Ivor D. Hill

Guideline for the diagnosis and treatment of celiac disease in children: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition.

Celiac disease is an immune-mediated enteropathy caused by a permanent sensitivity to gluten in genetically susceptible individuals. It occurs in children and adolescents with gastrointestinal symptoms, dermatitis herpetiformis, dental enamel defects, osteoporosis, short stature, delayed puberty and persistent iron deficiency anemia and in asymptomatic individuals with type 1 diabetes, Down syndrome, Turner syndrome, Williams syndrome, selective immunoglobulin (Ig)A deficiency and first degree relatives of individuals with celiac disease. The Celiac Disease Guideline Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition has formulated a clinical practice guideline for the diagnosis and treatment of pediatric celiac disease based on an integration of a systematic review of the medical literature combined with expert opinion. The Committee examined the indications for testing, the value of serological tests, human leukocyte antigen (HLA) typing and histopathology and the treatment and monitoring of children with celiac disease. It is recommended that children and adolescents with symptoms of celiac disease or an increased risk for celiac disease have a blood test for antibody to tissue transglutaminase (TTG), that those with an elevated TTG be referred to a pediatric gastroenterologist for an intestinal biopsy and that those with the characteristics of celiac disease on intestinal histopathology be treated with a strict gluten-free diet. This document represents the official recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition on the diagnosis and treatment of celiac disease in children and adolescents.

ACG clinical guidelines: diagnosis and management of celiac disease.

This guideline presents recommendations for the diagnosis and management of patients with celiac disease. Celiac disease is an immune-based reaction to dietary gluten (storage protein for wheat, barley, and rye) that primarily affects the small intestine in those with a genetic predisposition and resolves with exclusion of gluten from the diet. There has been a substantial increase in the prevalence of celiac disease over the last 50 years and an increase in the rate of diagnosis in the last 10 years. Celiac disease can present with many symptoms, including typical gastrointestinal symptoms (e.g., diarrhea, steatorrhea, weight loss, bloating, flatulence, abdominal pain) and also non-gastrointestinal abnormalities (e.g., abnormal liver function tests, iron deficiency anemia, bone disease, skin disorders, and many other protean manifestations). Indeed, many individuals with celiac disease may have no symptoms at all. Celiac disease is usually detected by serologic testing of celiac-specific antibodies. The diagnosis is confirmed by duodenal mucosal biopsies. Both serology and biopsy should be performed on a gluten-containing diet. The treatment for celiac disease is primarily a gluten-free diet (GFD), which requires significant patient education, motivation, and follow-up. Non-responsive celiac disease occurs frequently, particularly in those diagnosed in adulthood. Persistent or recurring symptoms should lead to a review of the patients original diagnosis to exclude alternative diagnoses, a review of the GFD to ensure there is no obvious gluten contamination, and serologic testing to confirm adherence with the GFD. In addition, evaluation for disorders associated with celiac disease that could cause persistent symptoms, such as microscopic colitis, pancreatic exocrine dysfunction, and complications of celiac disease, such as enteropathy-associated lymphoma or refractory celiac disease, should be entertained. Newer therapeutic modalities are being studied in clinical trials, but are not yet approved for use in practice. Given the incomplete response of many patients to a GFD-free diet as well as the difficulty of adherence to the GFD over the long term, development of new effective therapies for symptom control and reversal of inflammation and organ damage are needed. The prevalence of celiac disease is increasing worldwide and many patients with celiac disease remain undiagnosed, highlighting the need for improved strategies in the future for the optimal detection of patients.

Detection of Celiac Disease in Primary Care: A Multicenter Case-Finding Study in North America

