Children can be accurately diagnosed with celiac disease without biopsy analysis. Diagnosis based on level of TGA-IgA 10-fold or more the ULN, a positive result from the EMA tests in a second blood sample, and the presence of at least 1 symptom could avoid risks and costs of endoscopy for more than half the children with celiac disease worldwide. HLA analysis is not required for accurate diagnosis. Clinical Trial Registration no: DRKS00003555.

SUMMARY Background

SBS was the major indication for HPN in our cohort. IFALD and CRBSIs were potentially life-threatening problems. Nevertheless, complication rates were low, and deaths resulted mostly from the underlying disease.

AZA is efficacious in maintaining remission in pediatric CD patients, but to a lesser extent than previously suggested. The majority of patients who are in steroid-free remission at 12 months remained in prolonged remission. Overall tolerance of AZA was excellent.

We report the largest number of paediatric-onset IBD patients with cancer and/or fatal outcomes to date. Malignancies followed by infections were the major causes of mortality. We identified PSC as a significant risk factor for cancer-associated mortality. Disease-related adenocarcinomas were a commoner cause of death than lymphomas.

Marked advances were made over the last decade in deciphering the molecular mechanisms on how external, nutritional factors can impact on the regulation of genes and ultimately their function without modification of the genetic code. This field of nutrigenomic research is literally exploding. With the understanding of epigenetic control mechanisms, such as DNA methylation, histone acetylation, methylation or phosphorylation, as well as the posttranscriptional regulation of gene expression via non-coding microRNA, many different experimental and analytic approaches were possible to elucidate how varying nutritional support might impact on specific functions, with short- and potently long-term effects. This review highlights the major principles of epigenetic control mechanisms and their link to particular nutritional influences. Epidemiological data, such as the Dutch famine studies, suggest that targeted nutritional intervention might be causative for long-term effects on health, such as the increased risk of cardiovascular diseases and metabolic syndrome in this cohort. However, to date most of the knowledge comes from experimental and animal data, which cannot be easily transferred to human situations. It is anticipated that within the next few years, major advances will be made to translate this knowledge of nutritional intervention on gene regulation and expression into health preventive programs.

Inflammatory bowel disorders (IBD) are characterized by chronic and recurrent inflammatory reactions of the intestinal mucosa resulting in progressing ulcerating lesions. Research over the past decade clearly identified in patients with Crohn's disease (CD) a marked dysregulation of the intestinal microbiome (dysbiosis) as one trigger factor in these inflammatory processes, particularly in patients with a high genetic risk. When treating patients with CD, most drugs aim to control the inflammatory process (either by inhibiting inflammatory pathways or by reducing the activity of immune cells). Given the importance of the disturbed interaction between the microbiota and the host immune system, there might be a different therapeutic approach in targeting directly the intestinal microflora. There are good data to believe that the use of exclusive enteral nutrition (EEN) is one such option. Historically, enteral nutrition (EN) was used as supplemental nutritional therapy in CD patients with planned resection surgery. This treatment option showed unexpected and very powerful anti-inflammatory effects, and it was rapidly introduced as induction therapy for active CD. Several clinical trials and case series confirmed the efficacy of EN to induce remission in approximately 80% of patients equaling the potential of steroids. It is well established that EN has this strong anti-inflammatory potential only when given on an exclusive basis, without any additional food. This raises major compliance issues, probably one of the reasons why it is less used in adult patients. A recent study demonstrated that EEN has a specific effect on the intestinal microbiota, which is markedly different from steroid-induced remission, while all patients obtained complete clinical remission. These observations give a first basis for the understanding of the impact of EEN on dysbiosis in patients with CD.

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