Imad Absah

MR enterography in pediatric inflammatory bowel disease: retrospective assessment of patient tolerance, image quality, and initial performance estimates.

OBJECTIVE The purpose of this article was to evaluate image quality, oral contrast administration and bowel distention, side effects, and performance estimates of MR enterography in a large cohort of pediatric patients with inflammatory bowel disease (IBD). MATERIALS AND METHODS A retrospective analysis of the pediatric IBD clinic database (2007-2010) was performed. Eighty-five MR enterography studies in 70 patients were performed without sedation. All pediatric patients with the potential diagnosis of IBD were included, with the exception of studies performed on patients with ileoanal pouch anatomy. The quantity of ingested oral contrast material and number of adverse events were recorded. Retrospectively, image quality (including motion artifact and bowel distention) and enteric inflammation were assessed. Correlation between radiographic findings and endoscopic findings was tested by the Fisher exact test. RESULTS Eighty-five MR enterography studies were performed without sedation in 70 patients (mean age, 15.6 years; age range, 9-18 years) over 30 months. The mean image quality for unenhanced images was significantly higher than for contrast-enhanced images (4.7 vs 4.1, p < 0.0001), with unacceptable image quality occurring on both unenhanced and contrast-enhanced sequences in one patient. The amount of oral contrast material ingested correlated with patient age (p = 0.009), with acceptable bowel distention occurring in 93% (78/85). Two patients had nausea or emesis and one experienced a syncopal episode after MR enterography. Sensitivity and specificity of MR enterography for active disease of the terminal ileum, right colon, and left colon were 80% and 85.2%, 79.1% and 77.8%, and 90.3% and 63.6%, respectively. CONCLUSION MR enterography is feasible in patients 9 years old and older without sedation. Acceptable image quality can be achieved in nearly all patients, but a small minority will have suboptimal bowel distention or minor adverse events. Oral contrast ingestion regimes can be based on patient age. Performance estimates in children parallel reports in adults.

Concomitant therapy with methotrexate and anti‐TNF‐α in pediatric patients with refractory crohn's colitis: A case series

Background: Crohns colitis refractory to anti‐tumor necrosis factor alpha (TNF‐&agr;) therapy is commonly seen in tertiary care centers for pediatric inflammatory bowel disease (IBD). We report our experience in managing pediatric refractory Crohns colitis with concomitant use of methotrexate and anti‐TNF‐&agr; therapy. Methods: We reviewed records from 2007 to 2010 at the Mayo Clinic pediatric IBD center. We included all patients with Crohns disease (CD) failing anti‐TNF‐&agr; therapy who then received concomitant methotrexate. The primary endpoint was clinical remission, defined as inactive disease in accordance with the short pediatric CD activity index (PCDAI). The secondary endpoint was last day of follow‐up. Results: Fourteen patients with CD received concomitant methotrexate and anti‐TNF‐&agr; treatment (age, mean [range], 15.7 [6–20] years; standard deviation [SD], 3.4 years). Mean age at diagnosis was 12.5 years (range, 3–17 years; SD, 3.83 years). The male‐to‐female ratio was 10:4. All patients had moderate to severe disease activity using the short PCDAI and had predominately Crohns colitis. Twelve patients were previously treated with thiopurines (85.7%). Seven patients (50%) were in clinical remission within an average of 6 weeks postmethotrexate induction. Five patients (35.7%) experienced adverse events including nausea and headache, yet only one discontinued therapy due to adverse events. Infection with Clostridium difficile was common, complicating therapy in four patients (28.6%). Conclusions: Concomitant use of methotrexate and anti‐TNF‐&agr; therapy is a promising option for children with refractory Crohns colitis. (Inflamm Bowel Dis 2012)

Mucosal healing in children with treated celiac disease

Objectives:Limited data suggest complete mucosal healing in treated children with celiac disease (CD), but recent data from adult endoscopic biopsies have shown substantial numbers with persistent mucosal injury. We aimed to assess the rate of mucosal healing and indications for repeat small bowel (SB) biopsy in children with CD. Methods:We retrospectively reviewed records of children (ages 1–18 years) with CD who underwent a second SB biopsy. All of the children were seen at Mayo Clinic (Rochester, MN) from January 1997 through June 2013. Results:Forty children were identified (14 boys); average age at diagnosis was 8.5 years. Indications for second SB biopsy were abdominal pain (n = 20), diarrhea (n = 7), constipation (n = 5), non–celiac-related concern (n = 2), follow-up (n = 5), and persistent serology (n = 1). Average time between biopsies was 24 months (range 4–120 months). Histology on the second biopsy showed complete healing (n = 25), intraepithelial lymphocytes (n = 9), and persistent villous atrophy (n = 6). Of these, 3 patients had partial villous atrophy and 3 had with complete villous atrophy. Persistent villous atrophy was observed in 2 of 20 patients with abdominal pain and 1 of 7 with diarrhea. All of the patients with persistent constipation (n = 5) had complete resolution. Conclusions:Mucosal healing in children with CD may not be complete as previously assumed. Abdominal pain was the most common indication for repeating the SB biopsy. Persistence of abdominal pain, diarrhea, and constipation was poorly associated with persistence of mucosal injury.