BACKGROUND:Celiac disease (CD) is one of the most common lifelong disorders in western countries. However, most cases remain currently undiagnosed in North America, mostly due to poor awareness of CD by primary care physicians.OBJECTIVES:The aims of this study were (a) to determine whether an active case-finding strategy in primary care could increase the frequency of CD diagnosis and (b) to determine the most common clinical presentations of the condition.METHODS:This was a multicenter, prospective study involving adult subjects during the years 2002–2004, attending one of the participating practices. All individuals with symptoms or conditions known to be associated with CD were tested for immunoglobulin A anti-transglutaminase (tTG) antibodies, and those with elevated anti-tTG were subsequently tested for IgA antiendomysial antibodies (EMA). All subjects who were positive for EMA were advised to undergo an intestinal biopsy and HLA typing.RESULTS:The study group included 737 women and 239 men, with a median age of 54.3 yr. A positive anti-tTG test was found in 30 out of 976 investigated subjects (3.07%, 95% CI 1.98–4.16). CD was diagnosed in 22 patients (18 women, 4 men). The most frequent reasons for CD screening in these 22 cases were bloating (12/22), thyroid disease (11/22), irritable bowel syndrome (7/22), unexplained chronic diarrhea (6/22), chronic fatigue (5/22), and constipation (4/22). The prevalence of CD in the serologically screened sample was 2.25% (95% CI 1.32–3.18). The diagnostic rate was low at baseline (0.27 cases per thousand visits, 95% CI 0.13–0.41) and significantly increased to 11.6 per thousand visits (95% CI 6.8–16.4, P < 0.001) following active screening implementation.CONCLUSIONS:This study demonstrates that an active case-finding strategy in the primary care setting is an effective means to improve the diagnostic rate of CD in North America.

The prevalence of celiac disease in at-risk groups of children in the United States.

OBJECTIVE In contrast to its prevalence in Europe, celiac disease (CD) is considered rare in the United States. We aimed to determine the prevalence of CD in children presenting with symptoms or conditions associated with CD. STUDY DESIGN Individuals aged 6 months to 20 years were screened for IgG and IgA antigliadin (AGA-IgG and AGA-IgA) and antiendomysium (EMA) antibodies. Those with only elevated AGA-IgG were screened for selective IgA deficiency. Patients with elevated EMA, or AGA-IgG elevation and selective IgA deficiency, were advised to undergo small intestinal biopsy. RESULTS A total of 1200 individuals were studied; 34 were EMA positive-26 (19 EMA positive) consented to biopsy and 21 had CD, giving a prevalence of 1 in 57 (21/1200). Including the 15 EMA positive patients who refused a biopsy, the prevalence of CD in this study could be as high as 1 in 33 (36/1200). CONCLUSIONS CD is not rare in the United States and may be as common as in Europe. AGA and EMA are useful for identifying patients who should undergo a small intestinal biopsy.

Stool antigen test for diagnosis of Helicobacter pylori infection in Children with symptomatic disease: A prospective study

Objective: Noninvasive tests for the diagnosis of Helicobacter pylori (Hp) infection in children are limited by low accuracy rates and lack of validation. Existing studies indicate that the stool antigen test (HpSA) has an acceptable level of accuracy for the diagnosis of Hp infection in adults but not children. The aim of this study was to evaluate the accuracy of the HpSA test for the detection of Hp infection in U.S. children. Methods: Children requiring upper endoscopic procedures were prospectively recruited from two pediatric gastroenterology clinics. Stool samples were collected from each participant before endoscopy. The presence of Hp infection was determined by positive histologic findings and positive rapid urease test (RUT). The presence of Hp organisms in stool was determined by an enzyme-linked immunosorbent assay using a commercially available polyclonal antibody kit (Meridian Diagnostics, Cincinnati, OH, U.S.A.). Results of the stool antigen test were compared with histology findings and RUT results. Results: One hundred twenty-one children (mean age, 10.1 ± 3.7 years) participated, of whom 9 (7.4%) had Hp infection. Histologic findings and RUT results were concordant in 95% of the children. Per study protocol, HpSA had a sensitivity, specificity, positive and negative predictive value, and accuracy rate of 67%, 99%, 86%, 97%, and 96.5%, respectively. Conclusion: HpSA, a polyclonal antibody test, had a low sensitivity for infection in children in the United States and at present cannot replace histologic findings as the gold standard for the diagnosis of Hp infection in the pediatric population.

Celiac disease and other immunologically mediated disorders of the gastrointestinal tract: Working Group report of the second World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition.

Celiac DiseaseResearchTreatment alternatives to the gluten-free diet.Definition of new diagnostic criteria.Practical interventions to improve child healthImproved serologic testing.Worldwide accepted definition of gluten-free, labeling issues, availability and measures to monitor and assure compliance.Educational needs and strategies to improve medical knowledgeCeliac disease awareness campaign Food AllergyResearchIdentification of the whole spectrum of disorders due to food allergy.InterventionIdentification and implementation of prevention strategies for food allergy.Educational needs and strategies to improve medical knowledgeDiagnosis of food allergy, with a specific warning against non-orthodox diagnostic tests.

