Characteristics of Pediatric Patients With Celiac Disease Identified Due to an Affected First-Degree Family Member.

The incidence of celiac disease in first-degree relatives of affected individuals is higher than in the general population, yet the clinical characteristics of this unique subset of patients has not been well described. Through a retrospective review of patients seen in a tertiary care pediatric celiac disease clinic, we identified 49 patients diagnosed with celiac disease following screening due to an affected first-degree relative. Although 51% of patients screened due to an affected first-degree relative were asymptomatic, their disease histology was as severe as those screened for symptoms suggestive of celiac disease. These findings support current recommendations to screen all first-degree relatives of patients with celiac disease regardless of clinical symptoms.

Nutritional considerations of a paediatric gluten-free food guide for coeliac disease.

The gluten-free (GF) diet is the only treatment for coeliac disease (CD). While the GF diet can be nutritious, increased reliance on processed and packaged GF foods can result in higher fat/sugar and lower micronutrient intake in children with CD. Currently, there are no evidence-based nutrition guidelines that address the GF diet. The objective of this cross-sectional study was to describe the methodological considerations in forming a GF food guide for Canadian children and youth (4-18 years) with CD. Food guide development occurred in three phases: (1) evaluation of nutrient intake and dietary patterns of children on the GF diet, (2) pre-guide stakeholder consultations with 151 health care professionals and 383 community end users and (3) development of 1260 GF diet simulations that addressed cultural preferences and food traditions, diet patterns and diet quality. Stakeholder feedback identified nutrient intake and food literacy as important topics for guide content. Except for vitamin D, the diet simulations met 100 % macronutrient and micronutrient requirements for age-sex. The paediatric GF plate model recommends intake of >50 % fruits and vegetables (FV), <25 % grains and 25 % protein foods with a stronger emphasis on plant-based sources. Vitamin D-fortified fluid milk/unsweetened plant-based alternatives and other rich sources are important to optimise vitamin D intake. The GF food guide can help children consume a nutritiously adequate GF diet and inform policy makers regarding the need for nutrition guidelines in paediatric CD.

Evaluation of a paediatric gluten-free food guide by children and youth with coeliac disease, their parents and health care professionals.

There are currently no universal evidence-based nutrition guidelines that address the gluten-free (GF) diet for children/youth (4-18 years). A GF food guide was created to help children/youth with coeliac disease (CD) and their families navigate the complexities of following a GF diet. Guide formation was based on pre-guide stakeholder consultations and an evaluation of nutrient intake and dietary patterns. The study objective was to conduct an evaluation on guide content, layout, feasibility and dissemination strategies from end-stakeholder users (children/youth with CD, parents/caregivers and health care professionals). This is a cross-sectional study using a multi-method approach of virtual focus groups and an online survey to conduct stakeholder evaluations. Stakeholders included children/youth (4-18 years), their parents/caregivers in the coeliac community (n 273) and health care professionals (n 80) with both paediatric and CD experience from across Canada. Thematic analysis was performed on focus group responses and open-ended survey questions until thematic saturation was achieved. χ2 and Fisher's exact statistical analyses were performed on demographic and close-ended survey questions. Stakeholders positively perceived the guide for content, layout, feasibility, ethnicity and usability. Stakeholders found the material visually appealing and engaging with belief that it could effectively be used in multi-ethnic community and clinical-based settings. Guide revisions were made in response to stakeholder consultations to improve food selection (e.g. child-friendly foods), language (e.g. clarity) and layout (e.g. organisation). The evaluation by end-stakeholders provided practical and patient-focused feedback on the guide to enable successful uptake in community and clinical-based settings.

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.

Folate content of gluten-free food purchases and dietary intake are low in children with coeliac disease.

The lack of mandated folate enrichment of gluten-free (GF) grains in Canada has been suspected to contribute to suboptimal folate intake among children suffering from Celiac disease (CD). Children with CD on the gluten-free diet (GFD) face nutrient imbalances (higher fat/sugar, lower folate) from processed GF foods. The study objective examined folate intake in children with CD and folate content of household food purchases. Households collected food receipts for 30 days to assess folate content. Folate-rich foods were defined as ≥60 µg dietary folate equivalent (DFE)/100g. Two 24-hour recalls assessed children's intake. Households (n = 73) purchased >17,000 food items. Median child age was 10.5 y (IQR: 8.4-14.1). GF folate-rich foods represented <15% of all household food purchases and 69% of children had low folate intakes. Folate-rich foods consumed included legumes/GF-breakfast cereals. These represented 5% of GF-food purchases/intake. Few were fortified with folate. Findings highlight the need for mandated GF folate food fortification policy.

Iron Status in Pediatric Celiac Disease: A Retrospective Chart Review.

The present study assessed the role of serum ferritin as a noninvasive biomarker in the diagnosis and monitoring of pediatric celiac disease. A retrospective chart review was performed on patients younger than 18 years old at time of diagnosis (n = 193) between 1998 and 2015. A total of 653 paired values demonstrated a weak negative correlation between serum ferritin and tissue transglutaminase-immunoglobulin A (r = -0.114; P = 0.004), necessitating further evaluation. A significant relationship was found between reduction of tissue transglutaminase-immunoglobulin A and increase in serum ferritin after institution of a gluten-free diet (P < 0.0001), suggesting that resolution of villous damage is necessary for promoting adequate iron absorption.

