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Background and Aim: Even with increasing numbers of biologic agents available for management of ulcerative colitis (UC), infliximab (IFX) retains an important place in treatment of pediatric patients with this disease. As few reports have addressed outcomes in pediatric UC patients who had to discontinue IFX, we examined clinical course and prognosis after IFX failure in pediatric UC. Methods: A prospective cohort study of pertinent cases enrolled in the Japanese Pediatric Inflammatory Bowel Disease Registry between 2012 and 2020 was conducted to determine outcomes for pediatric UC patients who received IFX but required its discontinuation during follow‐up (IFX failure). Results: Of the 301 pediatric UC patients in the registry, 75 were treated with IFX; in 36 of these, IFX was discontinued during follow‐up. Severity of UC at onset and absence of concomitant immunomodulator therapy were significant risk factors for IFX failure (P = 0.005 and P = 0.02, respectively). The cumulative colectomy rate after IFX failure was 41.3% at 1 year and 47.5% at 2 years. Colectomy was significantly more frequent when IFX was discontinued before June 1, 2018, than when IFX was discontinued later (P = 0.013). This difference likely involves availability of additional biologic agents for treatment of UC beginning in mid‐2018 (P = 0.005). Conclusion: In pediatric UC patients, approximately 50% underwent colectomy during a 2‐year interval following IFX failure. Prognosis after IFX failure appeared to improve with availability of new biologic agents and small‐molecule drugs in mid‐2018. [ABSTRACT FROM AUTHOR]

Sweet syndrome (SS) is an acute febrile neutrophilic dermatosis. Many factors such as inflammatory and autoimmune diseases have been reported in its etiology. In this article, SS, which developed in a pediatric patient with ulcerative colitis, was presented and discussed in the light of the literature. SS can be seen both due to ulcerative colitis and azathioprine (AZT) treatment. Our case presented to the emergency department with high fever and erythematous plaques. Laboratory examination revealed leukocytosis, neutrophilia, high levels of both of erythrocyte sedimentation rate and C-reactive protein. Histopathological examination of biopsies taken from skin lesions revealed neutrophilic dermatosis consistent with SS. AZT treatment was discontinued in order to exclude the diagnosis of AZT-associated SS, however drug-related SS was excluded due to the absence of relapses after oral challenge with AZT. With systemic glucocorticoid therapy, the patient's fever and rash completely resolved and did not recur in the follow-up. Patients with acute onset erythematous plaques or nodules and high fever who have been diagnosed with SS based on histopathological analysis and clinical findings should be started on systemic glucocorticoids as soon as possible. [ABSTRACT FROM AUTHOR]

Background: Pediatric ulcerative colitis (UC) treatment has changed dramatically with the introduction of multiple biologics. The goal of this study was to determine the effectiveness of these new biologics on achieving remission, nutritional impact, and eventual need for surgery in children. Methods: We retrospectively analyzed hospital records of UC patients (ages 1-19) seen at a pediatric gastroenterology clinic between January 2012 and August 2020. Patients were divided into groups: 1) medically without biologics or surgery; 2) patients treated with one biologic; and 3) patients treated with multiple biologics 4)patients that underwent colectomy. Results: There were 115 UC patients with a mean follow-up of 5.9 ± 3.7 years (1 month-15.3 years). PUCAI score at diagnosis was mild in 52 patients (45%), moderate in 25 (21%), and severe in 5 (4.3%). PUCAI score for 33 patients (29%) could not be calculated. There were 48 (41.3%) in group 1 with 58% remission, 34 (29.6%) in group 2 with 71% remission, 24 (20.8%) in group 3 with 29% remission, and only 9 (7.8%) in group 4 with 100% remission. The majority (55%) of surgical patients had colectomy within the first year of diagnosis. BMI improved after surgery (P = 0.001). The change from one biologic to others did not improve nutrition over time. Discussion: New biologics are changing the landscape in maintaining remission from UC. The current need for surgery is much lower than previously published studies. In medically refractive UC, nutritional status only improved after surgery. Addition of another biologic for medically refractory ulcerative colitis in order to avoid surgery must take into account the positive impact surgery has on nutrition and disease remission. [ABSTRACT FROM AUTHOR]

