Libia Moy

Wireless capsule endoscopy in the pediatric age group : Experience and complications

Background: The development of wireless capsule endoscopy (CE) provides a unique opportunity to visualize the entire small bowel in a minimally invasive manner. Studies in adult patients have demonstrated that the disposable capsule is well tolerated and highly effective, but few studies have been done in children. The aims of our study were to compare the diagnostic yield of CE and small bowel series in children being evaluated for possible small intestine disease and to determine the risk of developing an adverse event following capsule endoscopy. Patients and Methods: We retrospectively reviewed the records of all children who underwent CE at 1 institution between August 2002 and July 2005. Results of CE were compared with those of small bowel radiographic studies when available. Results: There were 46 CE studies from 45 patients, 28 male and 17 female, with a mean age of 14.9 ± 3.6 years and mean weight of 49.7 ± 17.5 kg. The indications for CE included unresponsive Crohn disease (n = 16), possible intestinal polyps (n = 11), unexplained iron deficiency anemia (n = 7), growth failure (n = 5), unresponsive ulcerative colitis (n = 3), persistent abdominal pain (n = 1), protein-losing enteropathy (n = 1), and allergic enteropathy with occult gastrointestinal bleeding (n = 1). Of the 46 CE studies, 41 were completed and 5 were incomplete studies. Based on the CE, 9 patients were newly diagnosed with Crohn disease, 9 patients with Crohn disease were newly diagnosed with small bowel involvement, 8 patients had upper intestinal polyps, 1 patient had findings consistent with Ménétrièr disease, and 1 had a duodenal ulcer. Thirty-three patients had small bowel series before CE: 24 studies were normal, 6 had abnormal thickening of the small bowel, 2 had polyps, and 1 patient had antral narrowing. All 9 patients with abnormal small bowel series had abnormal CE studies. Of the 24 patients with normal small bowel series, 20 had completed CE studies, and in 10 children, the study was abnormal. Nine of the 45 subjects had adverse events. Five patients had delayed passage from the stomach, with 2 needing endoscopic retrieval of the CE, and 4 had delayed passage from the small intestine (>5 days), with 2 requiring surgical removal, 1 responding to steroids, and the final patient requiring an ileocolic resection 2 months after the CE for an undiagnosed ileal stricture. The only significant association noted was that older patients were more likely to have intestinal retention. Conclusions: CE provides a valuable tool in the evaluation of pediatric patients for possible small bowel disease. However, the risk of developing complications appears to be greater in the pediatric population, with 20% of our patients having an adverse event.

Capsule endoscopy as a diagnostic tool in the evaluation of graft-vs.-host disease.

Abstract:  Capsule endoscopy is a relatively new technology that has allowed gastroenterologists to visualize the mucosa of the small intestine. This technology is playing an expanding role in both adult and pediatric gastroenterology. In this report, we present an 8‐yr‐old child following allogeneic hematopoietic cell transplantation who developed large volume bloody diarrhea requiring multiple packed red blood cell transfusions that was resistant to aggressive therapy for GVHD. The capsule endoscopy performed on this patient provided significant information not provided by upper endoscopy and colonoscopy that allowed for successful treatment changes. This case demonstrates that capsule endoscopy is a diagnostic tool that may play an important role in the assessment of patients, including children, with possible GVHD.

Capsule endoscopy in the evaluation of patients with unexplained growth failure.

Background: Poor weight gain and growth can be caused by many medical, nutritional, behavioral, and psychological factors. Crohn disease is one of the more common gastrointestinal etiologies associated with growth failure. The aim of this study is to determine the role of capsule endoscopy (CE) in the evaluation of older children and adolescents who were referred to a pediatric gastroenterology service for a chief complaint of unexplained growth failure. Patients and Methods: We retrospectively reviewed the records of children with growth failure undergoing CE between August 2002 and November 2005. Height and weight (expressed as z scores) were recorded at least 6 months before study, at the time of the study, and at least 6 months poststudy. All of the patients had celiac disease and Crohn disease excluded using standard biochemical, radiologic, endoscopic, and histologic assessment. Results: Seven children (4 males and 3 females) were included in the study—mean age 11.7 ± 3.6 years. Indications for CE were growth failure associated with abdominal pain (3 patients), diarrhea and apthous ulcers (2 patients), delayed puberty (1 patient), or a family history of Crohn disease (1 patient). The mean z score for weight at the time of the study was −2.10 ± 1.0 and for height was −1.50 ± 0.7 All 7 children had normal small bowel series performed before the CE. All had both endoscopically and histologically normal esophagogastroduodenoscopy and colonoscopy. In 4 of 7 patients, multiple small bowel apthous ulcerations consistent with Crohn disease were identified by CE. All 4 patients who had abnormal CE were treated and started gaining weight. The mean z score for weight after 6 months of treatment was −1.35 ± 1.2 and for height was −0.50 ± 1.7. The mean z score for weight after treatment was significantly improved compared with the mean z score at diagnosis (P < 0.05). Conclusions: In our study, 4 of the 7 older children and adolescents with unexplained growth failure and normal small bowel series were found to have Crohn disease involving the small intestine. In addition, we were able to show the improvement on the mean z score for weight after treatment of small bowel Crohn disease was instituted.

Autoimmune hepatitis: a classic autoimmune liver disease.

