Impact of a Child's Celiac Disease Diagnosis and Management on the Family.

BACKGROUND: Little attention has been paid to family-wide repercussions of a child's celiac disease diagnosis and concomitant gluten-free diet management. AIMS: We quantitatively and qualitatively describe positive and negative family-wide effects of a child's celiac disease diagnosis and disease management. METHODS: We interviewed 16 families with at least one child currently following a gluten-free diet, with a biopsy-confirmed celiac disease diagnosis ≥ 1 year prior. Mothers and fathers independently rated child's dietary adherence, concern about child's health status, burden in caring for child's dietary needs, and level of change in various aspects of life post- diagnosis. Children rated their own celiac-specific quality of life through a validated scale. Seventy-one in-depth semi-structured interviews were conducted with 16 children with celiac disease, 31 parents, and 24 siblings. RESULTS: Mothers and fathers rated the effects of their child's celiac disease differently, with mothers reporting more lifestyle changes and heavier burden. Negative and positive themes emerged from the interviews. Mothers felt the burden of managing a gluten-free diet. Fathers felt guilty for carrying a celiac disease-associated gene and both fathers and siblings regretted limited food choices at restaurants and home. The need to be a more creative cook was seen as a positive effect by mothers. Fathers appreciated new family traditions. Siblings felt they had developed empathy for others. A framework is proposed to illustrate these family-wide interactions. CONCLUSIONS: A child's celiac disease diagnosis and disease management affects the entire family. Our results will inform family-centered interventions that maximize quality of life for families.

Frequency and Predictors of Successful Transition of Care for Young Adults With Childhood Celiac Disease.

OBJECTIVES: Transition from pediatric to adult care for individuals with chronic conditions is important to prevent gaps in care, though this has not been well-studied in celiac disease (CD). The aim of this study was to discern rates and predictors of successful transition of care for young adults with childhood-diagnosed CD. METHODS: An anonymous 21-question online survey was sent to individuals on our center's email contact list seeking responses from those ages 18 to 25 years diagnosed with CD before age 18 years. Information collected included method of diagnosis, demographics, CD-related care, reasons for not seeking care, and symptoms. RESULTS: Respondents (n = 98), 70% women, had a median age of 21 years (IQR 19--23 years). The majority were full or part-time students (67%; 95% CI 59%-77%). Only 31% of respondents had successfully transitioned to an adult CD provider. Some 37% (95% CI 29%-48%) were not receiving any CD medical care. An older age at diagnosis was associated with successful transition to adult gastroenterology (P = 0.002) as well as with greater symptom scores (P = 0.002). Receiving a referral for ongoing adult CD care predicted successful transition to an adult provider (odds ratio [OR] 3.92, 95% CI 1.58-9.72). CONCLUSIONS: Transition of care for young adults with CD is inconsistent, particularly among asymptomatic patients. Receipt of a referral for an adult provider significantly improves follow-up rates.

Rate, Risk Factors, and Outcomes of Nonadherence in Pediatric Patients With Celiac Disease: A Systematic Review.

BACKGROUND AND AIMS: The only treatment for celiac disease is strict adherence to a gluten-free diet (GFD). We performed a systematic review to investigate the rate of adherence to a GFD in children with celiac disease, risk factors that affect adherence, and outcomes of non-adherence. METHODS: We searched PubMed, Cochrane Library, EBSCO, and Scopus for studies through January 2019. We included observational studies of ≥50 children diagnosed with celiac disease and recommended for placement on a GFD. We collected data on adherence assessment (self-report, serology tests, structured dietary interview, biopsies, or assays for gluten immunogenic peptides), risk factors, and outcomes related to adherence. Findings were presented with medians, range, and a narrative synthesis. RESULTS: We identified 703 studies; of these, 167 were eligible for full-text assessment and 49 were included in the final analysis, comprising 7850 children. Rates of adherence to a GFD ranged from 23% to 98%. Comparable rates (median rates of adherence, 75%-87%) were found irrespective of how assessments were performed. Adolescents were at risk of non-adherence and children whose parents had good knowledge about celiac disease adhered more strictly. Non-adherence associated with patient growth, symptoms, and quality of life. CONCLUSION: In a systematic review of 49 studies of children with celiac disease, we found substantial variation in adherence to a GFD among patients. Rate of adherence was not associated with method of adherence measurement, so all methods appear to be useful, with lack of consensus on the ideal metric. Studies are needed to determine the best method to ensure adherence and effects on long-term health.

Benefits From and Barriers to Portable Detection of Gluten, Based on a Randomized Pilot Trial of Patients With Celiac Disease.

Research links diminished quality of life (QOL) to the challenges of a strict gluten-free diet (GFD), the only treatment for celiac disease (CD).1-4 This pilot study assessed the acceptability and feasibility of a portable gluten sensor device (Nima) to promote GFD adherence and QOL.