Objectives: We aimed to evaluate the frequency of extraintestinal manifestations (EIM) in children with inflammatory bowel disease (IBD) and the correlation of EIM with disease activity index, disease type and disease age. Methods: Records of patients who were under the 18 years of age and followed up with the diagnosis of IBD were included in the study. The demographic characteristics of the patients, the age of the patients, the sex of the patients, the type of disease and the age of the disease were recorded. When patients were enrolled in the study disease activity indexes were calculated by examining the physical examination and laboratory values of the patients in their last visits, by using the Pediatric Ulcerative Colitis Activity Index (PUCAI) in Ulcerative Colitis (UC) and the Pediatric Crohn's Disease Activity Index (PCDAI) in Crohn's Disease (CD). Results: It was conducted with a total of 44 patients, 40.9% (n=18) females and 59.1% (n=26) males diagnosed with IBD. The ages of the patients participating in the study ranged from 8 to 19 and the mean was found to be 14.64±3.19 years. According to the type of disease; 27.3% of the patients were CD and 72.7% were UC. When the disease activity is examined; 37.2% were in remission, 37.2% were mild, 16.3% moderate and 9.3% severe. The incidence of EIM in females is 77.8% and 65.4% in males. The incidence of EIM is 75% in CD and 68.8% in UC. Hepatobiliary involvement was detected in 41.5% (n=17) of the patients, joint involvement in 29.5% (n=13), osteopenia in bone in 16.3% (n=7), osteoporosis in 7% (n=3), ocular involvement (uveitis) in 2.3% (n=1) and skin involvement (erythema nodosum) in 2.3% (n=1). Conclusion: EIM are common in childhood IBD patients. It should be kept in mind that EIM are as common in UC as CD. [ABSTRACT FROM AUTHOR]

Evaluating progression risk and determining optimal therapy for ulcerative colitis (UC) is challenging as many patients exhibit incomplete responses to treatment. As part of the PROTECT (Predicting Response to Standardized Colitis Therapy) Study, we evaluated the role of the gut microbiome in disease course for 405 pediatric, new-onset, treatment-naive UC patients. Patients were monitored for 1 year upon treatment initiation, and microbial taxonomic composition was analyzed from fecal samples and rectal biopsies. Depletion of core gut microbes and expansion of bacteria typical of the oral cavity were associated with baseline disease severity. Remission and refractory disease were linked to species-specific temporal changes that may be implicative of therapy efficacy, and a pronounced increase in microbiome variability was observed prior to colectomy. Finally, microbial associations with disease-associated serological markers suggest host-microbial interactions in UC. These insights will help improve existing treatments and develop therapeutic approaches guiding optimal medical care.

Ulcerative colitis is a chronic inflammatory disease of the colon that carries a significant disease burden in children. Therefore, new therapeutic approaches are being explored to help children living with this disease. Fecal microbiota transplantation (FMT) has been successful in some children with ulcerative colitis. However, the mechanism of its therapeutic effect in this patient population is not well understood. To characterize changes in gut microbial and metabolomic profiles after FMT, we performed 16S rRNA gene sequencing, shotgun metagenomic sequencing, virome analysis and untargeted metabolomics by gas chromatography-time of flight-mass spectrometry on stool samples collected before and after FMT from four children with ulcerative colitis who responded to this treatment. Alpha diversity of the gut microbiota increased after intervention, with species richness rising from 251 (S.D. 125) to 358 (S.D. 27). In responders, the mean relative abundance of bacteria in the class Clostridia shifted toward donor levels, increasing from 33% (S.D. 11%) to 54% (S.D. 16%). Patient metabolomic and viromic profiles exhibited a similar but less pronounced shift toward donor profiles after FMT. The fecal concentrations of several metabolites were altered after FMT, correlating with clinical improvement. Larger studies using a similar multi-omics approach may suggest novel strategies for the treatment of pediatric ulcerative colitis.

