Colonoscopy Quality Assurance and Maintenance of Competency Among Pediatric Gastroenterology Staff Members: A Canadian Center Experience.

Introduction Colonoscopy quality indicators and maintenance of competency skills are relatively well established in the adult literature as compared to the pediatric gastroenterology. One of the suggested quality assurance measures is cecal intubation rate, which is suggested to be >90% in all colonoscopies as per American Society of Gastrointestinal Endoscopy (ASGE) guidelines. Terminal ileum (TI) intubations are essentially required for diagnostic reasons in pediatric colonoscopies as compared to the screening reasons in adults. Maintenance of competency in pediatric colonoscopies has been described in the literature but in smaller studies contrary to the adult ones. The aims of this study are to compare our center's individual and group cecal intubation rates and compare it with the published literature, assess the group's terminal ileal intubation rates in comparison with the published literature, assess the most common reasons for failure to intubate the cecum and/or terminal ileum, and to assess whether the presence of a trainee affects the intubation rates and the duration of the procedure. Methods A retrospective chart review was performed on all pediatric patients (0-18 years). Colonoscopies performed over a two-year period at our single center were included in the study. Patients scheduled for sigmoidoscopy and with altered anatomy of their colon were excluded from the study. The endoscopy and pathology reports were reviewed to ascertain whether the cecum and TI were reached. Quality of bowel preparation and any other stated reasons for incompletion were obtained. Clinical charts were reviewed to obtain indication for colonoscopy. Skin-to-skin time, which is the time from starting to the finishing of the procedure, was recorded for each procedure. Results A total of 391 colonoscopies were performed during the two-year study period by six gastroenterologists. The number of colonoscopies per staff ranged from 57 to 89 procedures. The overall cecal intubation rate was observed to be 98.5% (range: 95.9%-98.9%). TI intubation rate was lower at a rate of 83.1% (range: 63.3%-92.1%). The main stated reason for the inability to attain cecum/TI was technical difficulty and poor bowel prep. Daytime colonoscopies were shorter (39.5 minutes vs 50.3 minutes) compared to after-hours ones and had a higher TI intubation rate (84.5% vs 62.5%). No complications were encountered in the procedures. Conclusion Despite relatively low volumes, cecal intubation rates are very high, exceeding suggested standards. TI intubation rates were low, and there was noted to be a high degree of variability. However, multicentric collaborative evaluations are required over a longer period of time to establish relevant parameters for quality assurance and competency in pediatric endoscopy.

The Experience of a Gluten-free Diet in Children with Type 1 Diabetes and Celiac Disease.

OBJECTIVE: This study examined overall self-reported adherence to gluten-free diet (GFD) in children with type 1 diabetes and celiac disease (T1DCD) compared to children with celiac disease (CD). Secondary objectives included gaining insight into self-reported symptoms, barriers to adherence, and experience of a GFD between groups. METHODS: Children <18 years old who had been seen at BC Children's Hospital for T1DCD or CD were invited to participate in a web-based questionnaire and medical record review. RESULTS: A total of 26 children with T1DCD and 46 children with CD participated in the study. The groups' demographics and symptoms of CD were similar; however, a greater proportion of those with T1DCD were asymptomatic at diagnosis (T1DCD 27%; CD 7%; P = 0.016). Overall adherence to a GFD was high in both groups (T1DCD 92%; CD 100%; P = 0.38) but those with T1DCD reported a significantly less positive effect on their health (P = 0.006) and a significantly greater negative effect on activities from a GFD (P = 0.03). Children with T1DCD reported more significant barriers to eating gluten-free at home and at restaurants, specifically with social pressure, cost and taste compared to those with CD only. CONCLUSION: Children with T1DCD face specific barriers in adherence that are more impactful compared with children living with CD. These children are more often asymptomatic at diagnosis, and they go on to experience different impacts of a GFD spanning across home and social settings. Given the complexity of having a dual diagnosis, CD care should be tailored specifically to children living with T1DCD.

Role of transient elastography and APRI in the assessment of pediatric cystic fibrosis liver disease.