Celiac Disease: Clinical Features and Diagnosis.

The presentation in celiac disease is shifting from the classical malabsorptive presentation to more nonclassical presentations, requiring clinicians to maintain a high level of suspicion for the disease and to be aware of the possible extraintestinal manifestations. The diagnosis of celiac disease is guided by initial screening with serology, followed by confirmation with an upper endoscopy and small intestinal biopsy. In some pediatric cases, biopsy may be avoided.

Correction to: Hypervigilance to a Gluten-Free Diet and Decreased Quality of Life in Teenagers and Adults with Celiac Disease.

The original version of the article unfortunately contained formatting errors in Table 3. The correct version of Table 3 is given in the Correction article.

Outcome measures in coeliac disease trials: the Tampere recommendations.

OBJECTIVE: A gluten-free diet is the only treatment option of coeliac disease, but recently an increasing number of trials have begun to explore alternative treatment strategies. We aimed to review the literature on coeliac disease therapeutic trials and issue recommendations for outcome measures. DESIGN: Based on a literature review of 10 062 references, we (17 researchers and 2 patient representatives from 10 countries) reviewed the use and suitability of both clinical and non-clinical outcome measures. We then made expert-based recommendations for use of these outcomes in coeliac disease trials and identified areas where research is needed. RESULTS: We comment on the use of histology, serology, clinical outcome assessment (including patient-reported outcomes), quality of life and immunological tools including gluten immunogenic peptides for trials in coeliac disease. CONCLUSION: Careful evaluation and reporting of outcome measures will increase transparency and comparability of coeliac disease therapeutic trials, and will benefit patients, healthcare and the pharmaceutical industry.

Hypervigilance to a Gluten-Free Diet and Decreased Quality of Life in Teenagers and Adults with Celiac Disease.

BACKGROUND AND AIMS: Avoidance of gluten is critical for individuals with celiac disease (CD), but there is also concern that "extreme vigilance" to a strict gluten-free diet may increase symptoms such as anxiety and fatigue, and therefore, lower quality of life (QOL). We examined the associations of QOL with energy levels and adherence to, and knowledge about, a gluten-free diet. METHODS: This is a cross-sectional prospective study of 80 teenagers and adults, all with biopsy-confirmed CD, living in a major metropolitan area. QOL was assessed with CD-specific measures. Dietary vigilance was based on 24-h recalls and an interview. Knowledge was based on a food label quiz. Open-ended questions described facilitators and barriers to maintaining a gluten-free diet. RESULTS: The extremely vigilant adults in our sample had significantly lower QOL scores than their less vigilant counterparts [(mean (SD): 64.2 (16.0) vs 77.2 (12.2), p = 0.004]. Extreme vigilance was also associated with greater knowledge [5.7 (0.7) vs 5.1 (0.8), p = 0.035]. Adults with lower energy levels had significantly lower overall QOL scores than adults with higher energy levels [68.0 (13.6) vs 78.9 (13.0), p = 0.006]. Patterns were similar for teenagers. Cooking at home and using internet sites and apps were prevalent strategies used by the hypervigilant to maintain a strict gluten-free diet. Eating out was particularly problematic. CONCLUSION: There are potential negative consequences of hypervigilance to a strict gluten-free diet. Clinicians must consider the importance of concurrently promoting both dietary adherence and social and emotional well-being for individuals with CD.

Characteristics and comorbidities of inpatients without celiac disease on a gluten-free diet.

OBJECTIVES: Despite the increasing popularity of gluten-free diet (GFD), the demographic characteristics and medical features of patients without celiac disease on this diet have not been extensively investigated.We aimed to characterize the medical conditions and demographic backgrounds of hospitalized patients without celiac disease who adhere to a GFD, to further understand their reasons for gluten avoidance. MATERIALS AND METHODS: We performed an observational cohort study on all inpatients at Columbia University Medical Center on a GFD in 2011-2016, excluding those with celiac disease, compared with age-matched and sex-matched inpatients on a regular diet. We determined the odds ratio (OR) of being on a GFD for various comorbidities using conditional logistic regression. RESULTS: Of 769 inpatients on a GFD, most (63.6%) did not have celiac disease. Gluten-avoiding patients were more likely to be non-Hispanic Whites [OR: 2.92; 95% confidence interval (CI): 2.31-3.70]. They had a lower prevalence of hypertension (OR: 0.38; 95% CI: 0.27-0.52) and diabetes (OR: 0.58; 95% CI: 0.32-0.75) and higher prevalence of inflammatory bowel disease (OR: 1.56; 95% CI: 1.02-2.41), irritable bowel syndrome (OR: 6.16; 95% CI: 2.11-10.23), hyperthyroidism (OR: 2.73; 95% CI: 1.22-6.10), hypothyroidism (OR: 2.06; 95% CI: 1.39-3.06), lupus (OR: 2.87; 95% CI: 1.13-7.29), and autism spectrum disorder (OR: 23.42; 95% CI: 5.29-103.73). CONCLUSION: Nonceliac gluten-avoiding patients have higher prevalences of inflammatory bowel disease, irritable bowel syndrome, thyroid disease, lupus, and autism spectrum disorder, suggesting patients with these disorders have turned to a GFD for perceived benefit, despite a scant evidence basis.