Laparoscopic and thoracoscopic esophagomyotomy for children with achalasia.

Background Minimally invasive esophagomyotomy, consisting of a laparoscopic or thoracoscopic approach, has become a preferred surgical treatment for adults with achalasia. This multicenter study reports on the clinical status of children who have undergone minimally invasive esophagomyotomy for achalasia. Methods Symptomatology for achalasia was assessed in 22 pediatric patients who underwent minimally invasive esophagomyotomy for achalasia between 1995 and 2000. All patients were evaluated for duration of hospitalization, postoperative resumption of feeds, postoperative complications, and symptomatic relief. Participants were assigned pre-and postoperative symptom severity scores ranging from 0 (no symptoms) to 3 (severe). Results The median age of the 10 females and 12 males at time of surgery was 11.3 years ± 3.4 (standard deviation). Transabdominal laparoscopic esophagomyotomy with fundoplication was performed in 18 patients, and thoracoscopic esophagomyotomy without fundoplication was performed in 4. Two patients required conversion from transabdominal laparoscopic esophagomyotomy to open esophagomyotomy because of intraoperative esophageal perforation. The mean duration of postsurgical follow-up was 17 ± 16 (standard deviation) months (range, 1–54 months). Mean duration of hospitalization (days ± standard error or mean) was less for transabdominal laparoscopic esophagomyotomy than for converted open esophagomyotomy (2.7 ± 0.3 vs. 9.0 ± 3.0 days;P < 0.05) or for thoracoscopic esophagomyotomy (4.8 ± 1.7 days;P = not significant). Mean time to resumption of soft feedings (days ± standard error or mean) occurred sooner after transabdominal laparoscopic esophagomyotomy than after converted open esophagomyotomy (2.0 ± 0.2 vs. 5.5 ± 0.5 days;P < 0.001) or after thoracoscopic esophagomyotomy (4.0 ± 1.3 days;P = not significant). Patients experienced significant pre-to postoperative improvement in mean severity score with regard to dysphagia (2.6 vs. 0.4;P < 0.001) and regurgitation (1.7 vs. 0.2;P < 0.001). Conclusions Minimally invasive esophagomyotomy can provide excellent symptomatic relief from dysphagia and regurgitation for children with achalasia.

World perspective on celiac disease.

Received August 30, 201 From the Department o Ancona, Italy, the zIm University School of Haunersches Kindersp wig Maximilian Un University Departm Department of Gastroe Mohammed V, Rabat Rheumatologie und Charité—Universitäts ment of Pediatric G Institute of Medical S Address correspondence Department of Pediat Ancona, Italy (e-mail C.C. has served as scien Schär. The other auth Copyright # 2012 by E Hepatology, and Nut Gastroenterology, He DOI: 10.1097/MPG.0b01

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.

Anti-tissue transglutaminase antibody as the first line screening for celiac disease: good-bye antigliadin tests?

with the results reported with CT-guided pancreatic biopsies. The use of FNA in patients with a focal respectable mass will impact patient management in two aspects. In this series, 27% of the pancreatic malignancies were not adenocarcinomas and in 25 of 32 patients the management was affected by the results of FNA. In another 34 patients who were scheduled for a surgical resection, the results of EUS (liver and lymph node metastases) deemed them unresectable and their surgery was cancelled. Overall, the results of EUS and FNA had an impact on the care of 44% of patients. The results of FNA in patients with chronic pancreatitis demonstrated a reduced sensitivity for detecting malignancy, only 54%. However, the specificity remained very high, 100%. In the clinical scenario of a patient with a pancreatic mass associated with chronic pancreatitis, we cannot rely on the results of FNA when the cytology is negative for malignancy. Instead, the clinical setting will have to dictate the clinical management. In addition to demonstrating a high sensitivity and specificity for EUS-guided FNA, this study has also shown the safety of the procedure. There were no complications of the procedure and there was no evidence of malignant seeding of the needle tract. In previous series, very few complications of FNA have been reported, and they consist of small amounts of bleeding and transient abdominal pain (10). In conclusion, EUS-guided FNA for pancreatic masses is a highly accurate test that will have a significant impact on patient management. The growing acceptance of the test will enable clinicians to diagnose pancreatic malignancies at an early stage. Hopefully, this type of testing will improve the dismal survival often associated with this dreaded malignancy.