Background: Diagnosis and monitoring of cystic fibrosis liver disease (CFLD) is challenging. Transient elastography (TE) is a rapid, non-invasive method for assessing liver fibrosis. Its role in detecting fibrosis in CFLD has only begun to be explored. The aspartate aminotransferase to platelet ratio index (APRI) has been validated as a predictor of hepatic fibrosis in other chronic liver diseases. The purpose of this study was to assess the utility of APRI and TE in identifying liver fibrosis in pediatric CF patients. Methods: Patients aged 2-18 years were recruited from the British Columbia Children's Hospital CF clinic. Patients were determined to have CFLD using standard criteria. Charts were reviewed, and each patient underwent TE. Results: Of the 55 patients included in the study (50.9% male, mean age 11.6 y), 22 (40%) had CFLD. All mean liver enzymes were higher in the CFLD group, notably alanine transaminase (p = 0.031). Mean liver stiffness (LS) and APRI were also higher in the CFLD group (LS: 5.9 versus 4.5 kPa, p = 0.015; APRI: 0.40 versus 0.32, p = 0.119). Linear regression showed a mild positive association between the two (r 2 = 0.386). Conclusions: TE values were higher among CFLD patients and correlated with APRI values, suggesting that these tools may have clinical applications for identifying and following this population. Further research is needed on a larger scale to determine the relative value and clinical utility of TE and APRI among patients with CFLD.

Accelerated Idioventricular Rhythm in Inflammatory Bowel Disease: When the Gut Takes Charge.

Accelerated idioventricular rhythm (AIVR) is an uncommon but benign rhythm, seen most commonly in children. It is associated with reperfusion injury after myocardial infarction in adults. In children, it is usually seen as an idiopathic finding in the absence of heart disease. We present a case of AIVR in an adolescent associated with acute presentation of inflammatory bowel disease. Prompt treatment of the systemic inflammation led to the remission of both inflammatory bowel disease and AIVR. This report emphasizes the diverse causes of AIVR in children and our limited understanding of its pathophysiology. Treatment of the underlying condition resolved the arrhythmia.

Le rythme idioventriculaire accéléré (RIVA) est un rythme rarement rencontré mais bénin, observé le plus souvent chez les enfants. Il est associé à des lésions de reperfusion après un infarctus du myocarde chez l'adulte. Chez l'enfant, il est généralement catégorisé comme idiopathique en absence de maladie cardiaque. Nous présentons le cas d'un adolescent ayant un RIVA associé à une forme aiguë de maladie intestinale inflammatoire. Un traitement rapide de l'inflammation systémique a conduit à la rémission de la maladie intestinale inflammatoire et du RIVA. Ce rapport met en lumière les diverses causes du RIVA chez les enfants et notre compréhension limitée de sa physiopathologie. Le traitement de la condition sous-jacente a permis de résoudre l'arythmie.

Concomitant Therapy with Immunomodulator Enhances Infliximab Durability in Pediatric Inflammatory Bowel Disease.

BACKGROUND: Data on long-term durability of infliximab (IFX) and outcomes of concomitant therapy with immunomodulator in pediatric inflammatory bowel disease are limited. METHODS: Children with inflammatory bowel disease who received IFX ± immunomodulator were retrospectively reviewed. Predictors of induction response were assessed using a binary logistic regression model and long-term outcomes evaluated by Cox proportional hazards model. Propensity score matching examined long-term efficacy of concomitant therapy in patients with Crohn's disease (CD). RESULTS: Among 148 patients (113 CD, 35 ulcerative colitis; median age at IFX initiation 14.09 years [interquartile range 12.16-15.65]), 91% experienced response to induction therapy; patients with CD were more likely to respond (95% versus 77%, odds ratio = 2.63, 95% confidence interval, 1.01-6.85, P = 0.048). Despite dose optimization, secondary loss of response occurred at a rate of 9.01% and 8.33% per year for patients with CD and ulcerative colitis, respectively. A Cox proportional hazards model showed that concomitant therapy >6 months significantly lowered the risk of secondary loss of response in CD (hazard ratio = 0.39, 95% confidence interval, 0.17-0.88, P = 0.025). The same trend was observed in ulcerative colitis but did not reach significance. A higher proportion of patients on IFX monotherapy stopped IFX because of loss of response or infusion reactions (55% versus 21%, P < 0.001). Propensity score analysis of patients with CD showed significantly higher steroid-free remission rates for concomitant versus monotherapy at 1 year (78% versus 54%, P = 0.020) and 2 years (68% versus 46%, P = 0.044), and durability of response (P = 0.022). CONCLUSIONS: These data demonstrate sustained efficacy of IFX in a cohort of pediatric patients with inflammatory bowel disease with durability of response enhanced by concomitant therapy.