Coeliac disease: to biopsy or not?

Coeliac disease is increasingly recognized as a global problem in both children and adults. Traditionally, the findings of characteristic changes of villous atrophy and increased intraepithelial lymphocytosis identified in duodenal biopsy samples taken during upper gastrointestinal endoscopy have been required for diagnosis. Although biopsies remain advised as necessary for the diagnosis of coeliac disease in adults, European guidelines for children provide a biopsy-sparing diagnostic pathway. This approach has been enabled by the high specificity and sensitivity of serological testing. However, these guidelines are not universally accepted. In this Perspective, we discuss the pros and cons of a biopsy-avoiding pathway for the diagnosis of coeliac disease, especially in this current era of the call for more biopsies, even from the duodenal bulb, in the diagnosis of coeliac disease. In addition, a contrast between paediatric and adult guidelines is presented.

Adherence to Celiac Disease and Eosinophilic Esophagitis Biopsy Guidelines Is Poor in Children.

OBJECTIVES: Celiac disease (CD) and eosinophilic esophagitis (EoE) are underdiagnosed gastrointestinal conditions, which adversely affect children's health. Previous studies have shown that diagnostic guidelines for CD are not consistently followed in adults. The aims of the present study are to assess the frequency with which endoscopists comply with diagnostic guidelines for CD and EoE in children, and to determine whether an association exists between adherence to biopsy guidelines and disease detection in pediatric patients. METHODS: We reviewed pathology reports from 9171 children (ages 0-18) with at least 1 duodenal biopsy, and 8280 children with at least 1 esophageal biopsy, with specimens submitted to a national pathology laboratory. Frequency of adherence to diagnostic guidelines and recommendations for CD and EoE were determined, and the effect of this upon detection of CD and EoE. RESULTS: Overall, 35% of cases were biopsied according to the 2006 American Gastroenterological Association guidelines for CD diagnosis; 8% were biopsied according to the 2007 American Gastroenterological Association EoE consensus recommendations. Detection of CD and EoE increased with the number of biopsies collected (P for trend in each <0.001). Adherence to diagnostic guidelines was particularly poor among those found to have histologically normal mucosa in both cohorts. The likelihood of CD and EoE diagnosis was significantly associated with adherence to diagnostic guidelines (odds ratio for CD 6.3, 95% confidence interval 4.4-8.9; odds ratio for EoE 2.4, 95% confidence interval 1.9-2.9). CONCLUSION: Adherence to established guidelines is poor, and improved guideline adherence is associated with greater disease detection rates for CD and EoE.

Celiac Disease Does Not Influence Fracture Risk in Young Patients with Type 1 Diabetes.

OBJECTIVES: To examine the risk of any fractures in patients with both type 1 diabetes (T1D) and celiac disease (CD) vs patients with T1D only. STUDY DESIGN: We performed a population-based cohort study. We defined T1D as individuals aged ≤30 years who had a diagnosis of diabetes recorded in the Swedish National Patient Register between 1964 and 2009. Individuals with CD were identified through biopsy report data between 1969 and 2008 from any of Sweden's 28 pathology departments. Some 958 individuals had both T1D and CD and were matched for sex, age, and calendar period with 4598 reference individuals with T1D only. We then used a stratified Cox regression analysis, where CD was modeled as a time-dependent covariate, to estimate the risk of any fractures and osteoporotic fractures (hip, distal forearm, thoracic and lumbar spine, and proximal humerus) in patients with both T1D and CD compared with that in patients with T1D only. RESULTS: During follow-up, 12 patients with T1D and CD had a fracture (1 osteoporotic fracture). CD did not influence the risk of any fracture (adjusted hazard ratio = 0.77; 95% CI = 0.42-1.41) or osteoporotic fractures (adjusted hazard ratio = 0.46; 95% CI = 0.06-3.51) in patients with T1D. Stratification for time since CD diagnosis did not affect risk estimates. CONCLUSION: Having a diagnosis of CD does not seem to influence fracture risk in young patients with T1D. Follow-up in this study was, however, too short to ascertain osteoporotic fractures which traditionally occur in old age.

Pondering the potential of quantitative analysis of video capsule endoscopy images in the management of children with celiac disease.

