Double Lumen Polyamide Tube-stent for the Treatment of Recurrent Postcorrosive Esophageal Stenosis.

This article presents the results of endoscopic treatment for recurrent postcorrosive esophageal stenosis with a tube-stent developed at our institution. The tube-stent was implanted in 5 children with corrosive esophageal injury at the age of 2 to 8.5 years after 7 to 64 dilatation sessions during 5 to 118 months. In total, 13 tube-stents were implanted. One patient had undergone 9 procedures during 2.5 years and the tube-stent remained in place for 14 to 250 days. This patient was tube-stent-dependent due to the lack of any possibility of surgical reconstruction. Two patients had the tube-stent removed after 150 to 205 days and they remain free from esophageal restenosis. One patient did not tolerate the tube-stent, evacuated it after 1 day and was referred for surgical esophagus replacement. One patient is currently still being treated with the tube-stent. Tube-stent was well tolerated and it may be effective in children with recurrent critical postcorrosive esophageal stenosis.

Early Onset of Wilson Disease: Diagnostic Challenges.

OBJECTIVES: The aim of the study was to analyze the clinical presentations, diagnosis, and treatment of patients ages ≤5 years with early onset Wilson disease (WD). METHODS: Data from 143 pediatric patients with WD treated at our center between January 1996 and November 2015 were retrospectively analyzed. RESULTS: A review of the 143 pediatric patients with WD identified 21 (10 girls, 11 boys) with first symptoms or abnormal liver function test results at age ≤5 years. The diagnosis of WD was confirmed in 8 patients younger than 5 years. At baseline the mean serum alanine aminotransferase level was 222 U/L and the mean serum aspartate aminotransferase level was 130 U/L. The mean serum ceruloplasmin concentration in 16 tested patients was <20 mg/dL. Of the 15 patients who underwent urinary copper excretion testing, 8 had levels between 40 and 100 µg/day, with only 4 having levels >100 µg/day. Liver copper quantification was >250 µg/g dry weight in 16 patients. The most common mutation was p.H1069Q, with compound heterozygosity in 5 patients and homozygosity in 9. Sixteen patients were treated with zinc salts and 5 with D-penicillamine. Both treatments were effective, with no serious side effects observed after 3 to 24 months. CONCLUSIONS: WD can present as early as 2 years of age. Because biochemical tests may be less sensitive in very young children, diagnoses may require a combination of tests. If molecular tests are inconclusive, liver copper content should be measured.

The clinical course of hereditary pancreatitis in children - A comprehensive analysis of 41 cases.

BACKGROUND: Available data from adult patients do not reflect natural course of hereditary pancreatitis (HP) in children. To date, no study has assessed the clinical course of HP in children. OBJECTIVE: To investigate the clinical course of HP in children and compare it to non-HP group with chronic pancreatitis (CP). METHODS: A group of 265 children with CP, hospitalized from 1988 to 2014, were enrolled in the study. Medical records of those patients were reviewed for data on presentation, diagnostic findings and treatment. All children were screened for mutations in major pancreatitis-associated genes, i.e. PRSS1, SPINK1, and CFTR. RESULTS: HP was diagnosed in 41 children (15.5%). Family history was positive in 88% of children with HP. Mutations of PRSS1 gene were found in 80% (33/41) of HP patients. We detected p.R122H, p.R122C, p.N29I, and p.E79K mutation in 34% (14/41), 27% (11/41), 12% (5/41), and 7% (3/41) of HP patients, respectively. Patients with paternal inheritance had first symptoms earlier than those with maternal inheritance (5.9 vs. 9.1 years; P < 0.05). Children with HP showed more severe changes in ERCP then those from non-HP group (2.05 Cambridge grade, vs. 1.6°; P < 0.05). ESWL was performed more frequently in HP group (12.2% vs. 3.1%; P < 0.05). There was no difference in age of disease onset (7.98 vs. 8.9 years; NS), pancreatic duct stenting (46.3% vs. 33%; NS), or number of surgical interventions (12.2% vs. 14.3%; NS) between both groups. CONCLUSIONS: Children with HP reveal significantly more severe clinical presentation of the disease than non-HP patients, despite the same age of onset.

The Etiology and Clinical Course of Chronic Pancreatitis in Children With Early Onset of the Disease.