Unsuccessful Redo MitraClip Procedure Leads to Acute Right Ventricular Failure in a Patient With Homozygous Familial Hypercholesterolemia and a Preexisting Atrial Septal Defect.

The MitraClip procedure is an emerging endovascular technique for treating mitral regurgitation and an attractive alternative for patients who are at high risk for open heart mitral valve repair or replacement. We present the case of a failed redo MitraClip procedure that led to acute right ventricular failure in a patient with homozygous familial hypercholesterolemia and a preexisting secundum atrial septal defect. We highlight the sequelae of the failed redo MitraClip procedure and the anesthetic challenges associated with the emergent redo sternotomy and cardiopulmonary bypass procedure required to replace the mitral valve and repair the tricuspid valve and atrial septal defect.

25-Hydroxyvitamin D concentrations in children with Crohn's disease supplemented with either 2000 or 400 IU daily for 6 months: a randomized controlled study.

OBJECTIVES: To assess vitamin D status of pediatric patients with Crohn's disease (CD) and to compare their serum 25-hydroxyvitamin D (s-25OHD) with established cutoffs and assess whether 6 months of supplementation with 2000 IU/d, vs 400 IU/d, would reduce the group prevalence of vitamin D below these cutoffs. STUDY DESIGN: Subjects 8-18 years (n = 83) with quiescent CD were randomized to either 400 or 2000 IU vitamin D3/d for 6 months. RESULTS: Baseline mean ± SD s-25OHD was 24 ± 8 ng/mL; 13 subjects (16%) had an s-25OHD <16 ng/mL, 27 (33%) < 20 ng/mL, and 65 (79%) < 30 ng/mL. There was no significant difference between groups in achieving the cutoffs of 16 ng/mL or 20 ng/mL at 6 months; however, only 35% of the 400 IU group achieved the greater cutoff of 30 ng/mL compared with 74% in the 2000 IU group (P < .001). Baseline adjusted mean s-25OHD concentrations at 6 months were 9.6 ng/mL (95% CI 6.0-13.2, P < .001) greater in the 2000 IU than the 400 IU group. Disease activity was not affected by supplement dose. Few subjects exceeded safety marker cutoffs, and this did not differ by dose. CONCLUSIONS: At baseline, a high proportion of patients had a mean s-25OHD >20 ng/mL. 2000 IU vitamin D3/d is more effective in raising s-25OHD concentrations to > 30 ng/mL in children with CD than 400 IU/d, but both treatments were equally effective at achieving 16 or 20 ng/mL.

Does adjuvant steroid therapy post-Kasai portoenterostomy improve outcome of biliary atresia? Systematic review and meta-analysis.

BACKGROUND: The role of adjuvant steroid therapy in the postoperative management of patients with biliary atresia (BA) is unclear. OBJECTIVE: To systematically review the literature and perform a meta-analysis to determine the efficacy of adjuvant steroid therapy post-Kasai portoenterostomy (KP) on BA outcome. METHODS: A systematic review and meta-analysis of randomized trials and/or observational studies that examined the role of steroids on BA outcomes published between January 1969 and June 2010 was conducted. Studies were identified using the Medline, PubMed, EMBASE and Cochrane databases. RESULTS: Sixteen observational studies and one randomized controlled trial (RCT) were found. Four of the 16 observational studies (160 participants) and the RCT (73 participants) met the entry criteria and were eligible to be included in the analysis. There was no statistically significant difference in the effect of steroids either on normalizing serum bilirubin levels at six months (pooled OR 1.48 [95% CI 0.67 to 3.28]) or in delaying the need for early liver transplantation (within the first year post-KP (pooled OR 0.59 [95% CI 0.21 to 1.72]). CONCLUSION: The present meta-analysis did not find a significant effect of steroid over standard therapy, either in normalizing serum bilirubin levels at six months or at delaying the need for early liver transplantation post-KP. RCT studies of sufficient size and comprehensive design using high-dose steroids are needed to determine the effectiveness of steroids on the short and intermediate post-KP outcomes for BA patients.