The Association Between Celiac Disease and Eosinophilic Esophagitis: Mayo Experience and Meta-analysis of the Literature

Background: The association between celiac disease (CD) and eosinophilic esophagitis (EoE) has been the focus of multiple studies with variable results. Both diseases are immune mediated, and dietary triggers play a role in their pathogenesis. Objectives: The aim of the study was to analyze the risk of EoE in children with CD, assess the magnitude of association between CD and EoE in children, and report the characteristics and outcomes of children with both conditions. Methods: We conducted a retrospective study of the Mayo Clinic Electronic medical records between January 1, 1998 and December 31, 2015. Systematic review and meta-analysis of multiple databases was conducted to include studies reporting on the same association. Random-effects model was used to report pooled odds ratio (OR) and 95% confidence interval (CI). Results: In this cohort study, of 10,201 children who underwent at least 1 endoscopy, 595 had EoE, and 546 had CD. The risk of having EoE was not increased in children with CD compared to those without CD (OR, 0.29; 95% CI, 0.154–0.545). Nine of 10 children improved with gluten-free diet, topical glucocorticosteroid, and/or elimination diet. One child lost to follow-up. Meta-analysis of 5 studies showed similar results (OR, 0.525; 95% CI, 0.364–0.797). A total of 45 cases in the literature had both CD and EoE (mean age, 10 years; 64% boys; majority presenting with abdominal pain, vomiting, and diarrhea). Conclusions: Based on our cohort and the observational data, the diagnosis of CD in children is not associated with increased risk of EoE.

Rumination syndrome: pathophysiology, diagnosis, and treatment

Rumination syndrome is a functional gastrointestinal disorder characterized by effortless and repetitive regurgitation of recently ingested food from the stomach to the oral cavity followed by either re‐swallowing or spitting. Rumination is thought to occur due to a reversal of the esophagogastric pressure gradient. This is achieved by a coordinated abdominothoracic maneuver consisting of a thoracic suction, crural diaphragm relaxation and an increase in intragastric pressure. Careful history is important in the diagnosis of rumination syndrome; patients often report “vomiting” or “reflux” and the diagnosis can therefore be missed. Objective testing is available with high resolution manometry or gastroduodenal manometry. Increase in intra‐gastric pressure followed by regurgitation is the most important characteristic to distinguish rumination from other disorders such as gastroesophageal reflux. The mainstay of the treatment of rumination syndrome is behavioral therapy via diaphragmatic breathing in addition to patient education and reassurance.

Is it necessary to assess for fat-soluble vitamin deficiencies in pediatric patients with newly diagnosed celiac disease?

Background:The purpose of this study was to identify the frequency of fat-soluble vitamin deficiencies in children with celiac disease (CD) and to determine the value of routine testing for these deficiencies. Methods:We conducted a retrospective medical record review of patients with a confirmed diagnosis of CD and fat-soluble vitamin levels measured at diagnosis between 1995 and 2012 at Mayo Clinic. Patients’ demographics, fat-soluble vitamin levels, and pertinent clinical factors at the time of diagnosis were collected. Results:Eighty-three patients were included in the final analysis: 51 girls and 32 boys, with an average age at diagnosis of 12.8 years in girls and 13.0 years in boys. The most commonly reported symptoms were abdominal pain in 49 patients and diarrhea in 30 patients. Family history of CD was reported in 32 patients. Average vitamin levels for vitamin E, 25-hydroxyvitamin D (25 (OH) D), and vitamin A were 7.5 mg/L, 32.8 ng/mL, and 334.5 &mgr;g/dL, respectively. No patients had vitamin A deficiency, 2 patients had vitamin E deficiency, and 9 patients had mild-to-moderate vitamin D deficiency (none had severe deficiency). Both patients with vitamin E deficiency were symptomatic and had complete villous atrophy. Thirty-one patients had insufficiency of 25 (OH) D, which was less than the reported frequency of vitamin D insufficiency in the general pediatric population in the United States in 2004. None of the patients were receiving vitamin supplements at the time of diagnosis. Conclusions:Fat-soluble vitamin deficiencies are uncommon in children with new diagnosis of CD. Routine measuring of fat-soluble vitamins levels may not be necessary.

