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Objectives: Inflammatory bowel disease (IBD) is today a global disease, the incidence of which is growing in the pediatric population. This prospective study aims to decipher IBD incidence and its trend in a pediatric population through 16 years in the South Moravian Region of the Czech Republic. Methods: We evaluated data concerning 358 pediatric patients with newly diagnosed IBD at University Hospital Brno, which is a gastroenterology center for the entire pediatric population (0–18 years) and cares for all pediatric IBD patients in the South Moravian Region (1,187,667 inhabitants). Results: The study encompassed 3,488,907 children during 16 years. We diagnosed 192 children (53.6%) with Crohn disease (CD), 123 (34.4%) with ulcerative colitis (UC), and 43 (12.0%) with IBD-unclassified (IBD-U). The incidence of IBD increased from 3.8 (CD 2.9, UC 0.9, and IBD-U 0.0) per 100 000/year in 2002 to 14.7 (CD 9.8, UC 4.0, and IBD-U 0.9) per 100,000/year in 2017 (P

Spindle cell hemangioma is a benign vascular tumor typically occurring in the dermis or subcutis of distal extremities as red–brown lesions that can grow in both size and number over time. They can be very painful and potentially disabling. A family history of cancer or previous history may be relevant and must be taken into consideration. Juxtaglomerular cell tumor (reninoma) is an extremely rare cause of secondary hypertension diagnosed mostly among adolescents and young adults. Excessive renin secretion results in secondary hyperaldosteronism. Subsequent hypokalemia and metabolic alkalosis, together with high blood pressure, are clues for clinical diagnosis. Histological examination of the excised tumor leads to a definitive diagnosis. Reninoma is found in subcapsular localization, in most cases as a solitary mass, in imaging studies of kidneys. Exceptionally, it can be located in another part of a kidney. Both spindle cell hemangioma and reninoma are extremely rare tumors in children and adolescents. Herein, the authors present a case report of a patient with hereditary BRCA1 interacting protein C-terminal helicase 1 (BRIP1) mutation, spindle cell hemangioma, and secondary hypertension caused by atypically localized reninoma.

Inflammatory bowel disease is considered a global disease in the 21st century. Studies worldwide have shown an increase in incidence in paediatric population. The aim of the study was to detect the rising incidence of IBD in paediatric population (approximately 240 000) over a 15-year period in the South Moravian Region of the Czech Republic. Another objective was to characterize differences by sex, age and to determine the future projection of incidence of IBD in the region and, by extension, the whole Czech Republic. Methods In the Czech Republic, the treatment of paediatric IBD is centralized into hospitals that provide tertiary care. University Hospital of Brno is one of the gastroenterology centres that provides a complex treatment of IBD patients. We evaluated 321 consecutive paediatric IBD patients < 19 years of age in the South Moravian region (one of the 14 regional units in the Czech Republic). They all had been diagnosed according to the relevant guidelines. Results We evaluated 321 patiens with IBD: 167 of them had Crohn’s disease (52.0%), 114 had ulcerative colitis (35.5%) and 40 were IBD-unclassified (12.5%). Among all the cases of IBD, 53.8% were males. The average age of a diagnosed person was 13.37 years (95%CI: 12.97-13.76) and the patients went an average of 7.59 months (95%CI: 6.15-9.03) between the first symptoms and the diagnosis. The overall incidence of IBD significantly increased from 2.84 per 100 000 person-years in 2002 to 12.54 per 100 000 person-years in 2016 (P = 0.012). Conclusions Our results show that the overal incidence of paediatric IBD is increasing. These data highlight the need for a further research to identify the possible risk factors of IBD development This work was supported by the Ministry of Health of the Czech Republic, grant nr. 16-31314A, 16-31765A and RVO (FnBr, 65269705).

Deregulation of intestinal microRNAs (miRNAs) have been reported in adult and, in the last years, also in pediatric patients with Crohn’s disease (CD). We performed a literature search and chose 4 miRNAs from five relevant studies as candidates for an independent validation (miR-21-5p, miR-31-5p, miR-146a-5p, miR-155-5p). The goal of this study was to verify deregulated expression of the candidate miRNAs in inflamed terminal ileum of treatment-naive pediatric patients with CD. Methods We collected formalin-fixed paraffin-embedded terminal ileum biopsies from treatment-naive pediatric patients with CD (n=20, median age 15 (range 10-18) years, 9 females, 11 males) and matched controls (pediatric patients undergoing endoscopy for suspicion of polyp or IBD; N=10). Total RNA enriched for small RNAs was isolated, and expression levels of candidate miRNAs were detected by use of quantitative real-time PCR. Levels of the candidate miRNA were normalized to match the levels of the reference miRNA, and statistically evaluated. Results We confirmed that miR-21-5p and miR-146a-5p have significantly higher expression in the inflamed mucosa of terminal ileum of CD patients in comparison to the terminal ileum of healthy controls (P=0.047, AUC=0.732, and P=0.046, AUC=0.768; respectively). We further developed a model based on the combination of miR-21-5p and miR-146a-5p expression, which enabled the identification of CD patients with sensitivity 86% and specificity 75% (AUC = 0.898). We were not able to validate deregulated expression of miR-31-5p and miR-155-5p in pediatric CD patients. Conclusions To our knowledge, this pilot study is the first one to evaluate the candidate miRNA expression levels exclusively in the terminal ileum of pediatric CD patients. We confirmed some of the previous observations (e.g. increased levels of inflammatory miR-21-5p and miR-146-5p), but surprisingly miR-155-5p and miR-31-5p, previously observed to be increased in the inflamed colonic biopsies, were not deregulated in terminal ileum of our pediatric CD patients. Further research is necessary to clarify the pathophysiological roles of these miRNAs in pediatric CD. This work was supported by the Ministry of Health of the Czech Republic, grant nr. 16-31314A, 16-31765A and RVO (FnBr, 65269705).