Celiac disease is common throughout the world, affecting approximately 1% of patients of all age groups. In this review, the role of video capsule endoscopy in characterizing the small intestinal lumen of pediatric patients with celiac disease is discussed in detail. Quantitative aspects of video capsule endoscopy which may be helpful in diagnosing pediatric patients are highlighted.

Gastrojejunal tube placement through an established gastrostomy via an endoscopic transgastric approach in a pediatric population.

BACKGROUND AND AIMS: Gastrojejunal feeding tubes (GJTs) are typically converted from gastrostomy feeding tubes by interventional radiology in many pediatric centers to provide both postpyloric feeding and gastric decompression. Endoscopic transgastric GJT placement via an established gastric stoma can be performed without sedation and with minimal fluoroscopy but is relatively new in pediatrics with limited description. This study analyzed the success rate, adverse events, and technical issues associated with endoscopic GJT placement via a transgastric approach in pediatric patients at a large children's hospital. METHODS: We retrospectively reviewed endoscopic GJT placements in pediatric patients performed over a 16-month period at the Children's Hospital of New York-Presbyterian, Columbia University Medical Center. Indication for GJT placement, patient demographic characteristics and medical history, use of sedation, fluoroscopy time, and procedural and postprocedural adverse events were assessed. RESULTS: A total of 47 GJT placements were performed, all successful, in a patient cohort with a mean age of 8 years. The mean fluoroscopy time was 10 seconds, and sedation was used in 30% of placements. In 8 patients who had undergone GJT placement by endoscopy and interventional radiology, the fluoroscopy time was significantly reduced by using the endoscopic method (10 seconds vs 299 seconds, P = .001). CONCLUSIONS: Endoscopic transgastric GJT placement via an established gastrostomy with fluoroscopic confirmation can be safely performed by pediatric gastroenterologists without sedation and with minimal radiation exposure.

Increased risk of non-alcoholic fatty liver disease after diagnosis of celiac disease.

BACKGROUND & AIMS: Non-alcoholic fatty liver disease is a common cause of chronic liver disease. Celiac disease alters intestinal permeability and treatment with a gluten-free diet often causes weight gain, but so far there are few reports of non-alcoholic fatty liver disease in patients with celiac disease. METHODS: Population-based cohort study. We compared the risk of non-alcoholic fatty liver disease diagnosed from 1997 to 2009 in individuals with celiac disease (n = 26,816) to matched reference individuals (n = 130,051). Patients with any liver disease prior to celiac disease were excluded, as were individuals with a lifetime diagnosis of alcohol-related disorder to minimize misclassification of non-alcoholic fatty liver disease. Cox regression estimated hazard ratios for non-alcoholic fatty liver disease were determined. RESULTS: During 246,559 person-years of follow-up, 53 individuals with celiac disease had a diagnosis of non-alcoholic fatty liver disease (21/100,000 person-years). In comparison, we identified 85 reference individuals diagnosed with non-alcoholic fatty liver disease during 1,488,413 person-years (6/100,000 person-years). This corresponded to a hazard ratio of 2.8 (95% CI 2.0-3.8), with the highest risk estimates seen in children (HR = 4.6; 95% CI 2.3-9.1). The risk increase in the first year after celiac disease diagnosis was 13.3 (95% CI 3.5-50.3) but remained significantly elevated even beyond 15 years after the diagnosis of celiac disease (HR = 2.5; 95% CI 1.0-5.9). CONCLUSION: Individuals with celiac disease are at increased risk of non-alcoholic fatty liver disease compared to the general population. Excess risks were highest in the first year after celiac disease diagnosis, but persisted through 15 years after diagnosis with celiac disease.

Should intussusception in children prompt screening for celiac disease?

OBJECTIVES: An association between adult celiac disease (CD) and intussusceptions (ISs) has been described. Although more common among children, intussusception has not been linked with childhood CD aside from isolated case reports. Our aim was to investigate the frequency of IS among children with CD. METHODS: A patient database containing children with biopsy-proven CD was reviewed, in addition to radiology records contained in a hospital-maintained clinical data repository. RESULTS: Of 254 children with biopsy-proven CD and complete records available for review, abdominal imaging was performed in 21%, mainly because of abdominal pain. Among children with CD, 1.2% experienced an IS <9 months before their diagnosis with CD. Among children seen at our institution in the same time period, 0.07% experienced an IS. The majority of those children with CD who were found to have IS had no evidence of nutritional deficit at the time of IS. IS was not identified in any children with CD who had been treated with a gluten-free diet. CONCLUSIONS: IS was far more common among children in our cohort with untreated CD than in the general pediatric population simultaneously seen at our center. The diagnosis of CD should be considered in children with IS, even in the absence of signs of nutritional compromise.