OBJECTIVES: The etiological factors of chronic pancreatitis (CP) in children differ from those in adults. To date, no study has assessed the clinical course of CP in young children. The aim of our study was to evaluate the etiology and the clinical presentation of the disease in children with disease onset before 5 years of age in comparison to later-onset of CP. METHODS: A total of 276 children with CP, hospitalized from 1988 to 2015, were enrolled in the study. Data on presentation, diagnostic findings, and treatment were reviewed. Two hundred sixty patients were screened for the most frequent mutations in major pancreatitis-associated genes, such as cationic trypsinogen/serine protease gene (PRSS1), serine protease inhibitor, Kazal type 1 gene (SPINK1), and cystic fibrosis transmembrane conductance regulator gene (CFTR). RESULTS: The disease onset before the age of 5 years occurred in 51 patients (group 1), the later onset in 225 patients (group 2). We found no significant discrepancies in distribution of the etiological factors between groups. The youngest patients (group 1) had more pancreatitis episodes (median 5.0 vs 3.00; P < 0.05) and underwent surgeries more frequently (25.5% vs 8.9%; P < 0.05). It could be associated with significantly longer follow-up in early onset group (median 6 vs 4 years; P < 0.05). There were no differences in nutritional status or exocrine and endocrine pancreatic function. CONCLUSIONS: Early- and later-onset pancreatitis have similar etiological factors with predominance of gene mutations. The most frequent mutation found was p.Asn34Ser (N34S) in SPINK1 gene. The clinical presentation differed in number of pancreatitis episodes and frequency of surgeries.

Clinical evaluation of children with ulcerative colitis treated between 2013-2015 - own experience.

AIM: Evaluation of the changes in the endoscopic, laboratory and clinical status in children with ulcerative colitis (UC) with regard to the duration of the disease. MATERIAL AND METHODS: 91 children with UC were involved in the study. Each of them had colonoscopy and their laboratory values were tested. We assessed the colonoscopy results by the Paris classification and by the Baron score. Moreover, demographic, clinical and anthropometric data were collected. We divided our patients into five subgroups depending on the duration of the disease. In order to assess the changes in the variables, we conducted the Mann-Whitney U test. RESULTS: The most numerous group were patients whose disease had lasted between 1 and 2.5 years. At the time of assessment 39.6% did not have inflammation lesions in the mucosa and 60.4% were in sustained clinical remission. At the time of diagnosis 55% of the participants had pancolitis or extensive colitis and 66% had ulcers or ulcerations in the mucosa. We found a statistically significant decrease in the extension of the disease between the patient at diagnosis and the patient during the first year after diagnosis, with p=0.049, but there were no statistically significant differences in the activity of the inflammatory changes between those groups. No significant changes were found in laboratory values, apart from those pertaining to faecal calprotectin (FC). During our study 95% of the patients were exposed to mesalazin, 66% to corticosteroids, 57% to immunosuppressants and 10% to biologics. 20% of our patients were exposed to steroids more than once. CONCLUSIONS: The changes observed during colonoscopy in children with UC have a widespread localization and varied aggression. With the duration of the disease, inflammatory lesions tend to acquire more and more of the surface in the colon, but are not characterized by a progression of their activity. The issue requires further well-designed studies.

Clinical presentations of Wilson disease among Polish children.

INTRODUCTION: Wilson disease (WD) may present from early childhood up to the eighth decade, presenting with variable hepatic and neuropsychiatric symptoms. Establishing the diagnosis is straightforward if the major clinical and laboratory features are present. However, clinical phenotypes are highly varied and early, proper diagnosis can be challenging. AIM: The aim of our study was to analyze clinical presentations and diagnostic tests of Polish pediatric patients with WD. METHODS: We retrospectively analyzed medical history of 156 patients with confirmed diagnosis of WD treated at our Institute from 1996 till March 2016. RESULTS: The mean age at onset of symptoms was 10.15±4.23 years of age. Hepatic presentation was the most common one (94.23%) with either liver failure (16.03%) or more frequently increased transaminases (78.2%). In 90.26% cases ceruloplasmin serum concentration was ≤0,2 g/l, in 51.93% patients basal urinary copper excretion was >100 µg/24 h. Mutation analysis was performed in 155 (99.36%) cases. The most common mutation was p.H1069Q. CONCLUSIONS: Wilson disease can present with only significantly increased transaminases activity and hepatomegaly or liver failure, but neurological symptoms are very rare in children. Diagnostic approach is challenging due to wide spectrum of clinical presentations in a high variable degree of severity. Genetic screening is supportive, ceruloplasmin and urinary copper excretion are valuable tests in the majority of patients but do not allow to exclude WD.

