Systematic Review of Antimicrobial Lock Solutions for Prevention of Bacteremia in Pediatric Patients With Intestinal Failure.

OBJECTIVES: The goal of this systematic review was to determine whether antimicrobial lock (AML) solutions prevent catheter-related bloodstream infections (CRBSI) in children with intestinal failure (IF). METHODS: Electronic databases were searched: Ovid MEDLINE (1946-), Ovid Embase (1974-), Wiley Cochrane Library (inception-), and Web of Science Core Collection via Clarivate Analytics (1900-). Randomized and nonrandomized trials, case or cohort studies that studied any AML solution, and used comparator groups were included if they studied children with IF. A meta-analysis compared the rates of CRBSI with AML solutions versus controls, and a Boucher analysis was used to indirectly compare AML solutions. RESULTS: Twenty-eight studies met eligibility criteria (1 open label and 27 observational studies). Quality was good (N = 13), fair (N = 9), and poor (N = 6). All but 4 studied ethanol and taurolidine. Of 15 ethanol studies, 11 reported a decrease and 3 reported a trend toward a decreased incidence of CRBSI compared to controls; 1 reported no difference. Of 9 taurolidine studies, 7 reported a decrease and 2 a trend toward decreased CRBSI rates. There was a decrease in CRBSI with ethanol versus control ( P = 0.008) and with taurolidine-citrate versus control ( P < 0.0005). Using Bucher indirect comparison of the pooled estimates from ethanol versus control to taurolidine versus control, the estimated difference was -0.99 (-4.125, 2.27; P = 0.55). CONCLUSIONS: There were no randomized trials and over half of the 28 included studies were fair or poor quality. All but 1 reported at least a trend toward reduction in CRBSI. AML solutions appear to prevent CRBSI.

FODMAP intake in children with coeliac disease influences diet quality and health-related quality of life and has no impact on gastrointestinal symptoms.

Children with coeliac disease (CD) following the gluten-free diet may experience ongoing gastrointestinal symptoms despite strict adherence. The study objective was to evaluate the association between foods high in fermentable oligo/di/monosaccharides, and polyols (FODMAP) and gastrointestinal symptoms, and the potential implications to diet quality and health-related quality of life in CD children. Dietary intake was studied in age-sex matched children 5-18 years (CD, n = 46; non-coeliac mild chronic gastrointestinal complaints [GIC], n = 46; healthy controls [HC], n = 46). CD children consumed fewer foods high in FODMAPs compared to GIC and HC (p < .0001). FODMAP intake was not related to gastrointestinal symptoms in CD children (p > 0.05) but was positively associated with child health-related quality of life (p < 0.05). FODMAP intake from fruits and vegetables was positively associated with diet adequacy and total diet quality in CD children (p < 0.05). FODMAP intake may influence diet quality and health-related quality of life but has no impact on gastrointestinal symptoms in CD children.

Adherence to the Gluten-free Diet and Health-related Quality of Life in an Ethnically Diverse Pediatric Population With Celiac Disease.

OBJECTIVES: Celiac disease (CD) is an autoimmune disease that requires lifelong adherence to a gluten-free diet (GFD). Adherence to the GFD in childhood may be poor and adversely influence health-related quality of life (HRQOL). The study purpose was to determine sociodemographic and socioeconomic factors influencing adherence to the GFD and HRQOL in a multiethnic cohort of youth with CD. METHODS: A multisite (Edmonton, Hamilton, Toronto) study examining child-parent HRQOL in youth with CD (n = 243) and/or mild gastrointestinal complaints (GI-CON; n = 148) was conducted. Sociodemographic (age, child-parental age/education/ethnicity/place of birth), anthropometric (weight, height, body mass index), disease (diagnosis, age at diagnosis, duration, Marsh score, serology), household characteristics (income, family size, region, number of children/total household size), HRQOL (Peds TM/KINDL and Celiac Disease DUX), GI Complaints (PedsQL: Gastrointestinal Symptom Scale) and gluten intake were measured. RESULTS: Younger age (<10 years), non-Caucasian ethnicity (parent/child), and presence of GI symptoms were associated with the highest rates of adherence to the GFD in CD children (P < 0.05). CD children (parent/child) had higher HRQOL (average, composite domains) than GI-CON (P < 0.05), but CD children were comparable to healthy children. Lack of GI symptoms, non-Caucasian ethnicity and age (<10 years) were associated with increased HRQOL in composite/average domains for CD (P < 0.05). CONCLUSIONS: Child-parent perceptions of HRQOL in a multiethnic population with CD are comparable to healthy reference populations, but significantly higher than in parent/child GI-CON. Adherence to the GFD in ethnically diverse youth with CD was related to GI symptoms, age of the child, and ethnicity of the parent-child.