Biliary atresia with associated structural malformations in Canadian infants.

BACKGROUND: Biliary atresia (BA) is associated with extrahepatic congenital malformations in a minority of affected infants. The term commonly applied to this subgroup is 'BASM' for biliary atresia splenic malformation syndrome, as spleen abnormalities are prominent. AIMS AND METHODS: To examine clinical outcome in Canadian BA patients with extrahepatic congenital malformations in the Canada-wide BA database of patients born between 1985 and 2002, and additionally, to recharacterized the syndrome. Patients had ≥1 of the following: a/polysplenia, abnormal abdominal situs, intestinal malrotation, abdominal vascular anomaly or congenital heart disease. RESULTS: Among 328 BA patients, 44 (13%) had associated congenital abnormalities. Intra-abdominal anomalies included polysplenia (n=25), abnormal abdominal situs (n=9), intestinal malrotation (n=19), portal vein anomaly (n=12), hepatic artery anomaly (n=3) and inferior vena cava interruption (n=20). Twenty-six patients had cardiac malformations including pulmonary stenosis (n=11), ventricular septal defect (n=10), atrial septal defect (n=7), total anomalous pulmonary venous return (n=3), double outlet right ventricle (n=3), tetralogy of Fallot (n=2), atrioventricular canal (n=2), dextrocardia (n=2), bicuspid aortic valve (n=2), hypoplastic left heart (n=1) and partial anomalous pulmonary venous return (n=1). Age at Kasai operation, performance of liver transplant, overall survival, post-Kasai native liver survival and transplant survival were comparable to isolated BA. Presence of polysplenia or complex cardiac disease did not reduce post-Kasai native liver survival. Three patients had ≥2 typical abnormalities without polysplenia: thus, splenic malformations are not essential to this BA subgroup. Hierarchical cluster analysis demonstrated characteristic abnormalities grouped in a multiplicity of combinations, consistent with a spectrum of defective lateralization. CONCLUSION: We suggest that the acronym 'BASM' be redefined as 'biliary atresia structural malformation'.

Childhood obesity and outcomes after bone marrow transplantation for patients with severe aplastic anemia.

The prevalence of obesity in the pediatric population has increased in the last 2 decades and represents a serious health concern, with potential impact on outcomes of hematopoietic cell transplantation (HCT). We studied the effect of weight by age-adjusted body mass index (BMI) percentile in 1,281 pediatric patients (age 2-19 years) with severe aplastic anemia who underwent HCT between 1990 and 2005. The study population was divided into 5 weight groups-underweight, risk of underweight, normal BMI range, risk of overweight, and overweight-according to age-adjusted BMI percentiles. Cox proportional hazards regression models for survival and acute graft-versus-host disease (aGVHD), performed using weight groups as the main effect and the normal BMI range (26th-75th percentile) as the baseline comparison, found higher mortality among overweight children (>95th percentile adjusted for age). Weight at transplantation did not increase the adjusted risk of grade III-IV aGVHD. The 1-year and 2-year overall survival rates were 60% and 59% for overweight children, compared with >70% in children with lower BMI at both time points (P < .001). Other significant factors associated with survival included race and region, donor type, conditioning regimens in related donor transplants, performance score, and year of transplantation. In conclusion, overweight children with aplastic anemia have worse outcomes after HCT. The impact of obesity on survival outcomes in children should be discussed during pretransplantation counseling.

Biliary atresia in Canada: the effect of centre caseload experience on outcome.

OBJECTIVES: : Biliary atresia (BA) is a leading cause of end-stage paediatric liver disease. Standard BA treatment is sequential surgery with an initial Kasai procedure (KP) followed by liver transplant (LT) for patients who progress to liver failure. A key determinant for the post-KP patient survival with their native liver is patient age at KP (older age, poorer outcome). Recently, European studies have reported that caseload experience influences prognosis with centres managing <5 cases per year (UK) or <2 cases per year (France) having worse survival. Our study investigates the effect of caseload experience on outcomes of Canadian patients with BA. PATIENTS AND METHODS: : A national database of cases with BA, born from 1992 to 2002, was examined. Patients were grouped according to treatment centre size (A: on average <1 case per year; B: 1 to 3 cases per year; and C: >3 cases per year). Overall patient, post-KP native liver, and LT survivals were compared between centres. Outcome parameters were reevaluated for patients grouped by the largest Canadian centre (>5 cases per year) and all other centres (<5 cases per year). RESULTS: : Two-hundred thirty patients were identified among 6 group A, 4 group B, and 2 group C centres. The overall median age at KP was 64 days. There were no significant differences in patient, post-KP native liver, or LT survivals between the sized centres and even the largest centre, with the overall 4-year post-KP native liver survival being 39%. CONCLUSIONS: : Caseload experience does not importantly affect the outcomes for Canadian children with BA. Although outcomes in Canada are comparable to those elsewhere, national policies directed towards timely referral and earlier age at KP rather than centralisation of care are needed.

