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OBJECTIVES No formal comparative effectiveness studies have been conducted to evaluate the effect of eosinophilic esophagitis (EoE) treatment choice on long-term growth in pediatric patients. Long-term studies of inhaled corticoid steroids in asthma, however, suggest possible effects on linear growth. The aim of this study was to compare longitudinal, anthropometric growth in children with EoE according to treatment approach. METHODS We conducted a retrospective, multicenter cohort study of anthropometric growth (height and body mass index [BMI] z scores) in pediatric (

Background Mutations in ITCH, which encodes an E3 ubiquitin-protein ligase, can result in systemic autoimmunity and immunodeficiency. The clinical phenotype and mechanism of disease have not been fully characterized, resulting in a paucity of therapeutic options for this potentially fatal disease. Objective We aimed to (1) expand the understanding about the phenotype of human ITCH deficiency (2) further characterize the associated immune dysregulation, and (3) report the first successful hematopoietic cell transplant (HCT) in a patient with ITCH deficiency. Methods Disease profiling was performed in a patient with multisystem immune dysregulation. Whole exome sequencing with trio analysis and functional validation of candidate disease variants were performed, including mRNA and protein expression. Analyses to further delineate the immunophenotype included quantitative evaluation of lymphoid and myeloid subsets with flow cytometry and mass cytometry. Results A patient with multisystem immune dysregulation presenting with growth failure, very-early-onset inflammatory bowel disease, arthritis, uveitis, psoriasis, and type 1 diabetes mellitus underwent whole exome sequencing, which identified novel compound heterozygous mutations in ITCH. Reduced expression of ITCH mRNA and absent ITCH protein were found. Abnormalities in both lymphoid and myeloid lineages were identified. The patient underwent HCT. He demonstrated excellent immune reconstitution and resolution of many manifestations of his systemic disease. Conclusions Here we report ITCH deficiency with unique clinical features of colonic very-early-onset inflammatory bowel disease, arthritis, and uveitis in the setting of immune dysregulation and further characterize the underlying immune dysregulation. We demonstrate that HCT can be an effective, and potentially curative, therapy for ITCH deficiency.

Elemental diets, dietary elimination, and steroid therapies are the most common therapies in the clinical trials for eosinophilic esophagitis (EoE). Histological findings (usually reported as eosinophils per microscopic high-powered field [hpf]) remain the most common end-point used to define response. Yet, the threshold for defining "response" and "remission" are ill-defined among consensus guidelines and may vary from study to study. We conducted a systematic literature review of articles on eosinophilic esophagitis, published between January 2007 and November 2017, considering histological remission as the primary outcome. We abstracted treatment information and definitions of histological remission or response. A comparison of definitions of histological remission across and within institutions was performed. A total of 61 articles were included in this review, with approximately 60% of the studies published from centers in the United States. Histological definitions of remission of EoE ranged from 0 to ≤20 eosinophils/hpf. The most stringent criteria, ranging from 0 to ≤5 eosinophils/hpf, were commonly used in interventional trial studies that examined the effects of new treatments. We found remarkable variability in definitions between studies, treatment types, and regions. Age or epidemiological distribution of study subjects did not influence the criteria for histological remission. Clinical and histological improvements are important measures of the effects of treatment. Histological findings, the most objective measure of treatment, should provide an optimal method for comparing the effectiveness of various treatments. Yet, our findings suggest a lack of consistent remission criteria in published studies. Considering these inconsistencies, it is difficult to compare the effectiveness of various treatments.

BACKGROUND Eosinophilic esophagitis (EoE) is a chronic disease characterized by infiltration of eosinophils in the esophageal epithelium. There are limited treatment options for EoE. The rationale of the study was to evaluate the long-term safety and efficacy of reslizumab (RSZ) in pediatric patients who received RSZ in a randomized controlled trial (RCT) and expanded access program. METHODS Records of patients who received RSZ in our center were reviewed. Patients received RSZ 2 mg/kg (or placebo) every 4 weeks as part of the RCT, open-label extension (OLE), and compassionate use (CU). Data were analyzed as of their most recent evaluation in August 2017. Labwork, history, and examinations were conducted every 12 weeks. Biopsy results were compared from baseline (before RCT) and at the most recent evaluation. Adverse events (AE) were recorded. RESULTS Twelve patients entered the RCT at our center; 6 patients completed the OLE and 4 received RSZ through CU. Between the RCT, OLE, and CU periods, patients received 549 doses of RSZ (median 37, range 2-116). No serious AE were attributed to RSZ. Symptoms improved on treatment: dysphagia (42% vs 0%), abdominal pain (58% vs 0%), heartburn (18% vs 0%), vomiting (67% vs. 17%), reflux (58% vs. 0%). Median esophageal eosinophil count improved (35 eosinophils per high-power field vs 3, P