Capillary Flow Rates in the Duodenum of Pediatric Ulcerative Colitis Patients Are Increased and Unrelated to Inflammation.

BACKGROUND AND AIM: Inflammatory bowel diseases (IBD), including Crohn disease (CD) and ulcerative colitis (UC), are chronic pediatric disorders. Changes in vasculature are described in IBD, but these could be secondary to inflammation and the role in pathogenesis is poorly understood. Assessing circulatory changes in typically unaffected sites in IBD (eg, duodenum), when inflammation is absent, can identify vascular changes associated with pathogenesis. The aim of the study is to measure capillary flow rates in duodenal mucosa using probe-based confocal laser endomicroscopy (pCLE) during endoscopy in children with IBD. METHODS: Images of villi with visible blood vessels obtained using pCLE were captured as video sequences. Capillary flow rate (mm/s) was calculated by dividing the distance travelled by blood cells by the duration of the sequence. Flow rates were correlated with various clinical parameters. RESULTS: Forty-five patients (22 non-IBD, 14 CD, 9 UC) were included in the study. Duodenal capillary flow rates were significantly higher in UC patients (0.75 ±â€Š0.07 mm/s) as compared to non-IBD (0.57 ±â€Š0.03) and CD (0.65 ±â€Š0.04). There was no correlation between serum hemoglobin and albumin, disease activity indices, serum inflammatory markers, and capillary flow rates in patients. CONCLUSIONS: This pilot study shows, for the first time, increased capillary blood flow in the duodenum of UC patients that was unrelated to inflammatory markers or disease activity. Thus, early vascular changes can be assessed using pCLE during endoscopy.

Satisfaction in open access versus traditional referral for upper endoscopy in children.

OBJECTIVES: In traditional access endoscopy (TAE), patients are booked for endoscopy following a gastroenterology clinic assessment. In contrast, open access endoscopy (OAE) patients are seen for the first time on the day of the procedure, providing same day procedural consent. Controversy exists over the use of OAE in adults, both with the consent process and with patient satisfaction. No literature exists describing satisfaction with OAE in pediatrics. We therefore aimed to assess pediatric patient and caregiver satisfaction in OAE compared with TAE. METHODS: Consecutive pediatric patients, and their caregivers, undergoing elective upper endoscopy from May to December 2012 at the Stollery Children's Hospital (Edmonton, Alberta, Canada) were consented for a cross-sectional survey. Seven preprocedure and 5 postprocedure questions were completed regarding mood and satisfaction with the wait time and the information provided. Group demographics and endoscopy wait times were collected. RESULTS: Median wait time with OAE was less compared with TAE (57 days vs 196 days, P < 0.001). OAE patients reported worse mood preprocedure than TAE patients (35.3% vs 10.7%, P = 0.046). OAE caregivers and patients reported more mood disturbance if required to wait longer for endoscopy by attending clinic preprocedure (OAE caregivers 62.2%, OAE patients 64.7%). CONCLUSIONS: OAE is associated with worse preendoscopy patient mood; however, children and caregivers seem concerned about longer wait times associated with TAE. Given the significantly shorter wait times in OAE, identifying methods to minimize present limitations of OAE will be useful to improve clinical practices in pediatric gastroenterology.

Laboratory procedures update on Hirschsprung disease.