Biliary atresia: the Canadian experience.

OBJECTIVE: To determine the outcomes of Canadian children with biliary atresia. STUDY DESIGN: Health records of infants born in Canada between January 1, 1985 and December 31, 1995 (ERA I) and between January 1, 1996 and December 31, 2002 (ERA II) who were diagnosed with biliary atresia at a university center were reviewed. RESULTS: 349 patients were identified. Median patient age at time of the Kasai operation was 55 days. Median age at last follow-up was 70 months. The 4-year patient survival rate was 81% (ERA I = 74%; ERA II = 82%; P = not significant [NS]). Kaplan-Meier survival curves for patients undergoing the Kasai operation at age < or = 30, 31 to 90, and > 90 days showed 49%, 36%, and 23%, respectively, were alive with their native liver at 4 years (P < .0001). This difference continued through 10 years. The 2- and 4-year post-Kasai operation native liver survival rates were 47% and 35% for ERA I and 46% and 39% for ERA II (P = NS). A total of 210 patients (60%) underwent liver transplantation; the 4-year transplantation survival rate was 82% (ERA I = 83%, ERA II = 82%; P = NS). CONCLUSIONS: This is the largest outcome series of North American children with biliary atresia at a time when liver transplantation was available. Results in each era were similar. Late referral remains problematic; policies to ensure timely diagnosis are required. Nevertheless, outcomes in Canada are comparable to those reported elsewhere.

Inflammatory bowel disease in the South Asian pediatric population of British Columbia.

BACKGROUND: Geographical differences, population migration, and changing demographics suggest an environmental role in prevalence, modulation, and phenotypic expression of inflammatory bowel disease (IBD). AIM: To determine the incidence of IBD and disease subtype in the pediatric South Asian population in British Columbia (BC) compared with non-South Asian IBD patients in the same geographic area. METHODS: Chart review with data collected for all patients

Can tissue transglutaminase antibody titers replace small-bowel biopsy to diagnose celiac disease in select pediatric populations?

OBJECTIVES: The use of screening tests for celiac disease has increased the number of patients referred for evaluation. We proposed that the subgroup of patients with very high tissue transglutaminase antibody (TTG) titers is positive for celiac disease and a small-bowel biopsy is not necessary to make the diagnosis. A gluten-free diet should be attempted and, if the patient's symptoms do not improve, then a biopsy should be performed to confirm the diagnosis. METHODS: A chart review of data for 103 patients who underwent both TTG testing and a small-bowel biopsy was performed. We examined the impact of using TTG values of >100 U and <20 U as cutoff values and suggested performing biopsies for patients with TTG values of 20 to 100 U, as is current practice. RESULTS: Fifty-eight of 103 patients demonstrated positive biopsy results. Forty-nine of 103 patients had TTG levels of >100 U, with 48 of 49 exhibiting positive biopsy results. Only 7 of 16 patients with TTG values of 20 to 100 U exhibited positive biopsy results. Three patients with TTG levels of <20 U had positive biopsies; 2 were IgA negative and 1 had a duodenal ulcer. With the cutoff values of >100 U and <20 U with known IgA status, the sensitivity was 0.980 (48 of 49 cases) and the specificity was 0.972 (35 of 36 cases). An incremental cost analysis found that this proposal could potentially decrease the costs of investigation and diagnosis by almost 30%. CONCLUSIONS: When the cutoff values were changed to >100 and <20 U and IgA levels were verified, the sensitivity and specificity were very high. Patients with mid-range TTG values (20-100 U) or values of <20 U with negative IgA status should continue to undergo biopsies for diagnosis of celiac disease.