Mobile applications and interactive websites are an increasingly used method of telemedicine, but their use lacks evidence in digestive diseases. This study aims to explore digestive disease studies that use telemedicine to effectively manage disease activity, help monitor symptoms, improve compliance to the treatment protocol, increase patient satisfaction, and enhance the patient-to-provider communication. EBSCO, PubMed, and Web of Science databases were searched using Medical Subject Headings and other keywords to identify studies that utilized telemedicine in patients with digestive disease. The PRISMA guidelines were used to identify 20 research articles that had data aligning with 4 common overlapping themes including, patient compliance (n = 13), patient satisfaction (n = 11), disease activity (n = 15), and quality of life (n = 13). The studies focused on digestive diseases including inflammatory bowel disease (n = 7), ulcerative colitis (n = 4), Crohn’s Disease (n = 1), irritable bowel syndrome (n = 6), and colorectal cancer (n = 2). From the studies included in this systematic review, patient compliance and patient satisfaction ranged between 25.7–100% and 74–100%, respectively. Disease activity, measured by symptom severity scales and physiological biomarkers, showed improvements following telemedicine interventions in several, but not all, studies. Similar to disease activity, general and disease-specific quality of life showed improvements following telemedicine interventions in as little as 12 weeks in some studies. Telemedicine and mobile health technology may be effective in managing disease activity and improving quality of life in digestive diseases. Future studies should explore both gastrointestinal and gastroesophageal diseases using these types of interventions.

Adults and children with eosinophilic esophagitis (EoE) have distinct clinical and endoscopic presentations. Recognition of clinical signs, along with laboratory and endoscopic findings, is critical for the identification of patients with EoE because delay in diagnosis has been associated with esophageal remodeling and stricture formation. Clinical presentation varies considerably between adults and children. This is less due to differences in the disease and more due to patient differences. This article describes the similarities and differences in clinical presentation of children and adults with EoE, including areas of epidemiology, clinical and endoscopic presentation, pathophysiology, and treatment.

Background: A high prevalence (9.5–30%) of eosinophilic esophagitis (EoE) in patients with esophageal atresia (EA) has been reported. The application of the EoE criteria to EA patients might be problematic. To date, only studies using a “routine” biopsy approach, even in asymptomatic patients, have been performed. The aim of the study was to establish the prevalence of EoE among symptomatic EA patients (EA/EoE group) without anastomotic stricture (AS) and to compare their characteristics with those of EoE patients from general population (EoE group). Methods: From 2005 to 2018, we reviewed charts of children with EA and EoE. “Selective” biopsy approach only in EA children without AS and/or endoscopic feature of EoE was performed. Characteristics of EA/EoE and EoE groups were compared. Results: Among 370 EA and 118 EoE, 15 EA/EoE patients were detected (4.0% of EA patients). Male predominance and a high prevalence of allergy without differences between EA/EoE and EoE groups was observed. EA/EoE children were significantly younger (p

OBJECTIVES: Differences in the initial management of pediatric eosinophilic esophagitis (EoE) by practice setting have not been well characterized. We aimed to characterize these differences for sites in the Carolinas EoE Collaborative (CEoEC), a multicenter network of academic and community practices. METHODS: We performed a retrospective cohort study of pediatric EoE patients at five CEoEC sites: University of North Carolina (UNC) Hospital, Charlotte Asthma and Allergy Specialists, Greenville Health Systems, Wake Forest Baptist Medical Center, and the Medical University of South Carolina Hospital. Cases of EoE were defined by consensus guidelines. Data were extracted from electronic medical records. We tested for differences among sites and used a multinomial model (polytomous regression) to assess associations between treatment and site, adjusting on patient factors. RESULTS: We identified 464 children with EoE across the CEoEC sites. The median age was highest at Wake Forest (11.4 years), the median eosinophil count was highest at UNC (69 eos/hpf), and UNC had the most male patients (82%). UNC used topical steroids for initial treatment in 86% of cases, compared with