Assessment of induction therapy with infliximab in children with moderate to severe ulcerative colitis: a multi-center study.

THE AIM OF THE STUDY: Assessment of clinical and endoscopic efficacy of induction therapy with infliximab in children with ulcerative colitis. MATERIAL AND METHODS: This is a retrospective analysis of medical records of pediatric patients with moderate to severe UC who had received at least one infusion of infliximab in Polish pediatric academic clinical centers from 2003 to 2013. The primary endpoint was clinical remission rate at week 10, (PUCAI score <10 points) while the secondary endpoints were: clinical response rate (>19-points decrease in PUCAI), mucosal response rate (defined as an improvement of the Baron score), and mucosal healing rate (Baron score 0 or 1). RESULTS: 44 patients, at mean age of 14±3.9 years, were included into the study. 38 (86%) patients completed induction therapy regimen with infliximab and were finally included into the analysis. Clinical response and remission rates at week 10 there were 36% and 25% respectively. There was significant drop of PUCAI (58.31±15.5 vs. 24.23±23.83) and Baron score (2.63±0.49 vs. 1.44±0.99) at this time point. Mucosal response and mucosal healing rate were 57% and 48% respectively. Infliximab failure defined as non-clinical and non-mucosal response at week 10, occurred in 16 patients. Infliximab-associated adverse events occurred in 3 patients, with all severe hypersensitivity reactions to infliximab. CONCLUSIONS: Infliximab induction therapy was safe and effective in Polish moderate to severe UC pediatric patients with 50% rate of mucosal improvement. However, clinical response rate was lower than previously reported.

Monotherapy with infliximab versus combination therapy in the maintenance of clinical remission in children with moderate to severe Crohn disease.

OBJECTIVES: The aim of the present study was to compare the efficacy and safety of 2 protocols of maintenance therapy with infliximab (IFX) and an immunomodulatory agent in pediatric patients with Crohn disease (CD): withdrawal of immunomodulators versus continuation of immunosuppressants. METHODS: The present multicenter randomized open-label trial included 99 patients with CD (ages 14.5 ±â€Š2.6 years) who were administered IFX (5 mg/kg body weight) along with an immunomodulatory agent (azathioprine 1.5-3 mg/kg body weight per day, methotrexate 10-25 mg/week). After 10 weeks of the induction therapy, 84 responders were centrally randomized into 1 of the following groups: group I (n = 45) in which IFX and an immunomodulatory agent were continued up to week 54 and group II (n = 39) in which the immunomodulatory agent was discontinued after 26 weeks. RESULTS: The induction therapy was reflected by a significant decrease in Pediatric Crohn's Disease Activity Index (PCDAI) and Simplified Endoscopic Activity Score for Crohn's Disease (SES-CD) values. After the maintenance phase, the analyzed groups did not differ significantly in terms of the clinical response loss rates and final PCDAI and SES-CD scores. Furthermore, no significant intragroup differences were documented between mean PCDAI scores determined at the end of induction and maintenance phases. Intensification/modification of the treatment was required in 13 of 45 (29%) and 11 of 39 (28%) patients of groups I and II, respectively. A total of 9 serious adverse events were documented; none of the patients died during the trial. CONCLUSIONS: Twenty-six weeks likely represent the safe duration of combined IFX/immunomodulatory therapy in our sample of pediatric patients with CD.

Efficiency of pancreatic duct stenting therapy in children with chronic pancreatitis.

BACKGROUND: Chronic pancreatitis (CP) is a rare disease in childhood. Although ERCP is commonly performed in children, the effect of pancreatic duct stenting therapy in children with CP is unknown. OBJECTIVE: To investigate the efficacy of pancreatic duct stenting in children with CP. DESIGN: Retrospective analysis. SETTING: National referral center. PATIENTS: A total of 208 children with CP hospitalized between 1988 and 2012. INTERVENTIONS: ERCP with pancreatic duct stenting. MAIN OUTCOME MEASUREMENTS: Results of endoscopic therapy and number of pancreatitis episodes per year before and after treatment. RESULTS: A total of 223 pancreatic duct stenting procedures were performed in 72 children. The median number of stent replacements was 3 (range 1-21). A statistically significant decrease in the number of pancreatitis episodes per year was observed: from 1.75 to 0.23 after endoscopic treatment (P < .05). Pancreatic duct stenting was performed more frequently in patients with hereditary pancreatitis (61.5%) and in children with CP and anatomic anomalies of the pancreatic duct (65%; P < .05). LIMITATIONS: Retrospective analysis with the assessment of adverse events based on medical history. CONCLUSION: Pancreatic duct stenting therapy is a safe and effective procedure in children with CP. This therapy should be recommended especially for children with hereditary pancreatitis and patients with anatomic anomalies of the pancreatic duct.