Background: Although ulcerative proctitis (UP) in children is considered relatively mild, some patients have proximal disease extension and require immunosuppressive treatment. We investigated clinical characteristics and course of refractory UP in a multicenter pediatric cohort., Methods: Analyzing data obtained between 2013 and 2022 at 10 institutions specializing in pediatric inflammatory bowel disease, we elucidated natural history and factors predicting a need for immunosuppressive UP treatment. We compared patients given immunosuppressants and/or biologic agents (immunosuppressive treatment group) with those given 5-aminosalicylic acid (5-ASA) alone (5-ASA group)., Results: Fifty-five patients were followed for 3.5 years. The median Pediatric Ulcerative Colitis Activity Index at diagnosis was 20. The commonest treatment, 5-ASA suppository monotherapy in 40% of patients, showed the worst compliance. Clinical remission was achieved at least once in 95% of all patients. Disease extension beyond the splenic flexure occurred in 51%. Immunosuppressive treatment was given to 37%; biologic agents were used for 18%. Rates of endoscopically demonstrated inflammation, including Ra/Rs at diagnosis and extension beyond the left-sided colon, were higher in the immunosuppressive treatment group (70% vs 38%, P < 0.05; 95% vs 27%, P < 0.0001). The log-rank test and multivariate Cox proportional hazards regression showed that time to first clinical remission exceeding 3 months predicted the need for biologics., Conclusion: The typical initial treatment of pediatric UP was 5-ASA suppositories, despite poor compliance. Biologics or other immunosuppressive treatments were needed in 37% of patients. Close follow-up with adjustment of treatment should be considered in children with UP as its clinical course varies., (© 2024 Crohn’s & Colitis Foundation. Published by Oxford University Press on behalf of Crohn’s & Colitis Foundation.)

On February 24, 2021, the U.S. Food and Drug Administration (FDA) approved an efficacy supplement for HUMIRA® (adalimumab) injection to expand the indication of treatment of moderately to severely active ulcerative colitis (UC) to include pediatric patients 5 years of age and older. The effectiveness in pediatric patients with moderately to severely active UC was studied in a multicenter, randomized, double-blind trial (Study PUC-I, NCT02065557) in 93 pediatric patients 5 to 17 years of age. Adalimumab has been widely studied in multiple indications in adult and pediatric populations with a well-established safety profile; no apparent exposure-safety relationship has been identified in various pediatric populations treated with adalimumab across multiple indications. The approved dosing regimen in pediatric patients with UC differs from the regimen studied in the clinical trial and was determined based on a model-informed exposure bridging strategy, incorporating both efficacy and safety considerations. Specifically, the differences included switches from body weight-based (mg/kg) dosing regimens used in the pediatric trial to body weight-tiered, fixed-dose regimens, changes in dosing schedule, and the addition of an option of a less frequent dosing regimen for maintenance that was not studied in the clinical trial. This article provides a case example of successful model-informed drug development (MIDD), where modeling and simulation were utilized in combination with observed data from a clinical trial of limited size and scope to ultimately support the adalimumab approval in pediatric patients with UC. [ABSTRACT FROM AUTHOR]

Anemia is the most common extraintestinal manifestation and complication of inflammatory bowel disease (IBD). The aim of our study was to assess the prevalence of anemia in newly diagnosed pediatric patients with IBD and to analyze its association with disease type, extent, and severity. We retrospectively reviewed the medical records of all patients with IBD treated in our department in the period of November 2011 to November 2020. The final analysis included the records of 80 children with newly diagnosed IBD: 45 with ulcerative colitis (UC) and 35 with Crohn’s disease (CD). The prevalence of anemia was 60.0% in the UC patients and 77.1% in the CD patients. Of the UC patients with anemia, 37.1% had pancolitis, 18.5% extensive disease, 33.3% left-sided colitis and 11.1% ulcerative proctitis. Of the CD patients with anemia, 81.5% had ileocolonic disease, 11.1% colonic disease and 7.4% ileal disease. Anemia was less common in patients with mild disease than in patients with moderate–severe disease (22.2 vs. 77.8%, p < 0.001 in UC and 25.9% vs. 74.1%, p < 0.001 in CD). Our study confirmed anemia as a frequent problem in pediatric patients with IBD. Children with more extensive and more severe disease are at higher risk to develop anemia.

