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.
Pacemaker location in the abdominal wall is considered a contraindication to videocapsule endoscopy (VCE). The aim of this study was to review our experience on the use of VCE in patients with a pacemaker located in the abdominal wall. VCE was carried out with monitoring of cardiac rhythm. This was a retrospective review of VCE case studies performed at two tertiary care university medical centers (pediatric and adult). The main outcome measures were adverse events and quality of VCE images. No adverse events were experienced in any of the five patients with implanted cardiac pacemakers, including the two with abdominal pacemaker. No interference with the VCE recording was observed during the studies, although the capsule was observed to be briefly inactivated by the pacemaker in one case. The present study, though small, suggests that VCE is safe in adult and pediatric patients who are fitted with cardiac pacemakers, even when implanted in the abdominal wall. The VCE exam can be carried out successfully under close supervision. Dysfunction of the capsule appears to be more likely than problems with cardiac pacing.
Chronic granulomatous disease (CGD) is a rare primary immune deficiency caused by mutations in genes coding for components of the nicotinamide adenine dinucleotide phosphate oxidase, characterized by severe and recurrent bacterial and fungal infections, together with inflammatory complications. Dysregulation of inflammatory responses are often present in this disease and may lead to granulomatous lesions, most often affecting the gastrointestinal (GI) and urinary tracts. Treatment of inflammatory complications usually includes corticosteroids, whereas antimicrobial prophylaxis is used for infection prevention. Curative treatment of both infectious susceptibility and inflammatory disease can be achieved by hematopoietic stem cell transplantation. We report herein three patients with the same mutation of the CYBB gene who presented with very early-onset and severe GI manifestations of X-linked CGD. The most severely affected patient had evidence of antenatal inflammatory involvement of the GI and urinary tracts. Extreme hyperleukocytosis with eosinophilia and high inflammatory markers were observed in all three patients. A Mycobacterium avium lung infection and an unidentified fungal lung infection occurred in two patients both during their first year of life, which is indicative of the severity of the disease. All three patients underwent bone marrow transplantation and recovered fully from their initial symptoms. To our knowledge, these are the first reports of patients with such an early-onset and severe inflammatory manifestations of CGD.
Wireless capsule endoscopy represents an extraordinary technical innovation in diagnostic gastrointestinal endoscopy. As in adult patients, it opens new horizons that permit an accurate and noninvasive approach to identifying occult lesions in the small bowel in children and adolescents. A limitation in the pediatric age group is the size of the capsule, precluding its use in infants and small toddlers. In children unable to swallow the capsule, "front loading" the gastroscope to introduce it into the duodenum is a suitable alternative approach. Capsule endoscopy is highly useful to evaluate for inflammatory changes in patients suspected to have small bowel Crohn's disease in whom conventional imaging failed to confirm the diagnosis. It is now the method of first choice to assess for small bowel polyps or tumors, to find a source of blood loss in obscure intestinal bleeding, and for undiagnosed malabsorptive conditions such as intestinal lymphangiectasia. Capsule retention is the one major potential adverse effect of capsule endoscopy. In patients suspected to have a small bowel stenosis, consideration should be given to using the patency capsule prior to using the real videocapsule so as to decrease the risk of capsule retention.
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