OBJECTIVES: The detection of ganglion cells in rectal biopsies of infants or toddlers with severe constipation is routinely performed by pediatric pathologists in many institutions. Hirschsprung disease (HD) is defined by the lack of ganglion cells (aganglionosis). The early recognition and the prompt implementation of surgical procedures obviously protect infants affected with HD from potential life-threatening conditions, including enterocolitis and debilitating constipation. Image-based and non-image-based clinical techniques and some laboratory tests have been reevaluated along the years, but often fragmentarily. Immunohistochemical markers have been increasingly used in pathology laboratories to detect ganglion cells and nerve fibers. Recently, calretinin, a vitamin D-dependent calcium-binding protein with expression in ganglion cells and nerves, has been described as an adjunctive or primary diagnostic test in HD. The aim of the present study was to systematically summarize and update laboratory procedures targeting ganglion cells in rectal biopsies. METHODS: Procedures and tests have been reviewed and values of specificity and sensitivity have been calculated according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. RESULTS: Contrast enema has the lowest sensitivity and specificity of all of the 3-index investigations under the lens: contrast enema, anorectal manometry, and biopsy with histology. The latter procedure seems to have the highest sensitivity and specificity. Acetylcholinesterase staining on fresh-frozen material has been found to have slightly higher rates of sensitivity and specificity when compared with hematoxylin and eosin only. Calretinin staining may be supportive for the diagnosis, although some cases with false-positivity may be of some concern. CONCLUSIONS: Hematoxylin and eosin with or without acetylcholinesterase remains the criterion standard according to our PRISMA-based data. In our opinion, the number of false-positive results with potential overtreatment may limit the increasing advocacy for calretinin staining. Both the "primum non nocere" dictum and the "loss aversion heuristic" need to be satisfied harmoniously by preventing harm from unnecessary surgery.

Intestinal and gastric permeability in children with eosinophilic esophagitis and reflux esophagitis.

OBJECTIVES: Eosinophilic esophagitis (EoE) is an allergic and immune-mediated entity that leads to a characteristic inflammation of esophageal mucosa. Patients complain of dysphagia and reflux-like symptoms. As many as 80% of patients with EoE may also have a history of atopy, and patients with asthma and eczema have previously been shown to have increased intestinal permeability. This study was designed to assess small intestinal and gastric permeability in patients with EoE and to see whether it differed from healthy individuals and patients with reflux esophagitis (RE). METHODS: Gastric and small intestinal permeability was measured using sugar probe tests containing lactulose, mannitol, and sucrose. Lactulose-to-mannitol (L/M) ratios in the patient's urine were a measure for intestinal permeability, and total sucrose was a measure for gastric permeability. RESULTS: We analyzed samples from 23 patients with EoE, 20 RE, 14 normal upper endoscopy with gastrointestinal symptoms, and 26 healthy controls. All of the 4 groups had L/M ratios less than the upper limit of normal (<0.025). There was no statistically significant difference in gastric permeability between the 4 groups (L/M P = 0.26, sucrose P = 0.46). CONCLUSIONS: Our data suggest that an alteration in gastric and intestinal permeability does not play a role in EoE or RE pathogenesis.

Role of Environmental Factors in the Development of Pediatric Eosinophilic Esophagitis.

BACKGROUND: Despite accumulating data on the pathogenesis of eosinophilic esophagitis, not much is known about risk factors for the development of the disease. The role of factors such as smoking, breastfeeding, early antibiotic exposure and other factors that have been associated with other allergic diseases has not been well studied in children with eosinophilic esophagitis. AIM: To explore the role of environmental and medication exposures in the development of pediatric eosinophilic esophagitis. METHODS: We conducted a cross-sectional case-control study, utilizing a parent and child questionnaire and medical chart review. Urine cotinine levels, measured by high-performance liquid chromatography, were obtained as objective evidence for smoking exposure. RESULTS: One hundred and two children with eosinophilic esophagitis and 167 controls were recruited. The controls were mainly diagnosed with functional gastrointestinal disorders (33%) and gastroesophageal reflux disease (29%). Food allergy, specifically for peanuts and tree nuts, and allergy to pollen, tree, and grass were significantly higher among eosinophilic esophagitis children. Smoking exposure, both primary and secondary, was not associated with pediatric eosinophilic esophagitis when compared to controls (odds ratio 0.96, 95% confidence interval 0.58-1.59). Furthermore, early smoking exposure in the first year of life was higher among controls. Common accepted risk factors for allergy and atopy, such as breastfeeding practices, antibiotics exposure, animals' exposure, and others, were not found to be associated with eosinophilic esophagitis in our study. CONCLUSION: Common risk factors in other allergic and atopic conditions were not found to be associated with eosinophilic esophagitis.

Assessing Nutritional Quality and Adherence to the Gluten-free Diet in Children and Adolescents with Celiac Disease.

