Norelle R. Reilly

Celiac disease in normal-weight and overweight children: clinical features and growth outcomes following a gluten-free diet.

Objectives: There are few data on pediatric celiac disease in the United States. The aim of our study was to describe the presentation of celiac disease among children with a normal and an elevated body mass index (BMI) for age, and to study their BMI changes following a gluten-free diet (GFD). Patients and Methods: One hundred forty-two children (age 13 months–19 years) with biopsy-proven celiac disease, contained in a registry of patients studied at our center from 2000 to 2008, had follow-up growth data available. Patients’ height, weight, and BMI were converted to z scores for age and grouped by BMI as underweight, normal, and overweight. Compliance was confirmed using results of serological assays, and data of noncompliant patients were analyzed separately. Data were analyzed during the observation period and were expressed as change in height, weight, and BMI z score per month of dietary treatment. Results: Nearly 19% of patients had an elevated BMI at diagnosis (12.6% overweight, 6% obese) and 74.5% presented with a normal BMI. The mean duration of follow-up was 35.6 months. Seventy-five percent of patients with an elevated BMI at diagnosis decreased their BMI z scores significantly after adherence to a GFD, normalizing it in 44% of cases. Of patients with a normal BMI at diagnosis, weight z scores increased significantly after treatment, and 13% became overweight. Conclusions: Both normal weight and overweight frequently occur in North American children presenting with celiac disease. A GFD may have a beneficial effect upon the BMI of overweight and obese children with celiac disease.

Immunoglobulin A deficiency in celiac disease.

Goals: To determine the prevalence and significance of immunoglobulin A (IgA) deficiency and partial deficiency in patients with celiac disease (CD). Background: Selective IgA deficiency is a common primary immunoglobulin deficiency and has a higher prevalence in patients with CD. The prevalence and significance of IgA deficiency and partial deficiency in patients with CD in the United States has not previously been examined. Study: A retrospective, cohort study of 1498 adults and 317 children seen in a University Medical Center was conducted. Results: There were 26 patients (22 adults, 4 children) with CD who were IgA deficient and 11 (9 adults, 2 children) with CD who were partially IgA deficient. The prevalence of IgA deficiency/partial deficiency was similar among adults and children (2.1% and 1.9%, respectively, P=0.99). Among adults, concomitant autoimmune disease was present in 29% of IgA-deficient/partially deficient patients versus 12% of CD patients with normal IgA levels (P=0.0081). All 4 IgA-deficient patients who had persistently positive IgG celiac serologies while adherent to a gluten-free diet and were rebiopsied had a normal repeat biopsy. Both positive tissue transglutaminase IgG and antigliadin IgG were found in these patients. Conclusions: Selective IgA deficiency/partial deficiency is present in 2% of CD patients at this referral center and is equally prevalent among adults and children. IgA-deficient/partially deficient adults had a higher prevalence of concomitant autoimmune disease than those without IgA deficiency. In patients who are IgA deficient, IgG serologies may be persistently elevated despite histologic recovery.

Presentation of Celiac Disease

The mode of presentation of patients with celiac disease has changed dramatically over the recent decades, with diarrheal or classic presentations becoming less common. This trend is most markedly seen in children, whose main presentations include recurrent abdominal pain, growth issues, and screening groups at risk. Among adults, presentations include diarrhea, anemia, osteoporosis, and recognition at endoscopy performed for gastroesophageal reflux disease, as well as screening. The groups most commonly screened include family members of patients with celiac disease, Down syndrome, and autoimmune diseases.

Increased incidence of eosinophilic esophagitis in children and adults with celiac disease.

Goals and Background Case series have suggested an association between eosinophilic esophagitis (EoE) and celiac disease (CD) in children. We analyzed a cohort of patients with CD to confirm this association in children, and determine whether it extends into adulthood. Methods A database of patients with CD was reviewed to determine the number of patients with comorbid diagnoses of EoE. Histopathology reports of esophageal biopsies were reviewed to identify all cases of increased esophageal eosinophilia. Cases of EoE were diagnosed if biopsies revealed ≥15 eosinophils per high power field and associated symptoms were present. Age-adjusted and sex-adjusted standardized incidence ratios (SIR) with corresponding 95% confidence intervals (CI) were calculated in comparison to published US population-derived incidence data. Results EoE was diagnosed in 4 children and 10 adults. EoE is more common compared with the general population; SIR for children was 35.6 (95% CI, 9.3-79.0) and for adults 13.1 (95% CI, 6.2-22.5). Overall, the age-adjusted and sex-adjusted SIR was 16.0 (95% CI, 8.7-25.5). Conclusions The incidence of EoE in our cohort of patients with CD was increased compared with the general population. Coexistent EoE should be considered in patients with CD who have persistent esophageal symptoms.