Increasing Incidence and Altered Presentation in a Population-based Study of Pediatric Celiac Disease in North America

Objectives: Celiac disease (CD) is a common immune-mediated disorder that affects up to 1% of the general population. Recent reports suggest that the incidence of CD has reached a plateau in many countries. We aim to study the incidence and altered presentation of childhood CD in a well-defined population. Methods: Using the Rochester Epidemiology Project, we retrospectively reviewed Mayo Clinic and Olmsted Medical Center medical records from January 1994 to December 2014. We identified all CD cases of patients ages 18 years or younger at the time of diagnosis. Incidence rates were calculated by adjusting for age, sex, and calendar year and standardizing to the 2010 US white population. Results: We identified 100 patients with CD. Incidence of CD has increased from 8.1 per 100,000 person-years (2000–2002) to 21.5 per 100,000 person-years (2011–2014). There was an increase in CD prevalence in children from 2010 (0.10%) to 2014 (0.17%). Thirty-four patients (34%) presented with classical CD symptoms, 43 (43%) had nonclassical CD, and 23 (23%) were diagnosed by screening asymptomatic high-risk patients. Thirty-six patients (36%) had complete villous atrophy, 51 (51%) had partial atrophy, and 11 (11%) had increased intraepithelial lymphocytes. Two patients were diagnosed without biopsy. Most patients (67%) had a normal body mass index, 17% were overweight/obese, and only 9% were underweight. Conclusions: Both incidence and prevalence of CD have continued to increase in children during the past 15 years in Olmsted County, Minnesota. Clinical and pathologic presentations of CD are changing over time (more nonclassical and asymptomatic cases are emerging).

Lack of Utility of Anti-ttg Igg to Diagnose Celiac Disease When Anti-ttg Iga Is Negative

Objectives: Guidelines for diagnosing celiac disease (CD) recommend initial testing with a highly sensitive serologic test for anti–tissue transglutaminase immunoglobulin A antibodies (tTG IgA). When the probability of CD is high, IgA deficiency should be considered. The 2 approaches to address this include measuring “both tTG IgA and tTG IgG” or measuring “total IgA.” We aim to assess the utility of an isolated positive tTG IgG result in diagnosing CD. Methods: We conducted a retrospective review of patients undergoing serologic testing for CD from January 1997 to June 2014. Patients with positive tTG IgG and negative tTG IgA were included. Moreover, all patients who had any other positive CD-specific serologic findings were excluded. Demographics, clinical presentation, tests, and biopsy results were recorded. Results: The indication for checking celiac serology was gastrointestinal symptoms in 172 of 233 patients, iron deficiency anemia in 12, and high-risk screening in 48. Small bowel biopsy was performed in 178 patients (77%); 160 had normal results and 18 had histologic changes suggestive of enteropathy. Nine patients had increased intraepithelial lymphocytes, and 9 had partial villous atrophy. Only 6 cases of CD were, however, confirmed. The utility of isolated tTG IgG in diagnosis of CD was low at 3% (6/178). Conclusion: In this cohort of patients, the utility of isolated tTG IgG in diagnosing CD was low at 3%.

Successful Use of Esophageal Stent Placement to Treat a Postoperative Esophageal Stricture in a Toddler.

Esophageal atresia (EA) is the most common type of gastrointestinal atresia. The most common variant (type C) consists of a blind esophageal pouch with a fistula between the trachea and the distal esophagus. Surgical repair can be complicated by the development of benign stricture. Most strictures are amenable to dilation, but refractory strictures may require surgical intervention. A 24-month-old boy born with tracheoesophageal fistula and EA underwent surgical repair on day 1 of life. He developed esophageal stricture that responded to esophageal stent placement. Endoscopic biliary accessories can be safely used to dilate refractory esophageal strictures in children, and should be considered prior to seeking other complex alternatives.

Monitoring and Follow-Up of Patients with Celiac Disease

The goals of medical treatment of celiac disease (CD) are to: (1) encourage dietary compliance, (2) manage and monitor symptoms, and (3) prevent and detect associated diseases and/or complications. The underlying aim in the care of patients with CD is to ensure that patients have initial and sustained symptomatic improvement and to prevent long-term sequelae that can result from chronic gluten exposure. Currently, available guidelines on the follow-up of patients with CD are often incomplete or variable, partially due to a dearth of research on this topic. This chapter synthesizes available guidelines and expert opinion to recommend the essential components of medical follow-up of CD patients for practicing physicians.