m2/day IV x 3 days) in children with newly diagnosed ALL. Methods. Children (>1 to ≤ 18 years of age) enrolled on DFCI ALL05-01 protocol (n=30; mean age 6.3 years), without prior steroid therapy, were eligible for study. Overnight fasting pre- and post-MpMT samples were analyzed for coagulation factors [FVIII:C, von Willebrand factor antigen (vWF:Ag) and fibrinogen] and parameters of thrombin generation [prothrombin fragments 1.2 (F1.2), thrombin-antithrombin complex (TAT), and D-dimer]. Results. At diagnosis F1.2 (1.5 nmol/L), TAT (10.9 μg/L), and D-dimers (2,766 ng/ml) levels were increased indicating endogenous thrombin activation. Patients with peripheral blasts (n=17) had higher levels of vWF:Ag (1.89 vs. 1.14 P=0.001), TAT (15.39 vs. 5.02 P=0.038), and D-dimer (3,640 vs. 1,623 P=0.019) compared to those without peripheral blasts. Following MpMT the blast count decreased significantly from 24% to 3.5% (P<0.001) with reduction in level of vWF:Ag (1.5, P<0.01), TAT (8.9, P=0.42), and D-dimer (P=0.018) despite 30% increase in FVIII:C levels (P=0.005). However, patients without peripheral blasts had no significant change in vWF:Ag levels (1.14 vs. 1.25; P=0.142) and had an increase in thrombin generation parameters. Conclusions. We postulate that peripheral blasts through endothelial activation stimulate vWF:Ag production/secretion causing coagulation activation. Methylprednisolone therapy reduces the blast count and indirectly suppresses the coagulation activation. Future studies are required to confirm these findings. © 2010 Wiley-Liss, Inc.

Daymont C.,University of Manitoba | Daymont C.,Childrens Hospital Research Institute Of Manitoba | Klassen T.P.,University of Manitoba | Klassen T.P.,Childrens Hospital Research Institute Of Manitoba | And 15 more authors.
Canadian Journal of Emergency Medicine | Year: 2015

Objective: To evaluate the accuracy of physician estimates of the probability of intracranial injury in children with minor head trauma. Methods: This is a subanalysis of a large prospective multicentre cohort study performed from July 2001 to November2005. During data collection for the derivation of a clinical prediction rule for children with minor head trauma, physicians indicated their estimate of the probability of brain injury visible on computed tomography (P-Injury) and the probability of injury requiring intervention (P-Intervention) by choosing one of the following options: 0%, 1%, 2%, 3%, 4%, 5%, 10%, 20%, 30%, 40%, 50%, 75%, 90%, and 100%. We compared observed frequencies to expected frequencies of injury using Pearson’s χ2-test in analyses stratified by the level of each type of predicted probability and by year of age. Results: In 3771 eligible subjects, the mean predicted risk was 4.6% (P-Injury) and 1.4% (P-Intervention). The observed frequency of injury was 4.1% (any injury) and 0.6% (intervention). For all levels of P-Injury from 1% to 40%, the observed frequency of injury was consistent with the expected frequency. The observed frequencies for the 50%, 75%, and 90% levels were lower than expected (p<0.05). For estimates of P-Intervention, the observed frequency was consistently higher than the expected frequency. Physicians underestimated risk for infants (mean P-Intervention 6.2%, actual risk 12.3%, p<0.001). Conclusions: Physician estimates of probability of any brain injury in children were collectively accurate for children with low and moderate degrees of predicted risk. Risk was underestimated in infants. © Canadian Association of Emergency Physicians.

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