The Gluten-Free Diet: Recognizing Fact, Fiction, and Fad

T he gluten-free diet (GFD) is a critical medical treatment for the millions of individuals worldwide with celiac disease (CD), an autoimmune condition for which no other therapy is currently available. The prevalence of CD is increasing, reflected by escalating awareness of CD in the scientific community. This increase in disease prevalence and awareness of CD, however, does not account for the disproportionate increase in growth of the gluten-free food industry (Figure). According to market research, consumers without CD purchase the vast bulk of glutenfree products. In reality, remarkably little is known about the motives of most individuals who adopt a gluten-free lifestyle. According to a 2015 survey of more than 1500 American adults, “no reason” (35%) was the most common explanation for selecting gluten-free foods, followed by “healthier option” (26%), and “digestive health” (19%). “Someone in my family has a gluten sensitivity” (10%) was more common than those reporting, “I have a gluten sensitivity,” which was the least common rationale cited (8%). The increasing popularity of the GFD has important implications for children. Parents sometimes place their children on a GFD in the belief that it relieves symptoms, can prevent CD, or is a healthy alternative without previous testing for CD or consultation with a dietitian. Although some children experience relief of symptoms, signifying that CD testing is warranted, many are asymptomatic from the start. The health and social consequences worthy of consideration in advance of starting a child on a GFD are not described adequately online or in books promoting an empiric GFD trial. This Commentary will provide an update on the current GFD fad and will disentangle facts from commonly held beliefs regarding the GFD, its known benefits, and disadvantages, specifically addressing several issues related to children.

Fecal Microbiota Transplantation in Children With Recurrent clostridium difficile Infection

Clostridium difficile eradication using fecal microbiota transplantation (FMT) has been successful in adults but little information is available in pediatrics. We report 6 pediatric patients with refractory C. difficile cured by FMT with no recurrences to date. Our results demonstrate that FMT can be an effective treatment for refractory C. difficile infection in pediatrics. Long-term safety and efficacy need to be studied.

NASPGHAN Clinical Report on the Diagnosis and Treatment of Gluten-related Disorders.

Dietary exclusion of gluten-containing products has become increasingly popular in the general population, and currently ∼30% of people in the United States are limiting gluten ingestion. Although celiac disease (CD), wheat allergy (WA), and nonceliac gluten sensitivity (NCGS) constitute a spectrum of gluten-related disorders that require exclusion of gluten from the diet, together these account for a relatively small percentage of those following a gluten-free diet, and the vast majority has no medical necessity for doing so. Differentiating between CD, WA, and NCGS has important prognostic and therapeutic implications. Because of the protean manifestations of gluten-related disorders, it is not possible to differentiate between them on clinical grounds alone. This clinical report will compare and contrast the manifestations of gluten-related disorders, emphasize the importance of differentiating between these conditions, discuss initial and subsequent tests needed to confirm the diagnosis, and provide recommendations on treatment and follow-up for each condition.

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.

Transition from childhood to adulthood in coeliac disease: the Prague consensus report

The process of transition from childhood to adulthood is characterised by physical, mental and psychosocial development. Data on the transition and transfer of care in adolescents/young adults with coeliac disease (CD) are scarce. In this paper, 17 physicians from 10 countries (Sweden, Italy, the USA, Germany, Norway, the Netherlands, Australia, Britain, Israel and Denmark) and two representatives from patient organisations (Association of European Coeliac Societies and the US Celiac Disease Foundation) examined the literature on transition from childhood to adulthood in CD. Medline (Ovid) and EMBASE were searched between 1900 and September 2015. Evidence in retrieved reports was evaluated using the Grading of Recommendation Assessment, Development and Evaluation method. The current consensus report aims to help healthcare personnel manage CD in the adolescent and young adult and provide optimal care and transition into adult healthcare for patients with this disease. In adolescence, patients with CD should gradually assume exclusive responsibility for their care, although parental support is still important. Dietary adherence and consequences of non-adherence should be discussed during transition. In most adolescents and young adults, routine small intestinal biopsy is not needed to reconfirm a childhood diagnosis of CD based on European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) or North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) criteria, but a biopsy may be considered where paediatric diagnostic criteria have not been fulfilled, such as, in a patient without biopsy at diagnosis, additional serology (endomysium antibody) has not been performed to confirm 10-fold positivity of tissue transglutaminase antibodies or when a no biopsy strategy has been adopted in an asymptomatic child.