Paediatric Endoscopy Global Rating Scale: Development of a Quality Improvement Tool and Results of a National Pilot.

Introduction And Objectives: The endoscopy Global Rating Scale (GRS) is a web-based self-assessment quality improvement (QI) tool that provides a framework for service improvement. Widespread use of the GRS in adult endoscopy services in the United Kingdom (UK) has led to a demonstrable improvement in quality. The adult GRS is not directly applicable to paediatric endoscopy services.

Thymus transplantation for complete DiGeorge syndrome: European experience.

Background: Thymus transplantation is a promising strategy for the treatment of athymic complete DiGeorge syndrome (cDGS).

Methods: Twelve patients with cDGS underwent transplantation with allogeneic cultured thymus.

Objective: We sought to confirm and extend the results previously obtained in a single center.

Joint BSPGHAN and Coeliac UK guidelines for the diagnosis and management of coeliac disease in children.

The revised BSPGHAN guidelines for the diagnosis and management of coeliac disease represent an important shift in diagnostic strategy, aimed at simplifying and shortening the diagnostic process in selected cases. Guidance is given concerning the indications for testing for coeliac disease, which is still significantly underdiagnosed in the UK. While screening data suggest a likely incidence of 1 in 100 persons, only 10%-20% of this figure is currently being diagnosed.

Resolution of parenteral nutrition-associated jaundice on changing from a soybean oil emulsion to a complex mixed-lipid emulsion.

Background And Objective: Resolution of parenteral nutrition (PN)-associated jaundice has been reported in children given a reduced dose of intravenous fat using a fish oil-derived lipid emulsion. The aim of the present study was to examine the effect on PN-associated jaundice of changing from a soybean oil-derived lipid to a mixed lipid emulsion derived from soybean, coconut, olive, and fish oils without reducing the total amount of lipid given.

Methods: Retrospective cohort comparison examining serum bilirubin during 6 months in children with PN-associated jaundice who changed to SMOFlipid (n=8) or remained on Intralipid (n=9).

Sonographic evaluation of inflammatory bowel disease: a prospective, blinded, comparative study.

Background: Children with inflammatory bowel disease (IBD) undergo invasive and repeated investigations, including contrast radiology and endoscopy.

Objective: To assess transabdominal sonography of the colon and distal ileum compared to colonoscopy and barium radiology in known or suspected IBD.

Materials And Methods: A prospectively recruited cohort of 44 children (median age 12 years, range 3.

Microbial-gut interactions in health and disease. Immune responses.

The indigenous bacterial microflora colonize the gut at birth and remain there throughout life. Approximately 10(14) bacteria are present in the ileum and colon and they are clearly immunogenic. The evidence is strong that the vast majority of IgA plasma cells in normal human gut are responding to the antigens of the flora, and although the flora is also responsible for producing the large numbers of T cells which are present in the gut of healthy individuals, the types of T cell response which the flora elicits are less well understood.

Crohn's disease post-cardiac transplantation presenting with severe growth failure and delayed onset of puberty.

We report a 15 yr-old girl who 10 yr post-cardiac transplantation presented with severe growth failure and delayed onset of puberty. She was found to have pan-enteric Crohn's disease and has done remarkably well on principally nutritional therapy with a significant growth spurt and the onset of menarche. The development of bowel disease whilst on immunosuppression is rare and the literature is reviewed.

A failure of transforming growth factor-beta1 negative regulation maintains sustained NF-kappaB activation in gut inflammation.

Immunologically mediated tissue damage in the gut is associated with increased production of proinflammatory cytokines, which activate the transcription factor NF-kappaB in a variety of different cell types. The mechanisms/factors that negatively regulate NF-kappaB in the human gut and the pathways leading to the sustained NF-kappaB activation in gut inflammation remain to be identified. Pretreatment of normal human intestinal lamina propria mononuclear cells (LPMC) with transforming growth factor-beta1 (TGF-beta1) resulted in a marked suppression of TNF-alpha-induced NF-kappaB p65 accumulation in the nucleus, NF-kappaB binding DNA activity, and NF-kappaB-dependent gene activation.

Therapy of Crohn's disease in childhood.

Crohn's disease in childhood is a chronic relapsing and remitting condition that can significantly impact on normal growth and development. This influences the choice of both initial and ongoing management. The goal of therapy is to induce and maintain remission with minimal side effects.