Pediatric endoscopy in times of pandemic: A nationwide retrospective analysis.

BACKGROUND: Gastrointestinal endoscopy is a procedure that carries an increased risk of transmission of SARS-CoV-2 infection to medical staff. In patients, COVID-19 is a risk factor for adverse events of medical procedures. This study analyzed the real-life risk of, and factors contributing to, infection transmission to endoscopic personnel, and possible adverse events of the endoscopy procedure and anesthesia in children with COVID-19. METHODS: Nationwide retrospective analysis of medical records of children with confirmed SARS-CoV-2 infection who underwent gastrointestinal endoscopy in Poland between February 2020 and February 2022. RESULTS: Fifty-eight patients were included in the analysis, 35% of whom had COVID-19 symptoms at the time of endoscopy. The dominant indications for endoscopy were foreign body or corrosive substance ingestion and gastrointestinal bleeding. Nine cases of virus transmission were registered among endoscopic personnel. In all of these cases, the endoscopy team was unaware of the patient's infection (p < 0.01), although symptoms were present in 78% of the children. Lack of use of personal protective equipment was the strongest predictor of SARS-CoV-2 transmission (p < 0.01). The risk of infection was not statistically significantly dependent on the method of anesthesia, intubation or the type of endoscopy. No statistically significant correlation was found between symptomatic infection and adverse events of endoscopy or anesthesia occurrence. There was one reported anesthesia-related adverse event involving extubation difficulties due to worsening respiratory infection symptoms. CONCLUSIONS: The risk of transmitting SARS-CoV-2 to endoscopic personnel during procedures in children is low and depends on compliance with infection prevention and control measures. Performing gastrointestinal endoscopy in children with COVID-19 does not appear to be associated with an increased risk of adverse events.

High- versus low-volume polyethylene glycol plus laxative versus sennosides for colonoscopy preparation in children.

OBJECTIVE: Many protocols of bowel preparation are available for use in children; however, none of them is commonly accepted. The aim of the study was to evaluate the efficacy and acceptability of high-volume polyethylene glycol (PEG) versus low-volume PEG combined with bisacodyl (BPEG) versus sennosides for colonoscopy preparation in children. METHODS: Participants ages 10 to 18 years were randomly assigned to receive either PEG 60 or PEG 30 mL kg⁻¹ day⁻¹ plus oral bisacodyl 10 to 15 mg/day or sennosides 2 mg kg⁻¹ day⁻¹ for 2 days. A blinded assessment of bowel cleansing was made by the endoscopist according to the Aronchick and Ottawa scales. Patient acceptability was evaluated with the visual-analog scale. Analysis was done on an available case analysis basis. RESULTS: Of 240 patients enrolled in the study 234 patients were available for analysis of the efficacy of colon cleansing. There were no significant differences found among the 3 groups for the proportions of participants with excellent/good (PEG: 35/79, BPEG: 26/79, sennosides 25/76) and poor/inadequate (PEG: 20/79, BPEG: 28/79, sennosides 28/76) bowel preparation evaluated with the Aronchick scale and for the mean Ottawa total score (PEG: 5.47 ±â€Š3.63, BPEG: 6.22 ±â€Š3.3, sennosides: 6.18 ±â€Š3.53). Acceptability of bowel cleansing protocol was similar in all of the groups (P = 0.8). CONCLUSIONS: All 3 cleansing methods showed similar efficacy and tolerability; however, none of them was satisfactory.

Biological markers of disease activity in inflammatory bowel diseases.