PURPOSE: Little is known regarding nutritional adequacy of the gluten free diet (GFD) in children and adolescents with celiac disease (CD). The study aim was to examine macro- and micronutrient intake in children with CD. METHODS: A cross-sectional study was conducted in children and adolescents (4-18 years of age) with CD (n = 32) and healthy controls (n = 32). Macro- and micronutrient intake, and glycemic index (GI) and glycemic load (GL) intake was assessed using validated measures. Diet quality was assessed using the Canadian Healthy Eating Index (HEI-C) and the Alberta Nutrition Guidelines. Values are shown as mean ± SE. RESULTS: Age (10.4 ± 0.7 years vs 8.7 ± 0.7 years; P = 0.06), weight-for-age z score (P = 0.27), and height-for-age z score (P = 0.23) were not different between groups. CD children consumed more fibre (15.9 ± 1.2 g per day(CD) vs 10.8 ± 0.8 g per day (controls); P < 0.001), had higher GI (CD: 54 ± 2 vs 48 ± 1; P < 0.01) and GL (CD: 118 ± 8 vs 93 ± 7; P = 0.02), and lower folate (146.7 ± 15.3 (CD) vs 245.4 ± 21.8 µg per day (controls); P < 0.001) than controls. No differences in HEI-C scores (≤ and > 80) were observed between groups (P > 0.05). CONCLUSION: Children with CD had high intakes of fibre, GI, and GL and lower intakes of folate. This has implications for dietary counselling in this population.

Use of exclusive enteral nutrition is just as effective as corticosteroids in newly diagnosed pediatric Crohn's disease.

BACKGROUND: The efficacy of exclusive enteral nutrition (EEN) in induction of remission in pediatric Crohn's disease (CD) is reported to be equivalent to that of corticosteroids (CS). AIMS: Our objective was to compare the efficacy of EEN and CS in inducing remission in pediatric onset CD and the effects of the treatment on nutritional status and bone mineral density (BMD). METHODS: Medical charts were retrospectively studied for patients diagnosed with CD between 2000 and 2010 at the Stollery children's hospital in Edmonton, Alberta. Anthropometric and dual-energy X-ray absorptiometry (DXA) data were collected to assess effects of therapy; clinical remission, relapse, and severity were defined on the basis of the pediatric Crohn's disease activity index. RESULTS: To induce remission at first presentation, 36 patients (mean age 12.9 years) received EEN and 69 (mean age 11.2 years) received CS. Remission (88.9% in the EEN group versus 91.3% in the CS group (p=0.73) at 3 months) and relapse (40.6 vs. 28.6%, respectively (p=0.12) over 12 months) were similar in both treatment groups. Thirty-four patients had paired DXA scans at the time of diagnosis and one year later: 16 given EEN and 18 given CS. Change in BMD spine z-scores based on bone age adjusted for height and chronological age was greater for EEN patients but not statistically significant (Δz-score 0.30 vs. 0.03, p=0.28). CONCLUSIONS: EEN has similar efficacy to corticosteroids; however, EEN may lead to better BMD accrual. EEN should be preferred to corticosteroids as first-line therapy for induction of remission in pediatric CD.

Gluten-free diet does not appear to induce endoscopic remission of eosinophilic esophagitis in children with coexistent celiac disease.

BACKGROUND: Celiac disease and eosinophilic esophagitis are usually considered to be separate gastrointestinal diseases; however, it appears that they may coexist more often than would be expected. It is unknown whether eosinophilic esophagitis in patients with celiac disease responds to a gluten-free diet. OBJECTIVES: To examine the clinical, endoscopic and histological features of children with both conditions to evaluate whether eosinophilic esophagitis responds to a gluten-free diet. METHODS: From January 1, 2009, to June 30, 2011, the medical records of children <18 years of age diagnosed with eosinophilic esophagitis and/or celiac disease were reviewed. Patients with clinical, endoscopic and histological diagnoses of both diseases were identified and included. These findings were analyzed, as were laboratory results, treatment and follow-up. RESULTS: During the study period, there were 206 celiac disease patients, 86 eosinophilic esophagitis patients and nine (4.4% of total celiac) patients with both diagnoses. Gluten-free diet was the primary treatment for both conditions in seven of nine (78%) cases. In six of these seven (86%) patients, no endoscopic or histological improvement of eosinophilic esophagitis was observed, while in one patient, histological remission of esophageal eosinophilia occurred while on a gluten-free diet. CONCLUSION: The prevalence of eosinophilic esophagitis in patients with celiac disease was 4.4%, confirming a higher than expected prevalence of eosinophilic esophagitis compared with the general population. In patients with celiac disease, a gluten-free diet did not appear to induce remission of coexistent endoscopic and histological features of eosinophilic esophagitis.