Background & Aims: The aim of this study was to compare response to inactivated influenza vaccine in healthy children and pediatric patients with inflammatory bowel disease (IBD). Methods: A prospective, open-label, controlled clinical trial during influenza seasons of 2002–2004 was performed. Single-dose inactive trivalent influenza vaccine was administered. Immune response to vaccination was measured by pre-immunization and postimmunization hemagglutinin inhibition titers. A postimmunization hemagglutinin inhibition titer of 40 or higher was considered protective against influenza. IBD activity and adverse events were recorded. Results: Eighty subjects were enrolled (29 healthy controls, 51 IBD patients). One patient did not complete the study. Patients were divided into 3 subgroups: infliximab and immunomodulatory (16), immunomodulatory (20), and anti-inflammatory therapy (14). Immunomodulatory therapy included corticosteroids, 6-mercaptopurine, or methotrexate. Overall, there was a statistically significant decrease in immune response in patients compared with healthy controls who received 1 influenza vaccine antigen (B/Hong Kong, P = .0125). Patients receiving infliximab and immunomodulatory therapy were less likely to respond to 2 influenza vaccine antigens (A/New Caledonia/20/99 and B/Hong Kong/330/2001, P = .018 and .0002, respectively). Fifteen subjects (19%) reported 19 mild adverse events: 11 (14%) reported soreness at the site, 4 (5%) reported having a cold, 3 (4%) reported flu-like symptoms, and 1 (1%) reported a headache. The clinical activity of IBD was not affected by vaccination. Conclusions: The serologic conversion rate to influenza vaccine in patients with IBD ranged from 33% to 85%. Patients on concomitant infliximab and immunomodulatory therapy are at risk of inadequate response to vaccination. The vaccine was safe and did not affect IBD activity.

Dysregulation of interleukin-8 (IL-8) production has been proposed to contribute to intestinal inflammation in inflammatory bowel disease (IBD) patients. Previous studies, which evaluate adult patients with long-standing or steroid-modulated disease, have reported conflicting results regarding the role of IL-8 in IBD pathogenesis. The present study evaluates IL-8 in colonic organ cultures and sera of newly and previously diagnosed pediatric IBD patients with various degrees of histopathologic activity. Colon and terminal ileum biopsies were obtained from 26 patients with Crohn’s disease, 12 with ulcerative colitis, 4 with indeterminate colitis, and 12 age-matched normal controls. IBD patients were additionally characterized as newly or previously diagnosed. Supernatants from organ-cultured lamina propria biopsies and sera were evaluated by ELISA for IL-8 protein. IL-8 increased with degree of histologic inflammation regardless of diagnosis (no pathologic diagnosis, 62.6 ng/ml, interquartile range [IQR] 30.4–94.6 ng/ml; mild, 92.0 ng/ml, IQR 21.9–170.0 ng/ml; moderate, 676.2 ng/ml, IQR 46.4–2967.7 ng/ml; severe, 585.6 ng/ml, IQR 149.7–1602.2 ng/ml; P < 0.01). Lamina propria IL-8 was significantly elevated in moderately and severely inflamed tissue segments (603.26 ng/ml; IQR, 72.15–2240.4 ng/ml) compared to noninflamed and mildly inflamed segments (67.70 ng/ml; IQR, 30.38–124.1 ng/ml; P = 0.0009). There was no significant trend in IL-8 concentration when compared by clinical diagnosis. No significant difference was found in IL-8 concentrations in organ cultures from newly diagnosed patients versus those from previously diagnosed patients. There was no significant correlation between serum IL-8 concentration and organ culture IL-8 concentration. We conclude that higher concentrations of IL-8 are found in more histologically inflamed tissue segments from pediatric IBD patients. IL-8 does not appear to be associated with clinical IBD subtype. IL-8 appears to be an integral part of both early and established mucosal inflammation in pediatric IBD patients. These findings suggest that IL-8-specific therapies may universally modify inflammatory activity in IBD patients.