Pediatric-onset inflammatory bowel disease differs from adults in its epidemiological and clinical characteristics and courses. Since it is diagnosed at a young age, the duration of the disease is relatively longer than in adults. Therefore, it is necessary to select drugs in consideration of long-term risks and benefits, and efforts such as therapeutic drug monitoring to maximize the treatment effects and minimize side effects are required. In addition, special considerations for treating pediatric-onset inflammatory bowel disease include attention to the effects of the disease on growth and development, nutrition, and psychosocial problems. In children, more aggressive treatment is needed to avoid missing therapeutic window of opportunity during periods of rapid growth and development. Finally, efforts should be made to ensure that the therapeutic goals of mucosal healing are achieved, the quality of life is restored, and the transition to adult therapy is well carried out.

Introduction: Long-term disease duration of ulcerative colitis (UC) is known to increase the risk of developing colorectal cancer in adults; however, this association has not been genetically analyzed in children with UC. Herein, we examined the expression of cancer-related genes in the colonic mucosa of pediatric UC patients and their risk of developing colorectal cancer. Methods: Microarray analysis of cancer-related gene expression was conducted on rectal mucosa biopsy specimens randomly selected from pediatric cases, including 4 active-phase UC cases, 3 remission-phase UC cases, and 3 irritable bowel syndrome control cases. The subject pool was then expanded to 10 active-phase cases, 10 remission-phase cases, and 10 controls, which were analyzed by real-time polymerase chain reaction (PCR) and immunohistochemical staining. Results: The microarray results indicated significantly higher expression levels of cancer-related genes PIM2 and SPI1 in the active group than in the remission and control groups (p < 0.05). Real-time PCR confirmed that PIM2 and SPI1 expression levels were significantly higher, whereas TP53 and APC expression levels were significantly lower, in the active-phase group than in the remission and control groups (p < 0.05). Immunohistochemical staining for PIM2, SPI1, TP53, and APC proteins supported the real-time PCR results. Conclusions: Expression levels of previously unreported cancer-related genes in adult UC patients were significantly higher in pediatric UC patients than in controls. Inflammation of the gastrointestinal mucosa increased the expression levels of cancer-related genes even in childhood-onset UC cases, suggesting that chronic inflammation from childhood may increase the risk of colorectal cancer development. [ABSTRACT FROM AUTHOR]