Incidence of Pediatric Eosinophilic Esophagitis and Characterization of the Stricturing Phenotype in Alberta, Canada.

Limited work has been done to characterize the stricturing pediatric eosinophilic esophagitis (EoE) phenotype. We aimed to determine, in pediatric EoE: the local incidence, the frequency of esophageal stricturing, and the safety of mechanical dilations. Methods: We retrospectively identified all new cases of EoE at our center from 2015 to 2018 using esophageal biopsy reports, EoE clinic lists, and a local OR database of esophageal dilatations. Electronic medical records (EMRs) were reviewed to confirm EoE diagnosis. Clinical data were captured from the outpatient EMR and gastroscopy/pathology reports. Scope adverse event data were captured from multiple sources. The 2016 census data were used to calculate incidence rates. Results: One hundred eighty-five new cases of EoE were diagnosed during the study period. For patients <15 years old living in Edmonton, the incidence over the 4 years was 11.1 cases per 100,000 person years. Eight of 185 (4%) patients had endoscopically confirmed esophageal strictures, 4 of which required mechanical dilation. Eleven of 185 (5.9%) patients had more subtle signs of esophageal narrowing, but no focal strictures. No perforations or episodes of significant bleeding were reported. Pain was reported after 15% of all scopes, including 50% of the 28 scopes with focal strictures. No unexpected admissions or emergency department visits occurred within 72 hours of a gastroscope with esophageal narrowing. Conclusions: Edmonton zone has one of the highest incidences of pediatric EoE reported. In this cohort, 4% had focal esophageal strictures, and 6% had more subtle narrowing. Mechanical dilation of esophageal strictures was associated with no significant adverse events.

Canadian Nationwide Survey on Pediatric Malnutrition Management in Tertiary Hospitals.

Background: Disease-associated malnutrition (DAM) is common in hospitalized children. This survey aimed to assess current in-hospital practices for clinical care of pediatric DAM in Canada. Methods: An electronic survey was sent to all 15 tertiary pediatric hospitals in Canada and addressed all pillars of malnutrition care: screening, assessment, treatment, monitoring and follow-up. Results: Responses of 120 health care professionals were used from all 15 hospitals; 57.5% were medical doctors (MDs), 26.7% registered dietitians (RDs) and 15.8% nurses (RNs). An overarching protocol for prevention, detection and intervention of pediatric malnutrition was present or "a work in progress", according to 9.6% of respondents. Routine nutritional screening on admission was sometimes or always performed, according to 58.8%, although the modality differed among hospitals and profession. For children with poor nutritional status, lack of nutritional follow-up after discharge was reported by 48.5%. Conclusions: The presence of a standardized protocol for the clinical assessment and management of DAM is uncommon in pediatric tertiary care hospitals in Canada. Routine nutritional screening upon admission has not been widely adopted. Moreover, ongoing nutritional care of malnourished children after discharge seems cumbersome. These findings call for the adoption and implementation of a uniform clinical care pathway for malnutrition among pediatric hospitals.

Clostridium ventriculi Infection in a Child with Phenylketonuria.

Disabled individuals may be at risk for common and rare infections. We report on a 13-year-old female who had a diagnosis of phenylketonuria (PKU). The child received a percutaneous endoscopic gastrostomy (PEG) feeding tube at five years of age for the supplementation of her specialized formula. After eight years, she no longer required the gastrostomy tube for formula supplementation, and she presented for the closure of the gastrocutaneous fistula tract. The histological examination revealed acute and chronic inflammation and colonization by gram-positive bacteria with a characteristic tetrad packet arrangement known as Clostridium ventriculi (formerly Sarcina ventriculi). A review of the literature evidenced the rarity of this infection in children. This patient is the 11th case of such infection in literature, and the first patient affected with PKU. Physically and mentally disabled children are particularly vulnerable to infection because of their different feeding abilities, toilet needs, and sanitary arrangements.