Eosinophilic oesophagitis is an emerging disease, well known also in paediatric age, probably caused by both IgE and non-IgE mediated food allergies, diagnosed by upper endoscopy with biopsy. The most severe complication is oesophageal stenosis. The identification of the offending allergens is often difficult; therapy is focused to eliminate the supposed antigenic stimulus, to control the acute symptoms and to induce long-term remission. Aim We report the clinical outcome and the typical endoscopic findings of children and adolescents affected by eosinophilic oesophagitis, referring a proposal of diagnostic and treatment protocol. Patients and methods Twelve patients, affected by eosinophilic oesophagitis with a histological diagnosis, underwent radiographic upper gastro-intestinal series, 24 h pH-probe and standardised allergic testing; they were treated with steroids (oral prednisone and swallowed aerosolised fluticasone) and elimination diet. Dilations were performed when eosinophilic oesophagitis was not yet diagnosed, or in patients resistant to conventional treatment. Results Two patients were lost to follow up (mean follow up: 1 year 11 months); seven patients have no symptoms and normal histology, five of them on restricted diet (without cow's milk protein) and two patients on elemental diet (amino acid formula). In two patients (no allergens identified), mild dysphagia and eosinophilic infiltration persist; one patients underwent Nissen fundoplication for Barrett's oesophagus: he has no symptoms and normal oesophagus, on restricted diet (without cow's milk/eggs protein and wheat). Conclusion The recognition of typical endoscopic picture with careful biopsies extended to the whole oesophagus, even in emergency, could more quickly lead to the correct diagnosis and avoid severe complications of eosinophilic oesophagitis in children, as stricture and failure to growth. Elimination diet is the key of resolution when the allergens are identified. A great challenge remains the relation between gastro-oesophageal reflux disease and eosinophilic oesophagitis, which should however be explained.

Background & Aims: Eosinophilic esophagitis (EoE) is a disorder characterized by a severe, isolated eosinophilic infiltration of the esophagus unresponsive to aggressive acid blockade but responsive to the removal of dietary antigens. We present information relating to our 10-year experience in children diagnosed with EoE. Methods: We conducted a retrospective study between January 1, 1994, and January 1, 2004, to evaluate all patients diagnosed with EoE. Clinical symptoms, demographic data, endoscopic findings, and the results of various treatment regimens were collected and evaluated. Results: A total of 381 patients (66% male, age 9.1 ± 3.1 years) were diagnosed with EoE: 312 presented with symptoms of gastroesophageal reflux; 69 presented with dysphagia. Endoscopically, 68% of patients had a visually abnormal esophagus; 32% had a normal-appearing esophagus despite a severe histologic esophageal eosinophilia. The average number of esophageal eosinophils (per 400× high power field) proximally and distally were 23.3 ± 10.5 and 38.7 ± 13.3, respectively. Corticosteroids significantly improved clinical symptoms and esophageal histology; however, upon their withdrawal, the symptoms and esophageal eosinophilia recurred. Dietary restriction or complete dietary elimination using an amino acid–based formula significantly improved both the clinical symptoms and esophageal histology in 75 and 172 patients, respectively. Conclusions: Medications such as corticosteroids are effective; however, upon withdrawal, EoE recurs. The removal of dietary antigens significantly improved clinical symptoms and esophageal histology in 98% of patients.

Purpose: To describe the reported health-related quality of life (HRQOL) in children and adolescents with inflammatory bowel disease (IBD) after attending an IBD summer camp. Methods: A prospective analysis of quality of life was completed at an overnight camp that was exclusively for patients with IBD, which was sponsored by the Crohn's and Colitis Foundation of America. The IMPACT-II questionnaire (Canada and United States) and the State-Trait Anxiety Inventory for Children were administered to the campers at the beginning and at the end of a 1-week camp to assess HRQOL and anxiety. The IMPACT-II questionnaire consists of 35 questions measuring 6 quality-of-life domains (i.e., bowel domain, systemic symptoms, emotional functioning, social functioning, body image, and treatment/interventions). The State-Trait Anxiety Inventory for Children consists of 2 different 20-item sets of questions. One set assesses state anxiety, and the other, trait anxiety. A repeated-measures multivariate analysis of variance was performed to determine the differences between scores attained before and after camp on the IMPACT-II questionnaire and in each of its domains. Paired sample t tests were performed on state and trait anxiety before and after camp. Results: A total of 125 individuals consented to participate, but 61 patients (50 girls and 11 boys; age range, 9 to 16 y) completed the IMPACT-II questionnaire in full. Of those 61 patients, 47 had Crohn's disease and 14 had ulcerative colitis. There was statistically significant improvement between the mean (±SD) precamp total score (172.95 ± 36.61) and the mean postcamp total score (178.71 ± 40.97; P = 0.035), bowel symptoms scores (P = 0.036), social functioning scores (P = 0.022), and treatment interventions scores (P = 0.012). No difference was found between anxiety scores before and after camp on either the state or trait anxiety inventories (n = 55; P > 0.05). Conclusions: Overall, HRQOL improved in children after attending IBD summer camp. This exploratory study suggests that contributing factors for these improvements may be an increase in social functioning, a better acceptance of IBD symptoms, and less distress regarding treatment interventions, suggesting that a camp that is specifically designed for children with IBD may normalize the chronic illness experience. However, future research using a multimodal measurement approach is warranted to support these conclusions.

