Evaluation of guidelines for management of familial adenomatous polyposis in a multicenter pediatric cohort.

Objective: To retrospectively assess, in a pediatric multicenter cohort, guidelines for the management of familial adenomatous polyposis (FAP).

Methods: Ten centers from the French-speaking Pediatric Gastroenterology Hepatology and Nutrition Group provided follow-up data on patients up to 18 years of age. Clinical records, genetic test results, endoscopy with histopathology examination, and therapeutic modalities were reviewed.

12-month follow-up after successful infliximab therapy in pediatric crohn disease.

Aim: Infliximab (IFX) therapy is highly efficacious for the induction and maintenance of remission in pediatric Crohn disease (CD). However, to date it is unclear how long patients should be given IFX. Given the increasing safety concerns about the concomitant and prolonged use of IFX and azathioprine in CD, we wanted to address the clinical outcome in pediatric CD patients who responded to IFX medication, once IFX was stopped.

Characteristics of inflammatory bowel disease with onset during the first year of life.

Background: Inflammatory bowel disease (IBD) is recognized in young children, however, only rare data on onset and evolution are available in children younger than 1 year. In the present clinical study, we aimed to analyze characteristics and clinical course of children with very early onset IBD. We were particularly interested in the relationship between bacterial infections and the use of antibiotics before the onset of IBD.

Contiguous gene deletion within chromosome arm 10q is associated with juvenile polyposis of infancy, reflecting cooperation between the BMPR1A and PTEN tumor-suppressor genes.

We describe four unrelated children who were referred to two tertiary referral medical genetics units between 1991 and 2005 and who are affected with juvenile polyposis of infancy. We show that these children are heterozygous for a germline deletion encompassing two contiguous genes, PTEN and BMPR1A. We hypothesize that juvenile polyposis of infancy is caused by the deletion of these two genes and that the severity of the disease reflects cooperation between these two tumor-suppressor genes.

Ileal involvement is age dependent in pediatric Crohn's disease.

Background: Lymphoid follicles (LFs) have been suggested to play a role at the early stage of Crohn's disease (CD) lesions. In the small bowel, LFs are grouped, forming Peyer's patches, which develop early in fetal life, grow in size and number until puberty, and undergo involution. In contrast, colonic LFs are isolated and undergo little change during life.

Diagnosing celiac disease: a comparison of human tissue transglutaminase antibodies with antigliadin and antiendomysium antibodies.

Objective: To evaluate and compare the sensitivity and specificity of the new serologic marker human antitissue transglutaminase antibodies (IgA anti-tTG) with those of antiendomysium (IgA EMA) and antigliadin antibodies (IgA and IgG AGA) for the diagnosis of celiac disease (CD).

Methods: The level of IgA antibodies to tTG in serum was determined by an enzyme-linked immunosorbent assay (ELISA) test using recombinant human tTG as the antigen; IgA EMA, by indirect immunofluorescence; and IgA and IgG AGA, by ELISA. Sixty-eight serum samples from 59 patients with CD were studied-30 patients had untreated CD, 22 were on gluten-free diets, and 16 had been reintroduced to gluten-and compared with serum samples from 116 children examined for failure to thrive, short stature, various digestive diseases, or other non-CD conditions.

A prospective study of the efficacy and tolerance of a chimeric antibody to tumor necrosis factors (remicade) in severe pediatric crohn disease.

Objectives: To evaluate the efficacy and toxicity of infliximab in children with severe Crohn disease (CD), the authors prospectively monitored 21 children aged 15 +/- 2 years with severe CD who they treated with infliximab (5 mg/kg) on days 0, 15, and 45. One patient received only one injection. Eighteen patients were corticosteroid dependent, and 6 were receiving parenteral nutrition.