Inflammatory bowel diseases (IBD) are chronic intestinal conditions of multifactorial aetiology including genetic susceptibility, immunological impairment, dysbiosis, and environmental factors. The diagnosis is based on both clinical and endoscopic features, wherein histopathological evaluation remains a gold diagnostic standard. However, fast, reliable, and non-invasive biological markers have been used for years for diagnosis as well as for disease activity monitoring. Currently, commonly used faecal calprotectin is the only biomarker approved and recommended by the European Crohn's and Colitis Organization (ECCO). Nonetheless, other biological markers discriminating between functional and organic bowel conditions have been widely studied. Therefore, the aim of this manuscript was to review new potential biomarkers of inflammation in IBD. The aim of this study was to review currently available biomarkers of intestinal inflammation and increased gut permeability in IBD.

Immunological markers of drug resistant epilepsy and its response to immunomodulatory therapy with ACTH in children.

INTRODUCTION: Drug-resistant epilepsy in infancy and childhood is a devastating condition, frequently associated with neuropsychiatric comorbidities. West syndrome is one of the most severe epilepsy syndromes. Adrenocorticotropic hormone (ACTH) treatment is recommended in such cases, but its mechanism of action is still unknown. We prospectively observed levels of selected cytokines in order to identify biomarkers of response to ACTH and the potential mechanism of its antiseizure effect. MATERIAL AND METHODS: Fifty-three infants and young children with pharmacoresistant epilepsy receiving ACTH 1-24 were included. There were 2 control groups - children with epilepsy responding to the first medication and children with no history of epilepsy. Blood concentrations of IL-1b, IL-1Ra, IL-6, IL-8, IL-10, TNF-a, IFN-g, MCP-1 and MIP-1a were analyzed at three time points: T0 (before ACTH), T1 (after intensive ACTH treatment) and at T2 (at the end of ACTH withdrawal). The results were correlated with the response to treatment, dose of ACTH and concomitant medications. RESULTS: We found statistically significantly higher concentrations of IL-1, IL-8 and MIP-1a at baseline (T0) in the study group compared to the control groups. ACTH significantly lowered levels of IL-6, IFN-g and MCP from time T0 to T1. This effect was short lasting and no significant changes in cytokine levels were found between T2 and T0. We did not find any differences in immunological markers between the responders and non-responders to ACTH. Our research did not allow us to identify any reliable immunological marker of response to ACTH treatment. We did not observe a positive effect of higher ACTH doses on the response rate in the patients. Our study showed significantly lower concentrations of IL-10 and IFN in the group of patients receiving levetiracetam. The concentration of TNF was higher and the concentration of MIP was lower in the group receiving valproic acid. CONCLUSIONS: Our study indicates that increased levels of IL-1, IL-8 and MIP-1a are associated with drug-resistant epilepsy in infants and young children and might be considered immunological markers. IL-6, IFN-g and MCP-1 take part in the effect of ACTH. Immunological mechanisms seem also to be involved in the mechanism of action of classical antiseizure drugs.

New non-invasive biomarkers of intestinal inflammation and increased intestinal permeability in pediatric inflammatory bowel diseases and their correlation with fecal calprotectin: a pilot study.

BACKGROUND: Increased intestinal permeability is considered to play a crucial role in the pathogenesis of inflammatory bowel diseases (IBD). Therefore, recently, the use of non-invasive biomarkers in both diagnosis and monitoring IBD is emphasized. The aim of this study was to investigate fecal and serum zonulin and serum I-FABP in pediatric IBD patients and their correlation with fecal calprotectin (FCP). METHODS: Seventy-one individuals: 32 Crohn's disease (CD) patients, 33 ulcerative colitis (UC) patients and 6 controls were examined for fecal and serum zonulin and plasma I-FABP. Values were correlated to FCP and to each other for all children included in the study. A stool specimen and blood samples were collected during check-up visits at hospital. Then fecal and serum zonulin, I-FABP and FCP were tested by ELISA test. Non-parametric statistical tests were used for data analysis. RESULTS: The level of fecal zonulin and FCP were higher in IBD patients compared to control group (CG): median for CD - 46.0 (7.0-3854) ng/mL, 252.0 (77.0 -1054.2) ug/g; UC - 115.3 (50.7-418.3) ng/mL, 40 (16.0-1883.0) ug/g; CG - 60.8 (31.8-123.0) ng/mL, 41.5 (31.0-323.0) ug/g, respectively, (P<0.05). No statistically significant difference in concentrations of serum zonulin and I-FABP was reported between patients and CG (P=0.55). The only correlation that has been reported was between fecal zonulin and FCP and the strongest one was in CD: CD - R = 0.73, UC - R = 0.67, All - R=0.67, CG - R=0.65. CONCLUSIONS: According to our results it seems that only fecal zonulin may serve as another, next to FCP, biomarker of intestinal damage in IBD. However, both fecal and serum zonulin as well as IFABP need further studies to assess their usefulness in diagnostics and monitoring in IBD.