Pediatric endoscopy and colonoscopy are essential for the accurate diagnosis of inflammatory bowel disease (IBD). These procedures, when performed in children and adolescents, do not significantly differ in technical aspects compared with endoscopy in adult patients. However, important nuances unique to the pediatric population, like sedation, preparation for endoscopy, instruments, and technique remain and may warrant special attention. Preparation for endoscopic procedures involves appropriate bowel cleansing, adequate anticipation of the psychological needs, and sedation of the patient. All of these aspects of preparation should be individualized to allow for a successful and safe procedure. Upper endoscopy performed as part of the initial evaluation helps to distinguish between Crohn’s disease and ulcerative colitis early in disease presentation and commonly demonstrates upper tract disease, even in asymptomatic individuals. New endoscopic technologies, once evaluated in clinical trials, may offer improved comfort and safety leading to faster and more complete procedures in the pediatric age group.

Complementary and alternative medicine use is prominent in the United States. The use of complementary and alternative therapies appears to be common in patients with inflammatory bowel disease, but few studies have been completed in children. We sought to examine the extent that children with inflammatory bowel disease in the Greater Philadelphia area (Philadelphia County and the surrounding counties in Delaware, New Jersey, and Pennsylvania) use alternative therapies. We paid particular attention to the specific types of therapies used and whether certain demographic and disease associated factors influence the degree of usage. In this study, we questioned the families of all children diagnosed with inflammatory bowel disease, aged 6 to 16 years and living within Philadelphia and its surrounding counties, who were followed at 1 of the 2 academic pediatric gastroenterology programs that served the area. More than 80% of surveys were returned. Fifty-one percent (95% C.I. 45% to 56%) of patients surveyed reported some form of alternative medicine use within the previous year. Univariate analysis revealed increased use among patients who had Crohn disease, who used the Internet for research on their disease, who reported poor quality of life and had increased school absences in the past year. Therapies associated with alternative medicine use included biological and immunomodulatory therapy. Regression analysis revealed positive associations between use of alternative therapies and expenditure on nonprescription treatments, poor quality of life, Internet research, and the need for calorie supplementation, whereas there was a negative association with history of prior surgery for inflammatory bowel disease.

Background: Interleukin-13 (IL-13) is a multifunctional cytokine whose net principle action is to diminish inflammatory responses. Dysregulation of IL-13 production has been proposed to contribute to intestinal inflammation in inflammatory bowel disease (IBD) patients. Previous studies implicate IL-13 in IBD pathogenesis; however, they fail to accurately reflect in vivo intestinal IL-13 production. We evaluate IL-13, IL-6, and IL-1β elaborations from colonic organ cultures of pediatric IBD patients Methods: Endoscopic lamina propria biopsies or surgical specimens from pediatric patients with IBD were organ cultured and supernatants evaluated by enzyme-linked immunosorbent assay for IL-1β, IL-6, and IL-13. Results: IL-13 concentrations were significantly reduced in ulcerative colitis (UC) patients when compared with normal controls (P = 0.002) and Crohn disease (CD) patients (P = 0.001). End-stage UC patients at colectomy had lower intestinal IL-13 production than all other UC patients (P = 0.002). No significant correlation was found between IL-13 concentration and histologic disease severity (P = 0.134). Conclusions: Diminished intestinal IL-13 production is present in UC patients and wanes further with clinical disease progression. These findings suggest that UC patients may be differentiated from CD patients by intestinal IL-13 quantitation, and UC patients may benefit from IL-13 enhancing therapies.