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.

Implementation of a multidisciplinary team approach and fish oil emulsion administration in the management of infants with short bowel syndrome and parenteral nutrition-associated liver disease.

OBJECTIVE: To describe the authors' experience with the implementation of a multidisciplinary approach and use of fish oil emulsion (FOE) in the management of infants with short bowel syndrome (SBS) and parenteral nutrition-associated liver disease (PNALD). METHODS: Between August 2006 and June 2009, four cases of SBS and severe PNALD were managed by the team using specifically developed protocols. The FOE was initiated if serum direct bilirubin levels were ≥100 µmol/L. To quantify the degree of exposure to high serum direct bilirubin levels over time, the area under the curve (AUC) for each patient was calculated before and after initiation of FOE. Linear regression analyses were performed to evaluate correlations between the AUC, duration of cholestasis and initiation of FOE. RESULTS: All patients survived and no complications were observed during the study period. After the first patient, FOE was initiated progressively earlier, but poor correlation between the AUC before and after its introduction was observed (r(2)=0.41924). However, there was strong correlation between the duration of PNALD before FOE initiation and time to resolution (r(2)=0.72133): the earlier the FOE was initiated, the shorter the time to resolution. CONCLUSION: The authors report a positive experience with the implementation of a multidisciplinary approach and the use of FOE in infants with SBS and severe PNALD. The earlier the FOE was initiated during the cholestatic process, the shorter the time to resolution. The present study is a hypothesis generator, raising the question of whether an earlier introduction of this particular therapy can effectively shorten the cholestasis process in these patients.

The Gluten Free Diet: Assessing Adherence in a Pediatric Celiac Disease Population.

BACKGROUND: A strict, lifelong, gluten-free diet (GFD) remains the sole treatment for celiac disease (CD). The assessment of adherence to the GFD in pediatric studies is often based on self-report and visual analogue scales which lack proven validity. We sought to compare parental-report of GFD adherence to expert registered dietitian (RD) assessments, the best available standard. METHODS: Parents of children with biopsy-proven CD scored their adherence to the GFD on a five-point Likert scale similar to that used in previous pediatric CD studies. Each family was then evaluated by an RD expert in CD management who conducted a comprehensive and standardized assessment and scored the family's adherence. The agreement between parents and the RD was assessed using paired t test and intraclass correlation coefficient (ICC) based on their scores. RESULTS: One hundred twenty-two children and their families participated in the study, with a median of 32 months on a GFD. Excellent adherence (score 5 out of 5) was attributed to 60.5% of the sample by the RD. The parents scored adherence higher than the RD by an average difference of 0.41 scale points (95% CI, 0.28-0.54; P < 0.001). The agreement between parents and the registered dietitian was poor (ICC = 0.21). CONCLUSION: Reliance on self-report through Likert scales for GFD adherence overestimates adherence and misses opportunities for patient and family education. Approximately 40% of children with CD have ongoing gluten exposure, highlighting the need for regular assessment by an RD expert in the GFD to identify education and counselling needs for children with CD.

Starving the Gut: A Deficit of Butyrate in the Intestinal Ecosystem of Children With Intestinal Failure.

BACKGROUND: Intestinal epithelial integrity is influenced by short-chain fatty acids (SCFAs) and is of critical importance for children with intestinal failure (IF) given the known devastating infectious and gastrointestinal complications. The composition of the microbiome in IF represents an important variable in the physiology and prognosis of this disease. AIM: We sought to compare the intestinal microbiome and SCFA concentration of children who require parenteral nutrition (PN) with that of children with short-bowel syndrome (SBS) who have discontinued PN and with age-matched controls, using high-throughput sequencing to investigate host-microbe interactions. METHODS: Fifty-three samples were submitted over 6-15 months. Six children with SBS + IF submitted 34 samples, and 6 children with SBS with discontinued PN submitted 15 samples; these were compared with samples from 5 control children. Fecal samples were analyzed by 16S ribosomal RNA partial gene sequencing using the MiSeq Illumina sequencer. SCFAs were measured in stool samples by mass spectrometry. RESULTS: Butyrate quantity was near absent in children with IF compared with that in controls (median 0.37 nmol/mg vs 10.92 nmol/mg; P < .0001). Similarly, commensal anaerobes known to produce SCFA, including Ruminococcaceae and Lachnospiraceae, were reduced in those with SBS. SBS + IF enteric samples demonstrated a 168-fold increase in the relative abundance of the Escherichia genus seemingly attributable to the species Escherichia coli. CONCLUSION: The reduced relative abundance of butyrate-producing Clostridia as well as decreased intestinal butyrate concentration in children with IF support further investigation in therapeutic options that target butyrate-producing bacterial communities or butyrate supplementation.

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.