Background: Pediatric ulcerative colitis (UC) is more challenging to treat than adult UC. Qing-Dai therapy is effective in adults but reports of its efficacy in children are unavailable. We conducted a questionnaire survey on Qing-Dai use among pediatric patients with UC in Japan to determine its efficacy and safety. Methods: Questionnaires were sent to 31 high-volume centers treating pediatric patients with inflammatory bowel disease. The number of patients using Qing-Dai, short-term and long-term effects, and adverse events were assessed. A systematic review of studies on the efficacy and safety of Qing-Dai usage for UC was also performed. Results: Overall, 29/31 facilities (93.5%) responded, Qing-Dai was used in 107 patients with UC, and 84/107 patients (78.5%) initiated treatment. Within 6 months, 81/101 (80.2%) patients had clinical remission, while 59/92 (64.1%) patients had no relapse and 29/92 (31.5%) experienced only one to two relapses yearly. Eighty-seven percent of the patients underwent regular follow ups for adverse events, among whom one patient was diagnosed with pulmonary arterial hypertension (PAH), five with enteritis, and one with headache. In the systematic review, the clinical remission rate was 50–80%, and PAH was observed in 14 of 1,158 patients (1.2%). Conclusions: Qing-Dai is highly effective in treating pediatric UC. However, Qing-Dai should be administered with caution as it may cause adverse events such as PAH. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Ulcerative colitis (UC) is a chronic autoimmune inflammatory disease of the colon that infrequently affects children. The disease requires immunosuppressive therapy to achieve remission and keep the disease in remission. Currently, many therapies are approved for use in pediatric patients with UC, including steroid, 5-aminosali-cylic acid (5-ASA), azathioprine, and biologic therapy with anti-tumor necrosis factor (TNF) inhibitors. Despite their efficacy, many patients have refractory severe disease that fails therapy and may require surgical interventions. Recently, the small molecule Janus Kinase (JAK) inhibitor tofacitinib has been approved for moderate to severe UC that fails biologic therapy in adults. However, the safety and efficacy of this drug has not been tested in pediatric UC patients. Case Report: We describe a case of a 13-year-old girl with 2-year history of severe UC who had secondary loss response to both infliximab and adalimumab over 2 years, despite adequate trough serum drug levels and the concomitant use of azathioprine. She was also dependent on steroid to control her disease. Infectious work-ups were always negative for infectious organisms. She was then successfully treated with tofacitinib 5 mg orally twice daily. She went into complete clinical, endoscopic, and steroid-free remission. Conclusions: This case report highlights the safety and efficacy of tofacitinib in pediatric patients with severe refractory UC, potentially avoiding proctocolectomy in this young patient population. Future research should study the role of tofacitinib in patients with moderate to severe UC in children. [ABSTRACT FROM AUTHOR]

Ileal pouch-anal anastomosis (IPAA) is the procedure of choice for reconstruction after total proctocolectomy in pediatric patients with ulcerative colitis. 30–60 % of patients undergoing IPAA will experience a postoperative complication. The primary objective of this article is to address the most common complications specific to IPAA in the pediatric population and provide an up-to-date review of their presentation, risk factors, workup, and management. We also share our preferred approaches to management and prevention of complications, where relevant. We intend to provide a concise review on the topic aimed at pediatric surgeons and healthcare providers involved in the care of this population with the goal of contributing to improved outcomes and patient quality of life. [ABSTRACT FROM AUTHOR]

Introduction: The incidence of early-onset inflammatory bowel disease is increasing in Japan. Objective: This study aimed to analyze the treatment and progress of early-onset inflammatory bowel disease. Methods: This prospective survey evaluated the data of 43 patients aged <8 years who were diagnosed with inflammatory bowel disease (IBD) from the time of diagnosis to 36 months after registration. Results: A total of 12 patients with Crohn's disease (CD), 21 with ulcerative colitis (UC), and 3 with unclassified IBD were enrolled. The mean disease onset age was 3 years and 7 months. Colon and anal lesions were present in 100 and 50% of patients with CD, respectively. Granulomas were detected in 5 patients (41.7%). Dietary elimination including elemental diet was performed in all patients. Eleven patients (91.7%) were in remission by initial induction therapy, and 72.7% maintained remission for 36 months. Three patients (14.3%) with UC had familial history, 71.4% had pancolitis-type UC, and 66.7% exhibited disease of moderate severity. Colectomy was performed in 4 patients (21.1%). Eighteen patients (85.7%) were in remission by initial induction therapy; however, only 15.8% maintained remission for 36 months. Anal complication was more prevalent in infantile-onset IBD than in childhood-onset IBD (p = 0.014). Conclusions: Among Japanese patients aged <8 years who were diagnosed with IBD, colitis-type disease was more common in CD and pancolitis was more common in UC. As the courses of several patients were severe, identifying primary immunodeficiency appears to be necessary to confirm background disease. [ABSTRACT FROM AUTHOR]