Objective: The Pediatric Crohn's Disease Activity Index (PCDAI) is a validated measure of disease activity comprised of historical, laboratory and physical examination parameters. It has been suggested that an abbreviated PCDAI may be of similar utility without requiring laboratory evaluations or calculated height velocity. The aim of this study was to compare an abbreviated PCDAI and the original PCDAI and also compare the abbreviated PCDAI and a quality-of-life measurement. Methods: The authors prospectively analyzed quality of life and disease activity, using the IMPACT-35 Questionnaire, the PCDAI and an abbreviated PCDAI consisting of three historical items (abdominal pain, stools and patient functioning) and three physical examination items (weight, abdomen and perirectal disease). Results: Forty subjects aged 5-24 years (22 males) were included in analysis. Correlations were performed between the original PCDAI, an abbreviated PCDAI and the IMPACT-35, There was a significant, strong correlation between the PCDAI and the abbreviated PCDAI (n = 40, r = 0.849, p

We read the article by Molina-Infante et al 1 with great concern as it proposes a major change in the current practice guidelines for managing patients presenting for care by gastroenterologists worldwide. Proton pump inhibitor-responsive oesophageal eosinophilia (PPI-REE)1 is characterised by an eosinophilic infiltrate of the oesophageal epithelium that is responsive to PPI therapy alone. The authors suggest that endoscopies should be performed prior to a therapeutic trial of PPI, so as not to miss this subtype of eosinophilic oesophagitis (EoE). We maintain that these two entities not only have yet to be proven to be the same pathophysiological process, but also that performing an initial endoscopy with biopsy would not change the current practice of first using a PPI before or after the …

Several aspects of IBD overlap between pediatric and adult population. Those include nutritional issues, bone density, and medical and surgical therapies. Some aspects like natural course of the disease, and epidemiology and genetics are more easily examined and researched in the pediatric population. Others like pubertal and growth delay, and transition of health care are unique to pediatric patients. This article examines some of the similarities, as well as differences of IBD in these two populations.

A relationship between autism and gastrointestinal (GI) immune dysregulation has been postulated based on incidence of GI complaints as well as macroscopically observed lymphonodular hyperplasia and microscopically determined enterocolitis in pediatric patients with autism. To evaluate GI immunity, we quantitatively assessed levels of proinflammatory cytokines, interleukin (IL)-6, IL-8, and IL-1beta, produced by intestinal biopsies of children with pervasive developmental disorders.Fifteen patients, six with pervasive developmental disorders and nine age-matched controls, presenting for diagnostic colonoscopy were enrolled. Endoscopic biopsies were organ cultured, supernatants were harvested, and IL-6, IL-8, and IL-1beta levels were quantified by ELISA. Tissue histology was evaluated by blinded pathologists.Concentrations of IL-6 from intestinal organ culture supernatants of patients with pervasive developmental disorders (median 318.5 pg/ml, interquartile range 282.0-393.0 pg/ml) when compared with controls (median 436.9 pg/ml, interquartile range 312.6-602.5 pg/ml) were not significantly different (p = 0.0987). Concentrations of IL-8 (median 84,000 pg/ml, interquartile range 16,000-143,000 pg/ml) when compared with controls (median 177,000 pg/ml, interquartile range 114,000-244,000 pg/ml) were not significantly different (p = 0.0707). Concentrations of IL-1beta (median 0.0 pg/ml, interquartile range 0.0-94.7 pg/ml) when compared with controls (median 0.0 pg/ml, interquartile range 0.0-60.2 pg/ml) were not significantly different (p = 0.8826). Tissue histology was nonpathological for all patients.We have demonstrated no significant difference in production of IL-6, IL-8, and IL-1beta between patients with pervasive developmental disorders and age-matched controls. In general, intestinal levels of IL-6 and IL-8 were lower in patients with pervasive developmental disorders than in age-matched controls. These data fail to support an association between autism and GI inflammation.