Screening for Pediatric Malnutrition at Hospital Admission: Which Screening Tool Is Best?

BACKGROUND: Identifying children at malnutrition risk on admission to hospital is considered best practice; however, nutrition screening in pediatric populations is not common. The aim of this study was to determine which screening tool is able to identify children with malnutrition on admission to hospital. METHODS: A nurse administered 2 pediatric nutrition screening tools, Screening Tool for Risk on Nutritional Status and Growth (STRONGkids) and Pediatric Nutrition Screening Tool (PNST) to patients admitted to medicine and surgery units (n = 165). The Subjective Global Nutritional Assessment (SGNA) was then completed by a dietitian, blinded to the results of the screens. Sensitivity, specificity, and κ were calculated for both screening tools against the SGNA. A receiver operating characteristic (ROC) curve assessed alternate cutoffs for each tool. Length of hospital stay (LOS) was used to assess prospective validity. RESULTS: Using the recommended cutoffs, the sensitivity of STRONGkids was 89%, specificity 35%, and κ 0.483. The sensitivity of PNST was 58%, specificity 88%, and κ 0.601. Using adjusted cutoffs, PNST's sensitivity improved to 87%, specificity 71%, and κ 0.681, and STRONGkids specificity improved to 61%, sensitivity 80%, and κ 0.5. Children identified at nutrition risk had significantly longer LOS (P < 0.05). CONCLUSION: This study showed neither tool was appropriate for clinical use based on published cutoffs. By adjusting the cutoffs using ROC curve analysis, both tools improved overall agreement with the SGNA without significantly impacting the prospective validity. PNST with adjusted cutoffs is the most appropriate for clinical use in this population.

Diet patterns in an ethnically diverse pediatric population with celiac disease and chronic gastrointestinal complaints.

INTRODUCTION: Celiac disease (CD) is an autoimmune disease requiring lifelong adherence to the gluten-free diet (GFD). The GFD has significant nutritional limitations which may result in poor diet quality (DQ). We hypothesized that biopsy-proven children with CD (CCD) would have dietary patterns characterized by high saturated fat/simple sugar intake with a low micronutrient density contributing to lower DQ when compared to children with mild-gastrointestinal complaints (GI-CON). In addition, we hypothesized that ethnicity may further impact DQ. METHODS: Socio-demographic (age, CD duration, parent/child ethnicity, education), household characteristics, anthropometric, dietary intake (24-h recalls), gastrointestinal pain and adherence was collected in CCD (n = 243) and GI-CON (n = 148). Dietary patterns were determined using k-mean Cluster Analysis. RESULTS: GI-CON had significantly lower DQ than CCD (p < 0.001). Most CCD and GI-CON (>80%) had dietary patterns characterized by1) Western Diet (Cluster 1: %BMR: 110-150, low DQ, high fat, moderate CHO, high sodium) and 2) High Fat-Western Diet (Cluster 2: %BMR:130-150, low DQ, high Fat, high processed meats, high fat dairy products, CHO. Fewer children (<20%) had Prudent, Lower Fat/High Carbohydrate dietary patterns (% BMR:100-150, higher DQ, lower fat/sodium, higher CHO) with a greater proportion of non-Caucasian CCD consuming a Prudent dietary pattern. Seventy-seven percent and 37.5% of CCD and GI-CON, respectively, did not meet estimated average requirements for folate (p < 0.001). CONCLUSIONS: CCD and GI-CON have predominantly Western dietary patterns with low DQ, particularly GI-CON. Non-caucasian CCD consume more prudent dietary patterns with higher DQ. Nutrition education is warranted to ensure optimal DQ in children with chronic gastrointestinal diseases.

Can energy intake alter clinical and hospital outcomes in PICU?