Fecal Zonulin as a Noninvasive Biomarker of Intestinal Permeability in Pediatric Patients with Inflammatory Bowel Diseases-Correlation with Disease Activity and Fecal Calprotectin.

BACKGROUND: Recent data indicate that increased intestinal permeability plays a key role in the pathogenesis of inflammatory bowel diseases (IBD) and correlates with disease flare. Since zonulin related proteins (ZRP) are the proteins that increase permeability in the epithelial layer of the small intestine by reversibly modulating the intercellular tight junctions, they may serve as a new, noninvasive biomarker of disease activity. The aim of this study was to investigate fecal ZRP in pediatric IBD patients as well as its correlation with disease activity and fecal calprotectin (FCP). METHODS: Ninety-four individuals: 47 Crohn's disease (CD) patients, 41 ulcerative colitis (UC) patients, and 6 healthy controls were examined for fecal ZRP. Values were correlated to IBD type, disease activity for IBD patients, and FCP for all children included in the study. A stool specimen was collected the day before the visit to the hospital, then fecal ZRP and FCP were tested using the ELISA test. Non-parametric statistical tests were used for data analysis. RESULTS: The level of fecal ZRP was higher among IBD patients compared to the control group (CG): medians for CD-113.3 (53.6-593.6) ng/mL; UC-103.6 (50.7-418.3) ng/mL; and CG-46.9 (31.8-123.0) ng/mL (p < 0.05). No difference in fecal ZRP concentration was observed between children with CD and those with UC (p = 0.55). A slight correlation between disease activity (PCDAI for CD and PUCAI for UC) and the fecal ZRP level was found for CD (p = 0.03/R = 0.33), but not UC (p = 0.62/R = 0.08), patients. A correlation between fecal ZRP and FCP was observed (R = 0.73, p = 0.00). CONCLUSIONS: Fecal ZRP levels are increased among those with IBD, are associated with CD activity, and strongly correlate with FCP. Further research into the role of intestinal permeability in IBD and the clinical usefulness of ZRP in IBD is warranted.

Usefulness of Colon Assessment by Magnetic Resonance Enterography in Pediatric Patients with Inflammatory Bowel Disease-Retrospective Case Series.

BACKGROUND: Magnetic resonance enterography (MRE) is an excellent way to study the small bowels. During such an examination, the colon is also seen within the field of study. The aim of this study was to evaluate the effectiveness of MRE in detecting characteristics of active inflammatory bowel disease (IBD) in the colon, in comparison to different features seen in colonoscopies. METHODS: This retrospective study was conducted with 41 children. Features of active inflammation we considered were wall thickening; contrast enhancement; incorrect signal in the DWI sequence in the MRE; and presence of ulceration, erosion, erythema, spontaneous bleeding and a decrease of the vascular pattern seen in colonoscopy. The colon was divided into six segments: caecum, ascending, transverse, descending, sigmoid and rectum. RESULTS: The sensitivity of MRE was, on average, 50-75%, and as high as 92-100%, depending on the segment. The most important feature for which there was the most dependencies was ulceration. In the analysis of intestinal wall thickness, the AUC value >0.8 was detected as ulceration (segments: cecum, ascending, descending colon, sigmoid), spontaneous bleeding (ascending colon and sigmoid) and decreased vascular pattern (ascending, transverse, descending colon). CONCLUSIONS: Evaluation of qualitative structural changes in MRE distinguishes patients with inflammation in colonoscopy from patients without lesions, with high diagnostic accuracy, albeit higher specificity than sensitivity.

Premedication Does Not Influence the Incidence of Infliximab Infusion Reactions in Pediatric Patients with Inflammatory Bowel Disease-A Single Center Case-Control Study.