Eosinophilic esophagitis (EoE), a disorder characterized by eosinophilic infiltration of the esophageal mucosa, has been defined in large part through published case reports and series leading to ambiguity in both diagnostic and treatment options. Corticosteroids, cromolyn, and elemental diet have all been reported as successful treatments for EoE. In this study, we sought to accurately define a population of patients with EoE and then assess their response to elemental diet.A series of patients with chronic symptoms of gastroesophageal reflux disease and an isolated esophageal eosinophilia on esophagogastroduodenoscopy (EGD) were identified. Therapy with a proton pump inhibitor was instituted for 3 months, followed by repeat EGD when symptoms persisted. A 24-h pH probe study was performed, and those with significantly abnormal studies were excluded. The remaining patients were diagnosed with EoE and placed on an elemental diet for 1 month, followed by a repeat EGD.Of 346 patients with chronic gastroesophageal reflux disease symptoms and eosinophils on esophageal biopsy, 51 (14.7%) were ultimately diagnosed with EoE. There was significant improvement in vomiting, abdominal pain, and dysphagia after the elemental diet. The median number of esophageal eosinophils per high-powered field (HPF) decreased from 33.7 before the diet to 1.0 after the diet (p0.01). The average time to clinical improvement was 8.5 days.Elemental diet resulted in striking improvement in both symptoms and histologic evidence of disease in children and adolescents with EoE, as identified by strict diagnostic criteria.

The published experience using infliximab (Remicade, Centocor, Malvern, PA) for the treatment of pediatric Crohn's disease is limited but suggests utility in the treatment of refractory disease. Experience using infliximab at a large pediatric center is reviewed.A retrospective review of all infliximab infusions administered to patients with Crohn's disease (CD) was undertaken. Data were obtained from database and pharmacy records. Chart review and interviews with physicians, patients, and families were used to obtain missing data.A total of 432 infusions were administered to 82 patients (34 female and 48 male) with CD. The number of infusions each patient received ranged from one to 18, with a mean of 5.3 (SD 4.6) and median of 3. Of 33 patients, 19 (57.6%) became independent and remained free of corticosteroids. There was a statistically significant difference in the steroid dose between 0 and 4 wk and 0 and 8 wk. In all, 23 infusion reactions occurred (5.3%). Three patients developed herpes zoster, and one developed Listeria monocytogenes meningitis. No patients were documented to have delayed hypersensitivity reactions or malignancies.Infliximab is safe and effective for treating pediatric patients with CD. A steroid-sparing effect was demonstrated. The most common adverse reaction to infliximab was infusion reaction. These reactions did not preclude further use of the agent. Serious infections were seen in a small number of patients.

Understanding cytokine production patterns in early mucosal lesions of pediatric patients newly diagnosed with inflammatory bowel disease (IBD) may be critical to understanding IBD pathogenesis. Interleukin-6 (IL-6) has a central role in a multitude of immune system reactions; however, inconsistent lamina propria and serum IL-6 has been reported in IBD patients. Newly diagnosed pediatric IBD patients have not previously been evaluated for lamina propria or serum IL-6.Serum and intestinal lamina propria biopsy whole organ culture supernatants were evaluated by ELISA for IL-6 obtained from newly diagnosed IBD patients, before initiation of immunomodulatory therapies.Levels of lamina propria IL-6 demonstrated significant correlation with graded severity of histological inflammation (p0.001). Log-transformed serum and organ culture IL-6 levels demonstrated significant correlation (p0.0001, R2 = 0.6226). Assigning a demarcation level of400 pg/ml, serum IL-6 concentrations were a superior marker for the presence of microscopic intestinal inflammation than erythrocyte sedimentation rate (ESR), with a sensitivity of 82%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 82%. When evaluating subtypes of IBD, serum IL-6 levels were correlated more significantly with active disease in ulcerative colitis patients (p = 0.01, R2 = 0.74) than in Crohn's disease patients (p = 0.21, R2 = 0.33).This study outlines graded production of IL-6 in intestinal lamina propria and serum of newly diagnosed pediatric IBD patients, confirming the presence of IL-6 in early IBD patients. In addition, serum IL-6 may be a good predictor of IBD in pediatric patients with suspected or newly diagnosed IBD.

