Home Enteral Nutrition in Patients with Cerebral Palsy in the Years 2012-2022: A Longitudinal Analysis of Data from the National Health Fund of Poland.

BACKGROUND: Cerebral palsy (CP) often correlates with a higher risk of malnutrition, negatively affecting the quality of life of patients and their families. Enteral nutrition via a feeding tube should be considered to improve the nutritional status of CP patients. To date, there has been no nationwide registry of patients with CP in Poland. This study aimed to assess the prevalence of home enteral nutrition (HEN) provision in pediatric and adult patients with CP. METHODS: We retrospectively analyzed data from the Polish National Health Fund (NFZ) on the provision of HEN in patients with CP in 2012-2022. A specially designed and validated questionnaire was sent to the 16 regional branches of NFZ. RESULTS: Completed questionnaires were sent back from 12 NFZ branches. In 2022, CP cases increased by 7%, primarily among adults, while pediatric cases dropped by 21%. Despite a rising trend, the proportion of patients receiving HEN remained relatively low. Among children, it increased from 2.1% in 2012 to 3.3-3.5% in 2019-2021. For adults, it nearly doubled from 0.8% in 2012 to 1.7% in 2022. The prevalence of enteral feeding correlated with patient age, with a noticeable increase among older children and adolescents. CONCLUSIONS: National Health Fund data highlight the need for a nationwide registry of patients with CP. A relatively small proportion of pediatric and adult CP patients receive HEN. Increasing clinicians' awareness of HEN availability is necessary to improve the quality of life for more CP patients.

The Role of Vitamin D and Vitamin D Receptor Gene Polymorphisms in the Course of Inflammatory Bowel Disease in Children.

Background: The etiopathogenesis of inflammatory bowel disease (IBD) is still unclear. Prior studies suggest genetic components that may influence the incidence and severity of the disease. Additionally, it was shown that low levels of serum vitamin D may have an impact on the clinical course of the disease due to its effect on the immunological system. Methods: We aimed to investigate the correlation between the incidence of vitamin D receptor (VDR) gene polymorphisms (rs11568820, rs10735810, rs1544410, rs7975232, and rs731236, commonly described as Cdx2, FokI, Bsm, ApaI, and TaqI, respectively) and vitamin D concentration with the clinical course of IBD (disease activity, extent of the intestinal lesions). Data were obtained from 62 patients with IBD (34 with Crohn's disease, 28 with ulcerative colitis), aged 3-18 years, and compared with controls (N = 47), aged 8-18 years. Results: Although there was no difference in the incidence of individual genotypes between the study groups (IBD, C) in all the polymorphisms examined, we described a significant increase in the chance of developing IBD for heterozygotes of Cdx2 (OR: 2.3, 95% CI 0.88-6.18, p = 0.04) and BsmI (OR: 2.07, 95% CI 0.89-4.82, p = 0.048) polymorphisms. The mean serum 25OHD level in patients with IBD was significantly higher compared with the controls (19.87 ng/mL vs. 16.07 ng/mL; p = 0.03); however, it was still below optimal (>30 ng/mL). Furthermore, a significant correlation was found between vitamin D level and TaqI in patients with IBD (p = 0.025) and patients with CD (p = 0.03), as well as with the BsmI polymorphism in patients with IBD (p = 0.04) and patients with CD (p = 0.04). A significant correlation was described between the degree of disease activity and genotypes for the FokI polymorphism in patients with UC (p = 0.027) and between the category of endoscopic lesions and genotypes for the Cdx2 polymorphism also in patients with UC (p = 0.046). Conclusions: The results suggest a potential correlation of VDR gene polymorphism with the chance of developing IBD, and the clinical course of the disease requires further studies in larger group of patients. Vitamin D supplementation should be recommended in both children with inflammatory bowel disease and in healthy peers.

High-Resolution Anorectal Manometry as a Screening Tool for Hirschsprung's Disease: A Comprehensive Retrospective Analysis.

Hirschsprung's disease (HD) is characterized by a congenital absence of enteric ganglion cells in the intestine, posing challenges in diagnosis, particularly in pediatric patients. The gold standard, rectal suction biopsy (RSB), carries risks, prompting an exploration of non-invasive alternatives such as high-resolution anorectal manometry (HR-ARM) for HD screening. We conducted a retrospective analysis of 136 patients suspected of HD between 2018 and 2022, which were stratified into three age groups: ≤12 months, ≤24 months, and >24 months. Criteria for suspicion included delayed meconium passage, unresponsive chronic constipation, and abnormal prior test results. HR-ARM, supplemented by additional tests, confirmed 16 HD cases. HR-ARM exhibited 93.75% sensitivity, 89.47% specificity, 99.03% negative predictive value (NPV), and 55.56% positive predictive value (PPV). Notably, HR-ARM consistently performed well in patients ≤ 2 years old but demonstrated reduced efficacy in older children, which was likely due to complications from chronic constipation. This study underscores HR-ARM's promise as a non-invasive HD screening tool, especially in younger patients. However, its limitations in older children warrant consideration. Establishing standardized protocols, particularly for assessing the recto-anal inhibitory reflex, is crucial. Further research is imperative to optimize HR-ARM's diagnostic role across varied age groups in HD assessment.

Does Massive Bowel Resection in Newborns Affect Further Immunity in Children?

BACKGROUND: The massive resection of the small intestine leading to short bowel syndrome (SBS) deprives an organism of many immunocompetent cells concentrated in gut-associated lymphoid tissue, the largest immune organ in humans. We have aimed to access the influence of bowel resection on adaptive immunity in children, based on peripheral lymphocyte subsets and serum immunoglobulins. METHODS: 15 children who underwent bowel resection in the first months of their life and required further home parenteral nutrition were enrolled into the study. Based on flow cytometry, the following subsets of lymphocytes were evaluated: T, B, NK, CD4+, C8+, and activated T cells. RESULTS: Statistically significant differences were found for the rates of lymphocytes B, T, CD8+, and NK cells. The absolute count of NK cells was lower in the SBS group than in the control group. Absolute counts of lymphocytes, lymphocytes B, T, CD4+, and percentages of lymphocytes CD4+, and activated T cells inversely correlated with age in SBS group. CONCLUSIONS: Children with SBS do not present with clinical signs of immunodeficiency as well as deficits in peripheral lymphocyte subsets and serum immunoglobulins. The tendency of the lymphocyte subpopulations to decrease over time points out the necessity for longer follow- up.

Changes in Body Composition and Physical Performance in Children with Excessive Body Weight Participating in an Integrated Weight-Loss Programme.

The problem of overweight and obesity is a growing phenomenon in the entire population. Obesity is associated with many different metabolic disorders and is directly associated with an increased risk of death. The aim of the study was to assess the changes in body composition and physical fitness in children participating in an integrated weight-loss programme and to analyse the possible relationship between changes in body composition and improvements in fitness. Participants of the study were recruited from the "6-10-14 for Health"-multidisciplinary intervention programme for children aged 6 to 15 years old. A total of 170 patients qualified for the study, and 152 patients were enrolled. Statistically significant changes in body composition were found after the end of the intervention program, as measured by both BIA (bioimpedance) and DXA (Dual Energy X-ray Absorptiometry). The differences in KPRT (Kasch Pulse Recovery Test) results at baseline and after intervention are positively correlated with the difference in fat mass between baseline and the after-intervention measure. Improving physical fitness is positively correlated with a decrease in FM (fat mass) and an increase in FFM (fat-free mass) measured in both absolute values and %. Both BIA and DXA methods proved to be equally useful for measuring body composition.