The musculoskeletal system can be involved as an extra-intestinal manifestation of inflammatory bowel disease. Among these, myositis in ulcerative colitis (UC) is very rare. A 14-year-old girl was admitted due to severe shoulder tenderness. She had complained of left jaw pain and swelling for the past 10 days. Inflammatory markers were elevated with no evidence of infectious etiology. Myositis was suspected by shoulder magnetic resonance imaging. Three days after admission, she developed hematochezia. Muscle biopsy and colonoscopy was performed due to worsening left mandibular area pain and persistent hematochezia. Colonoscopy showed consistent findings with UC. She was finally diagnosed with UC with myositis as an extra-intestinal manifestation. She showed a dramatic response to UC treatment. Gastrointestinal symptoms were well-controlled. After 14 months, UC symptoms and muscle pain were aggravated, which were relieved after steroid and cyclosporin treatment. We report a unique case of UC initially presented with myositis, preceding gastrointestinal symptoms. [ABSTRACT FROM AUTHOR]

Background: Pediatric inflammatory bowel disease (PIBD) affects different age groups and its incidence is increasing worldwide. However, there is a lack of research focusing on age subgroups in Asian countries. In this nationwide population-based study, we investigated the epidemiology of PIBD among different age subgroups in Korea., Methods: We analyzed Korean health administration data from 2005 to 2016. Data were divided by age at diagnosis as follows: group 1, 0-1 years; group 2, 2-5 years; group 3, 6-9 years; group 4, 10-16 years. We analyzed the overall incidence, temporal changes, and regional differences by age subgroups, using Poisson regression analysis., Results: From 2005 to 2016, 2734 inflammatory bowel disease (IBD) cases were diagnosed among patients under 17 years of age. In the overall population, the incidence rate of PIBD over the entire study period was 2.248/10 5  person-years (PY), significantly increasing from 1.173/10 5  PY in 2005-2007 to 3.267/10 5  PY in 2014-2016. The incidence rates in groups 1 and 2 remained unchanged, whereas those of groups 3 and 4 increased significantly. The same trend was observed when analyzed separately for Crohn's disease (CD) and ulcerative colitis (UC). The incidence rates of CD in groups 3 and 4 showed differences between metropolitan and non-metropolitan areas, whereas those in groups 1 and 2, and UC of all age subgroups showed no difference., Conclusions: The temporal trend and regional differences of PIBD differed among age subgroups, suggesting that genetic and environmental factors have varying impacts on IBD development across different subgroups., (© 2024 Japan Pediatric Society.)

OBJECTIVES: Epidemiological studies on pediatric‐onset inflammatory bowel disease (PIBD) are scarce in South–East Asia (SEA). This study aimed to evaluate the incidence trend and clinical characteristics of PIBD in a SEA cohort in Singapore over 22 years (1994–2015). METHODS: Case records of PIBD ≤18 years from the only two tertiary pediatric hospitals in Singapore were reviewed. The mean annual incidence (MAI) of PIBD was calculated based on Singapore's age‐specific population data. RESULTS: Overall MAI of PIBD was 1.26 per 100 000 (95% confidence interval [CI] 0.56–1.96). During the first decade (1994‐2004) MAI was 0.23 per 100 000 (95% CI 0.08–0.39); this rose almost 10‐fold to 2.28 per 100 000 (95% CI 1.15–3.41) during the second decade (2005–2015). Linear regression analysis showed significant increase in MAI over the 22‐year period (r = 0.826, P < 0001). Of the 228 patients, 61.0% had Crohn's disease (CD), 30.3% ulcerative colitis and 8.7% IBD‐unclassified, with a mdian age at diagnosis of 10.47 years and a male predominance (58.3%); 37.7% of them aged <10 years at diagnosis and 17.5% were very early‐onset IBD. In CD, 27.3% had stricturing and/or penetrating disease and 21.6% were with perianal disease. Indians had a disproportionately high representation while positive family history was rare (1.3%). CONCLUSIONS: Although PIBD is uncommon in Singapore, its incidence has risen dramatically over recent decades. A younger age of disease onset and higher proportions of perianal and stricturing/penetrating diseases suggest more aggressive disease than in Western data. [ABSTRACT FROM AUTHOR]