Background: Iron-deficiency anemia is a frequent complication in children with inflammatory bowel disease (IBD). Parenteral iron therapy is rarely prescribed because of concern about potential side effects. The aim of this study was to retrospectively evaluate the safety and efficacy of total dose intravenous (TDI) iron therapy. Methods: Charts of all the pediatric patients with IBD who received TDI iron therapy between February of 1994 and February of 2000 were reviewed. Results: Seventy patients (20 with ulcerative colitis and 50 with Crohn disease) received a total of 119 TDI iron dextran infusions. Thirty-four patients qualified for the efficacy analysis. The average increase in hemoglobin concentration was 2.9 g/dL. (P < 0.0001). Eleven immediate hypersensitivity reactions developed in 10 patients (9% of the total number of infusions). None of the reactions was life threatening and none required hospitalization. Conclusions: Total dose intravenous infusion of iron dextran, when appropriately used, is a safe and potentially efficacious treatment for children with inflammatory bowel disease and iron deficiency anemia who are unresponsive to or noncompliant with oral iron therapy.

Eosinophilic gastroenteritis (EG) was first described over 50 years ago. Despite its long history, it remains an ill-defined and poorly understood entity. EG can present in a number of ways, none of which are exclusive to the disorder. EG has features of allergy and immune dysregulation but does not clearly fit into the category of allergic or immune disorder. While EG has been reported to affect all locations and layers of the gastrointestinal tract, the vast majority of reported cases have demonstrated mucosal involvement of the gastric antrum and small intestine in addition to disease activity of other locations of the gastrointestinal tract. Recently, several reports have identified a disease consisting of an isolated esophageal eosinophilia. Eosinophilic esophagitis (EE), also known as primary eosinophilic esophagitis or idiopathic eosinophilic esophagitis, occurs in adults and in children and represents a subset of EG with an isolated severe esophageal eosinophilia. Patients with EE present with symptoms similar to those of gastroesophageal reflux but are unresponsive to antireflux medication. Reports have demonstrated that patients with EE respond to either dietary restriction or corticosteroids.

INTRODUCTIONThe risk of malignancy as a complication of Crohn disease has been well described (1-3). In addition to the established risk of colorectal cancer associated with inflammatory bowel disease, extraintestinal malignancies such as lymphoma have been reported. Areas of intestine with chronic

Background/purpose Minimally invasive approaches have been shown to decrease hospital length of stay (LOS), decrease postoperative pain, and speed return to normal activity for a number of intraabdominal procedures. In this study, laparoscopic (LAP)-assisted bowel resection is compared with an open technique for patients undergoing an initial bowel resection. Methods A retrospective review was conducted of 28 patients (12 LAP, 16 open) undergoing initial bowel resection for segmental Crohn's disease. Results Patients in the LAP group had decrease LOS (5.5 days v 11.5 days) decreased days of parenteral narcotics (3 days v 5 days) and more rapid return to regular diet (3 days v 5 days). Conclusions The data suggest that the laparoscopic approach may offer advantages to pediatric patients undergoing an initial bowel resection for segmental Crohn's disease.

Over the past 20 years, eosinophilic esophagitis (EoE) has become one of the most discussed diseases among pediatric gastroenterologists and allergists in the United States. According to a consensus statement drafted by a multidisciplinary working group, EoE is a clinicopathological disease characterized by symptoms such as gastroesophageal reflux, dysphagia, or feeding intolerance (although not limited to these) in the presence of at least 15 eosinophils seen per microscopic high-powered field in at least one biopsy specimen taken from the esophagus, without another identifiable etiology. Likely owing to a combination of increased awareness among physicians and an increasing incidence among the population, EoE is now one of the most important chronic gastrointestinal diseases to affect children. Yet, despite the increased attention paid to this problem, there remains considerable debate as to the optimal way to diagnose and treat the disorder. This chapter will discuss the various potential approaches to diagnosing and treating EoE with regard to the latest information on the subject.

Eosinophilic esophagitis (EoE), an isolated esophageal eosinophilia associated with clinical symptoms, is a disease that has received a great deal of attention over the last 10–15 years. EoE, previously known as primary eosinophilic esophagitis, idiopathic eosinophilic esophagitis or allergic esophagitis, occurs in both children and adults. Prior to 1995, the literature contained only rare reports of individuals diagnosed with an isolated esophageal eosinophilia. However, since 1995, reports in the literature and information related to EoE has grown tremendously. This chapter focuses on the history of EoE including initial reports of esophageal eosinophilia prior to 1995, the landmark article identifying EoE as a disorder in 1995, the growth of EoE in the literature since 1995, the development of the First International Gastrointestinal Eosinophilic Research Symposium (FIGERS) in 2006, and the creation of The International Gastrointestinal Eosinophilic Researchers (TIGERS).