Province-wide Biliary Atresia Home Screening Program in British Columbia: Evaluation of First 2 Years.

BACKGROUND AND OBJECTIVES: Biliary atresia (BA), a rare newborn liver disease, is the leading cause of liver-related death in children. Early disease recognition and timely surgical Kasai hepatoportoenterostomy (KP) offers long-term survival without liver transplant. Universal BA screening in Taiwan using infant stool color cards (ISCCs) has proven effectiveness. We report our experience with infant stool color card (ISCC) BA screening in a province-wide program in British Columbia (BC). The objective of this study is to assess program performance and cost from launch April 1, 2014 to March 31, 2016. METHODS: ISCCs distributed to families upon maternity ward discharge. Parents were instructed to monitor their infant's stool color for 1 month and contacted the screening center with concerns. The number of live births, ISCC distribution, BA cases, and costs were recorded. Cases with Program screen success had both acholic stool recognition (ISCC screen success) and timely referral for BA. RESULTS: All 126 maternity units received ISCCs. Of 87,583 live births there were 6 BA cases. Of the 5 cases with ISCC Screen Success 3 had Program Screen Success. The median KP age in the program screen success and failure groups was 49 (42-52) and 116 (49-184) days, respectively. Program sensitivity was 50%, specificity 99%, positive predictive value 4%, and negative predictive value 99%. A random sample of 1054 charts at BC Children's Hospital found an ISCC distribution rate of 94%. After a phase-in period, the annual program cost was $30,033.82, and the ISCC cost per birth was $0.68. CONCLUSIONS: The screening program has high specificity and distribution with low cost. Successful program case identification had earlier age at KP. Program modifications aim to improve sensitivity. Longer-term studies will determine program impact on health outcomes.

The Canadian Biliary Atresia Registry: Improving the care of Canadian infants with biliary atresia.

Biliary atresia is the most common cause of end-stage liver disease and liver cirrhosis in children, and the leading indication for liver transplantation in the paediatric population. There is no cure for biliary atresia; however, timely diagnosis and early infant age at surgical intervention using the Kasai portoenterostomy optimize the prognosis. Late referral is a significant problem in Canada and elsewhere. There is also a lack of standardized care practices among treating centres in this country. Biliary atresia registries currently exist across Europe, Asia and the United States. They have provided important evidence-based information to initiate changes to biliary atresia care in their countries with improvements in outcome. The Canadian Biliary Atresia Registry was initiated in 2013 for the purpose of identifying best standards of care, enhancing public education, facilitating knowledge translation and advocating for novel national public health policy programs to improve the outcomes of Canadian infants with biliary atresia.

L'atrésie des voies biliaires est la principale cause d'insuffisance hépatique terminale et de cirrhose chez les enfants, et la première indication de transplantation du foie au sein de la population d'âge pédiatrique. Aucun traitement ne guérit l'atrésie des voies biliaires, mais un diagnostic rapide et le jeune âge du nourrisson au moment de l'intervention chirurgicale par hépato-porto-entérostomie de Kasai optimisent le pronostic. L'orientation tardive vers un spécialiste constitue un problème important au Canada et ailleurs. Par ailleurs, il n'existe pas de protocole de soins standardisés dans les centres de traitement du pays. On trouve des registres d'atrésie des voies biliaires en Europe, en Asie et aux États-Unis, lesquels ont fourni de l'information importante fondée sur des données probantes pour susciter des changements aux soins de cette affection dans ces pays et favoriser une amélioration des résultats. Le Registre canadien d'atrésie des voies biliaires a été créé en 2013 pour définir les meilleures normes de soins, améliorer l'éducation publique, favoriser le transfert des connaissances et prôner de nouveaux programmes de politiques en santé publique en vue d'améliorer le sort des nourrissons canadiens présentant une atrésie des voies biliaires.

Don't Throw Your Heart Away: Increased Transparency of Donor Utilization Practices in Transplant Center Report Cards Alters How Center Performance Is Evaluated.

BACKGROUND: Publicly available report cards for transplant centers emphasize posttransplant survival and obscure the fact that some centers reject many of the donor organs they are offered (reflecting a conservative donor acceptance strategy), while others accept a broader range of donor offers (reflecting an open donor acceptance strategy). OBJECTIVE: We assessed how the provision of salient information about donor acceptance practices and waitlist survival rates affected evaluation judgments of hospital report cards given by laypeople and medical trainees. METHODS: We tested 5 different report card formats across 4 online randomized experiments (n1 = 1,003, n2 = 105, n3 = 123, n4 = 807) in the same hypothetical decision. The primary outcome variable was a binary choice between transplant hospitals (one with an open donor acceptance strategy and the other with a conservative donor acceptance strategy). RESULTS: Report cards featuring salient information about donor organ utilization rates (transplant outcomes categorized by quality of donor offers accepted) or overall survival rates (outcomes from both waitlist and transplanted patients) led lay participants (studies 1, 3, and 4) and medical trainees (study 2) to evaluate transplant centers with open donor acceptance strategies more favorably than centers with conservative strategies. LIMITATIONS: Due to the nature of the decision, a hypothetical scenario was necessary for both ethical and practical reasons. Results may not generalize to transplant clinicians or patients faced with the decision of where to join the transplant waitlist. CONCLUSIONS: These findings suggest that performance evaluations for transplant centers may vary significantly based not only on what outcome information is presented in report cards but also how the information is displayed.

The Canadian Pediatric Surgery Network (CAPSNet): Lessons Learned from a National Registry Devoted to the Study of Congenital Diaphragmatic Hernia and Gastroschisis.

The Canadian Pediatric Surgery Network (CAPSNet) was created in 2005 by a geographically representative, multidisciplinary group of clinicians and researchers with the intent of establishing a national research registry for gastroschisis (GS) and congenital diaphragmatic hernia (CDH). Since then, CAPSNet has used this registry and its 16-center network to make contributions to the knowledge base informing best practices for GS and CDH care. More recently, CAPSNet has expanded its focus to include quality assurance and improvement at each of its sites, by issuing a benchmarked outcomes "report card" with its annual report. Finally, a major objective of CAPSNet has been to establish and adopt standardized, evidence-based practice guidelines for GS and CDH across all Canadian perinatal centers.