BACKGROUND & AIMS: Energy is essential for the treatment and recovery of children admitted to Pediatric Intensive Care Units (PICU). There are significant immediate and long-term health consequences of both under- and over-feeding in this population. Energy requirements of critically ill children vary depending on age, nutritional status, sepsis, fever, pharmacotherapy, and duration and stage of critical illness. This study aimed to determine the incidence of over- and under-feeding and to compare hospital outcomes between these feeding categories. Secondary outcomes were collected to describe the association between feeding categories and biochemistries (serum lactate, triglycerides, C-reactive protein). METHODS: An ethics approved retrospective study of children admitted to PICU was performed. All intubated patients admitted to PICU (2008-2013) were included, except those in which an IC test was not feasible. Data collection included demographics, the primary outcome variable reported as under feeding (<90%MREE), appropriate (MREE ±10%) or overfeeding (>110% MREE) determined through comparison of measured resting energy expenditure (MREE) using indirect calorimetry (IC) to actual energy intake based on predicted basal metabolic rate (PBMR) and clinical outcomes mechanical ventilation and PICU length of stay (LOS). Data were analysed with descriptive methods, ANOVA and linear regression models. RESULTS: A total of 139 patients aged 10 (range 0.03-204) months were included. Sixty (43%) were female and 77 (55%) were admitted after a surgical procedure. A total of 210 IC tests were conducted showing a statistically significant difference between MREE measurements and PBMR (p = 0.019). Of the 210 measurements, only 26 measures (12.4%) demonstrated appropriate feeding, while 72 (34.3) were underfed and 112 (53.3%) were overfed. Children who were overfed had significantly longer PICU LOS (median 45.5, IQR 47.8 days) compared to those children in the appropriately fed (median 21.0, IQR 54.5 days), and underfed groups (median 16.5, IQR 21.3 days). There was a mean difference between the over and under feeding category and ventilation days after adjusting for age and PRISM score (p = 0.026), suggesting decreased mechanical ventilation days for underfed. Children who were underfed had significantly higher CRP (median 75.5, IQR 152.8 mg/L) compared to those children in the appropriately fed (median 57.8, IQR 90.9 mg/L) and overfed groups (median 22.4, IQR 56.2 mg/L). CONCLUSIONS: This retrospective study confirms that estimations of energy expenditure in critically ill children are inaccurate leading to unintended under and overfeeding. Importantly under feeding seems to be associated with fewer mechanical ventilation days and PICU LOS. Further research is required to elucidate the role of optimal nutrition in altering clinical variables in this population.

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.

Atypical Kawasaki disease and gastrointestinal manifestations.

Kawasaki disease is a syndrome that usually occurs in infants and children. It is characterized by an exanthem, enanthem, fever, lymphadenopathy, and polyarteritis of variable severity. The present report describes cases in which an initial presentation of Kawasaki disease included abdominal and gastrointestinal symptomatology.

Dysphagia in healthy children: Characteristics and management of a consecutive cohort at a tertiary centre.

OBJECTIVE: Whereas the literature is replete with reports on complex children with dysphagia (DP), the parameters characterizing non-neurologically impaired (NNI) children have been underreported, leaving a substantial knowledge gap. We set to characterize a consecutive cohort of NNI children, their management, and outcomes. METHODS: We undertook a retrospective case series. Children (<18 years old) attending a tertiary multidisciplinary swallowing clinic were eligible. Patients with neuro-developmental, neuromuscular, or syndromic abnormalities were excluded. Primary outcomes included demographics, co-morbidities, presentations, McGill score, swallowing and airway abnormalities (and their predictors). Secondary outcomes were interventions and management response. RESULTS: From 171 consecutive patients (37-month period), 128 were included (69 males, median age 6.6 months (0.5-124.2)). Significant clinical presentations included recurrent pneumonias (20), cyanotic spells (14) and life-threatening events (10). Swallowing assessments revealed laryngeal penetration (67), aspiration (25). Other investigations included overnight oximetry (77), airway (70), and gastrointestinal endoscopy (24); revealing laryngomalacia (29), laryngeal mobility disorder (8), and subglottic stenosis (8). Non-surgical interventions involved oral diet modifications (85) and enteral nutrition (15). Surgical interventions included supraglottoplasties (18), endoscopic laryngeal cleft repair (14), and injection (19). 119 patients received intervention and at last follow-up (median 5.2 months (0.3-88.8)) 94 had improved. Of those treated 116 were on an unmodified oral diet, and 24 on a modified diet. ALTE and snoring predicted airway abnormalities, recurrent pneumonia predicted swallowing abnormalities, and age and airway lesions predicted the McGill score. CONCLUSION: a significant proportion of NNI children with DP harbor airway and swallowing abnormalities warranting endoscopic and instrumental assessment.