BACKGROUND: Infusion reactions (IRs) are the most common adverse events (AEs) of infliximab (IFX) treatment in patients with inflammatory bowel disease (IBD). Prophylactic premedication (PM) with corticosteroids or antihistamines prior to IFX infusions has been used in clinical practice, but its efficacy is not known. The aim of this study was to assess the influence of steroid PM on IR incidence in pediatric patients with IBD receiving IFX. METHODS: We performed a case-control study that included pediatric patients with IBD receiving IFX. Patients were divided into four subgroups according to the agent and PM they received: Remicade (original drug) + PM, and two biosimilars-Reshma +/- PM, and Flixabi-PM. At our site, until 2018, PM with steroids was used as a part of standard IFX infusion (PM+); however, since then, this method has no longer been administered (PM-). IRs were divided into mild/severe reactions. Differences between subgroups were assessed with the appropriate chi-square test. Multivariate logistic regression was used to assess associations between PM and IR incidence, correcting for co-medication usage. RESULTS: There were 105 children (55 PM+, 44 male, mean age 15 years) included in the study who received 1276 infusions. There was no difference between the PM+ and PM- subgroups, either in incidence of IR (18.2% vs. 16.0% of patients, p > 0.05) or in percentage of infusions followed by IR (2.02% vs. 1.02% of infusions, p > 0.5). The OR of developing IR when using PM was 0.34, and the difference in IRs ratio in PM+ and PM- patients was not statistically significant (95% CI, 0.034-1.9). There were 11/18 (61.1%) severe IRs (anaphylactic shock) reported in all patients (both PM+ and PM-). CONCLUSION: At our site, the incidence of IR was low, and PM did not decrease the incidence of IR in pediatric patients with IBD receiving IFX. These results indicate that PM with steroids should not be a standard part of IFX infusion to prevent IR.

The Course of Ulcerative Colitis After Pediatric Liver Transplantation for Sclerosing Cholangitis.

BACKGROUND: Primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC) are often associated with ulcerative colitis (UC). The impact on the course of UC remains unclear, and up-to-date evidence in pediatric populations is scarce. The aim of the study was to analyze the course of UC in pediatric patients transplanted owing to PSC or ASC. MATERIAL AND METHODS: We retrospectively reviewed data from children with PSC/ASC and UC who underwent orthotopic liver transplantation (OLT). In all patients UC diagnosis was based on clinical presentation, endoscopy, and histology. RESULTS: Seventeen patients (9 female) with PSC or ASC underwent OLT from deceased donors at a median age of 16.8 years (range = 11.5-18.2 years). In 15 patients, UC was diagnosed before OLT (median age of diagnosis = 10.6 years; range = 6.6-18.0 years), and 2 patients developed UC after OLT. Ten patients (59%) presented with pancolitis on initial endoscopy. Disease activity was severe in 9 patients (53%) and most patients improved after initial treatment with steroids. Before OLT only 2 patients (13%) had severe disease activity. After OLT, 4 patients developed flares of the disease. These patients were successfully treated and remained in remission at the end of the posttransplant follow-up period (median = 3.76 years; range = 0.4-15.5 years). None of the patients developed colorectal cancer or underwent colectomy during 3.7 years of post-OLT follow-up. CONCLUSION: In our experience, the course of UC was not aggravated by OLT for PSC, and UC did not adversely affect patient or graft survival.

The level of faecal calprotectin as a noninvasive biomarker of mucosal healing in children with ulcerative colitis.

INTRODUCTION: Recently, faecal calprotectin (FC) has been used as a marker of inflammatory processes in the gastrointestinal tract, such as inflammatory bowel disease (IBD), and has served to assess and monitor disease activity, mucosal healing (MH), and disease recurrence. AIM: To assess the correlation between FC and endoscopic activity of inflammation. MATERIAL AND METHODS: This retrospective study included 81 patients with ulcerative colitis (UC), with a median age of 15 years (range: 3-18 years), who were treated in the Children's Memorial Health Institute (CMHI) between 2013 and 2015. Within the study group there were two sub-groups created: patients with Baron score = 0 (n = 34, 42%) and ≥ 1 (n = 47, 58%). RESUTS: Statistical analysis was performed using Statistica 10 software (StatSoft, USA), and the value of p ≤ 0.05 was established as a significance level. In patients with Baron score ≥ 1, significantly higher FC values and PUCAI scores were found in comparison to children with Baron score = 0. The level of FC had greater accuracy than the PUCAI score in differentiation between patients with Baron score = 0 and ≥ 1 (Z = -1.73, p = 0.082). There was a significant correlation between PUCAI score and FC (R = 0.55, p < 0.001). CONCLUSIONS: Faecal calprotectin may be a good, noninvasive biomarker of mucosal healing in paediatric patients with UC.