Comparison of laparoscopic and open ileocecal resection for Crohn's disease in children.

PURPOSE: Ileocecal resection (ICR) is the most frequently performed surgery in paediatric Crohn's disease (CD) patients. The aim of the study was to compare laparoscopic-assisted and open ICR. METHODS: Retrospective review of consecutive CD patients undergoing ICR between March 2014 and December 2021 was performed. The patients were divided into open (OG) and laparoscopic (LG) groups. Compared parameters included patients' demographics, clinical characteristics, surgery, duration of hospitalisation and follow-up. Complications were classified according to the Clavien-Dindo classification (CDc). Risk factors were identified using multivariable analysis. RESULTS: Sixty-two patients (29 females, 46.7%) were included in the analysis, forty-two patients in OG. The median duration of surgery was 130 in OG versus 148 in LG (p = 0.065) minutes. Postoperative complications were reported in 4 patients (12.1%). There was no significant difference in postoperative complications according to CDc (OG 7.14 vs LG 5%, p = 1). The median length of hospitalisation was 8 in OG and 7 days in LG (p = 0.0005). The median length of follow-up was 21.5 months. CONCLUSION: The laparoscopic-assisted approach had shorter hospital stay and was not associated with increased risk of 30-day postoperative complications. Laparoscopic surgery should be considered the preferred surgical approach for primary ICR.

Substantial proportion of MODY among multiplex families participating in a Type 1 diabetes prediction programme.

AIMS: Patients with maturity-onset diabetes of the young (MODY) might be over-represented in families with histories of Type 1 diabetes. Our aim was to re-evaluate families participating in the Czech T1D Prediction Programme (PREDIA.CZ) with at least two members affected with diabetes to assess the proportion of MODY among these families and determine its most significant clinical predictors. METHODS: Of the 557 families followed up by the PREDIA.CZ, 53 (9.5%) had two or more family members with diabetes. One proband with diabetes from these families was chosen for direct sequencing of the GCK, HNF1A, HNF4A and INS genes. Non-parametric tests and a linear logistic regression model were used to evaluate differences between MODY and non-MODY families. RESULTS: MODY was genetically diagnosed in 24 of the 53 families with multiple occurrences of diabetes (45%). Mutations were detected most frequently in GCK (58%), followed by HNF1A (38%) and INS (4%). MODY families were more likely to have a parent with diabetes and had a higher proportion of females with diabetes than non-MODY families. Higher age (P < 0.001), a lower level of HbA1c (P < 0.001) at clinical onset and at least two generations affected by diabetes were the variables most predictive for probands of MODY families already presenting with diabetes. CONCLUSIONS: A prediction programme for Type 1 diabetes would provide a useful new source of patients with MODY most likely to benefit from an accurate diagnosis. This identification has implications for patient treatment and disease prognosis.

Pilot study: Association between Helicobacter pylori in adenoid hyperplasia and reflux episodes detected by multiple intraluminal impedance in children.

OBJECTIVES: The aim of this pilot study was to investigate an association between laryngopharyngeal reflux detected by combined multiple intraluminal impedance and pH monitoring and Helicobacter pylori in adenoid hyperplasia detected with real time polymerase chain reaction (PCR). METHODS: The study group consisted of 30 children (median age 5.34 years) with extraesophageal symptoms of gastroesophageal reflux disease with adenoid hyperplasia. All children underwent adenoidectomy with subsequent PCR detection of H. pylori DNA in the tissue and multiple intraluminal impedance and pH monitoring. The most proximal impedance sensor was located 1cm caudal to the entrance of the oesophagus. RESULTS: We found significant differences in the number of reflux episodes among patients with PCR positivity (median 35) and negativity (median 0) of H. pylori (p-value of Mann-Whitney U-test 0.0056). Patients with PCR positivity of H. pylori had significantly more reflux episodes reaching the upper oesophageal sphincter (p-value of Mann-Whitney U-test 0.023). The absence of reflux episode was the only independent factor for PCR negativity of H. pylori in the multiple logistic regression model. CONCLUSIONS: These results support the hypothesis that reflux episodes reaching the upper oesophageal sphincter may play an important role in the transmission of H. pylori into lymphoid tissue of the nasopharynx and thus may contribute to adenoid hyperplasia in children.

Infliximab dependency in children with Crohn's disease.

BACKGROUND: Recently, infliximab dependency has been described. AIM: To assess frequency of ID in 82 consecutive Crohn's disease children treated with infliximab 2000-2006 and to describe clinical and genetic predictors of long-term infliximab response. METHODS: A phenotype model of infliximab dependency was used to assess treatment response: 'immediate outcome' (30 days after infliximab start)--complete/partial/no response. 'Long-term outcome': (i) prolonged response: maintenance of complete/partial response; (ii) infliximab dependency: relapse < or = 90 days after intended infliximab cessation requiring repeated infusions to regain complete/partial response or need of infliximab >12 months to sustain response. Polymorphisms TNF-308 A>G, TNF-857 C>T, Casp9 93 C>T, FasL-844 C>T, LTA 252 C>T and CARD15 (R702W, G908R, 1007fs) were analysed. RESULTS: Ninety-four per cent of children obtained complete/partial response. In long-term outcome, 22% maintained prolonged response, 12% had no response, while 66% became infliximab dependent. Perianal disease and no previous surgery were associated with infliximab dependency (OR 5.34, 95% CI: 1.24-22.55; OR 6.7, 95% CI: 1.67-26.61). No association was found with studied polymorphisms. The cumulative probability of surgery 50 months after starting infliximab was 10% in infliximab dependency, 30% in prolonged responders and 70% in nonresponders (P = 0.0002). CONCLUSIONS: Sixty-six per cent of children became infliximab dependent. Perianal disease and no surgery prior to infliximab were associated with infliximab dependency phenotype.

Variants of CARD15, TNFA and PTPN22 and susceptibility to Crohn's disease in the Czech population: high frequency of the CARD15 1007fs.

Crohn's disease (CD) has been shown to be associated with the variants in the CARD15 gene as well as in other genes involved in the immune response. The frequencies of the variants profoundly differ among populations and so does the associated risk. We examined the associations of variants in the CARD15, TNFA and PTPN22 genes with pediatric-onset and adult-onset CD in the Czech population. Genotype, phenotype and allelic frequencies were compared between 345 patients with CD (136 pediatric-onset and 209 adult-onset patients) and 501 unrelated healthy controls. At least one minor allele of the CARD15 gene was carried by 46% patients and only 21% control subjects (OR = 3.2, 95% CI 2.4-4.4). In a multiple logistic regression model, the strongest association with CD was found for the 1007fs variant (OR = 4.6, 95% CI 3.0-7.0), followed by p.G908R (OR = 2.9, 95% CI 1.5-5.7) and p.R702W (OR = 1.7, 95% CI 1.0-2.9), while no independent association was found for the remaining variants in the CARD15 gene (p.268S, p.955I and p.289S), for the p.R620W variant in the PTPN22 gene or for the g.-308G>A variant in the TNFA gene. The age at CD onset was strongly modified by positivity for the 1007fs allele: it was present in 42% pediatric-onset and only 25% adult-onset patients. In conclusion, we report a high frequency of the minor allele of the CARD15 1007fs polymorphism in the Czech population and a strong effect of this allele on the age at disease onset.