AIH is characterized by chronic inflammation of the liver, interface hepatitis, hypergammaglobulinemia, and production of autoantibodies. Based on the nature of the serum autoantibodies, two types of AIH are recognized: type 1 (AIH-1), positive for ANA and/or anti-smooth muscle antibody, and type 2 (AIH-2), defined by the positivity for anti-liver kidney microsomal type 1 antibody or for anti-liver cytosol type 1 antibody. AIH demonstrates a female preponderance with the female-to-male ratio of 4:1 in AIH-1 and 10:1 in AIH-2. Several genes confer susceptibility to AIH and influence clinical manifestation, response to treatment, and overall prognosis. Most are located within the human leukocyte antigen (HLA) region, which is involved in the presentation of antigenic peptides to T cells and thus in the initiation of adaptive immune responses. The strongest associations are found within the HLA-DRB1 locus. In patients with increased genetic susceptibility to AIH, immune responses to liver autoantigens could be triggered by molecular mimicry. Because of molecular mimicry, different environmental agents, drugs, and viruses might produce AIH. In AIH, T cells are numerically and functionally impaired, permitting the perpetuation of effector immune responses with ensuing persistent liver destruction. AIH is rare but highly treatable inflammatory condition of the liver. Subclinical and asymptomatic disease is common. AIH therefore needs to be considered in the differential diagnosis of all patients with elevated liver enzymes. Clinical response to immunosuppressive therapy is characteristic and supports the diagnosis.

Respiratory care in familial dysautonomia: Systematic review and expert consensus recommendations

BACKGROUND Familial dysautonomia (Riley-Day syndrome, hereditary sensory autonomic neuropathy type-III) is a rare genetic disease caused by impaired development of sensory and afferent autonomic nerves. As a consequence, patients develop neurogenic dysphagia with frequent aspiration, chronic lung disease, and chemoreflex failure leading to severe sleep disordered breathing. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of respiratory disorders in familial dysautonomia. METHODS We performed a systematic review to summarize the evidence related to our questions. When evidence was not sufficient, we used data from the New York University Familial Dysautonomia Patient Registry, a database containing ongoing prospective comprehensive clinical data from 670 cases. The evidence was summarized and discussed by a multidisciplinary panel of experts. Evidence-based and expert recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system. RESULTS Recommendations were formulated for or against specific diagnostic tests and clinical interventions. Diagnostic tests reviewed included radiological evaluation, dysphagia evaluation, gastroesophageal evaluation, bronchoscopy and bronchoalveolar lavage, pulmonary function tests, laryngoscopy and polysomnography. Clinical interventions and therapies reviewed included prevention and management of aspiration, airway mucus clearance and chest physical therapy, viral respiratory infections, precautions during high altitude or air-flight travel, non-invasive ventilation during sleep, antibiotic therapy, steroid therapy, oxygen therapy, gastrostomy tube placement, Nissen fundoplication surgery, scoliosis surgery, tracheostomy and lung lobectomy. CONCLUSIONS Expert recommendations for the diagnosis and management of respiratory disease in patients with familial dysautonomia are provided. Frequent reassessment and updating will be needed.

Aerodigestive dysbiosis in children with chronic cough

In pediatric patients with chronic cough, respiratory culture techniques commonly yield negative results. Studies using culture‐independent methods have found a high relative abundance of oral microbes in the lower airways, suggesting that the topographical continuity, and dynamics of the intraluminal contents of the aerodigestive system likely influence the lower airway microbiota. We hypothesize that in subjects with chronic cough, clinical diagnosis will correlate with distinct microbial signatures detected using culture‐independent methods.

W1194 Clinical Outcome of Children with IBD and Preferential MMP Metabolism: Allopurinol vs. Alternative Therapy

Azathioprine (AZA) and 6-mercaptopurine (6MP) are well established treatments for adults and children with inflammatory bowel disease (IBD). However, up to 40% of patients do not respond, at times because of an individuals preferential metabolism of the thiopurine to an inactive and potentially hepatotoxic metabolite, 6-methylmercaptopurine (6MMP), rather than the active 6-thioguanine nucleotides (6TGN) (Dubinsky et al., 2002). The use of allopurinol in AZA/6MP treated adults with preferential 6MMP production has improved levels of 6TGN, reduced 6MMP and been associated with clinical improvement (Sparrow et al., 2005, 2007). Parents of children with IBD and preferential 6MMP metabolism have been offered the opportunity to initiate similar therapy for their children when clinical circumstances warranted a change in therapy. We describe the clinical outcomes of children receiving allopurinol with low dose AZA/6MP versus alternative therapies. Methods: Retrospective chart review identified children who were poor responders to AZA/6MP and had preferential production of 6MMP (defined as 6MMP/6TGN ratio >11). Poor AZA/6MP response was defined as any of the following: 1) persistent IBD-related symptoms, 2) elevated transaminases 3) steroid dependence. Subjects were grouped according to therapeutic intervention (Grp1: allopurinol and reduced dose AZA/6MP; Grp2: other therapies). Clinical goals for each subject were defined at initiation of therapy and response defined as achieving the goals by 3-6 months after beginning treatment. Thiopurine metabolites pre and post therapy were also determined. Results: 27 children had 6MMP/6TGN >11 (mean age 12.4 yrs, 63% male, 20 Crohn, 7 UC). 14 received allopurinol (Grp1), and 13 (Grp2) alternative therapies (infliximab, methotrexate, surgery, adjusted 6MP/AZA dose, 5ASA). Clinical goals were achieved in 12/14 Grp1 subjects and only 4/13 Grp2 subjects (p<0.01). All 4 steroid dependent Grp1 subjects successfully discontinued steroids by 6 months, compared to 0/ 2 in Grp2. Similarly, transaminases normalized in 6/7 Grp1 vs 3/7 Grp2. Lab findings pre and post treatment are summarized in the Table.Conclusion: The combination of allopurinol and low dose AZA/6MP effectively achieves clinical goals in children with IBD and preferential 6MMP metabolism. Children on alternative therapy have a less favorable pattern of improvement. Thiopurine Metabolite Levels Preand PostTreatment