Children and young adults with ulcerative colitis tend to present with more extensive colonic disease than an adult population. The need for surgical intervention in the pediatric population with ulcerative colitis occurs earlier after diagnosis and has a greater incidence than a comparably matched adult population with an estimated need for colectomy at 5 years following diagnosis of 14-20%. Perhaps, even more than the adult population, there is a desire to restore intestinal continuity for the pediatric patient to achieve as healthy and normal quality of life as possible. With surgery playing such a prominent role in the treatment of ulcerative colitis in this age group, an understanding of the surgical treatment options that are available is important. The surgeon's awareness of the complexities of the different operations associated with proctocolectomy and reestablishing intestinal continuity may help to avoid early complications and minimize the risk of less than ideal long-term outcomes. [ABSTRACT FROM AUTHOR]

Background: There is currently no consensus on the definition of an abnormal intestinal ultrasound (IUS) for children with ulcerative colitis (UC). This cross-sectional study aimed to externally validate and compare 2 existing IUS indices in children with UC., Methods: Children undergoing colonoscopy for UC assessment underwent IUS the day before colonoscopy, assessed with the Mayo endoscopic subscore. The UC-IUS index and the Civitelli index were compared with the Mayo endoscopic score in the ascending, transverse, and descending colon. The area under the receiver-operating characteristic curve for detecting a Mayo endoscopic score ≥2 of both scores was compared and sensitivity and specificity were calculated., Results: A total of 35 UC patients were included (median age 15 years, 39% female). The area under the receiver-operating characteristic curve was higher for the UC-IUS index in the ascending colon (0.82 [95% confidence interval (CI), 0.67-0.97] vs 0.76 [95% CI, 0.59-0.93]; P = .046) and transverse colon (0.88 [95% CI, 0.76-1.00] vs 0.77 [95% CI, 0.60-0.93]; P = .01). In the descending colon, there was no difference (0.84 [95% CI, 0.70-0.99] vs 0.84 [95% CI, 0.70-0.98]). The optimal cutoff for the UC-IUS was <1 point to rule out a Mayo endoscopic score ≥2 (sensitivity: 88%, 100%, and 90% in the ascending, transverse, and descending colon, respectively) and a Mayo endoscopic score ≥2 could be detected using a cutoff of >1 (specificity: 84%, 83%, and 87%, respectively). For the Civitelli index, in our cohort, the optimal cutoff was <1 to rule out a Mayo endoscopic score ≥2 (sensitivity 75%, 65%, and 80%, respectively) and a cutoff >1 to detect a Mayo endoscopic score ≥2 (specificity 89%, 89%, and 93%, respectively)., Conclusions: In this cohort, the UC-IUS index performed better than the Civitelli index. The UC-IUS index had both a high sensitivity and specificity in this cohort, when using 1 point as cutoff for a Mayo endoscopic score ≥2., (© 2022 Crohn’s & Colitis Foundation. Published by Oxford University Press on behalf of Crohn’s & Colitis Foundation.)

Surgery for the treatment of ulcerative colitis (UC) can be performed in one-, two-, or three-stage procedures [1]. The more traditional approach is a total proctocolectomy and creation of an ileo pouch-anal anastomosis and diverting stoma at the initial operation, followed by ileostomy closure several weeks later (TIPPA) [1]. An alternative is an initial subtotal colectomy and end ileostomy [2]. In this alternative approach (NIPAA), a completion proctectomy and definitive ileo pouch-anal anastomosis can be performed without a diverting stoma. We hypothesize that functional outcomes following a NIPAA approach when performed in children, in our experience, are likely similar or improved when compared to those treated by TIPAA. After IRB approval, a review of patients who underwent a two-stage Laparoscopic IPAA from 2004 to 2017 occurred. Data included demographics, diagnosis, surgical intervention time to full diet, level of continence, use of antidiarrheals and complications. N = 41 (NIPAA = 14, TIPAA = 27). After establishment of bowel continuity, no significant differences in appetite recovery, continence, or complications were noted. The number of antidiarrheals prescribed were significantly higher in the TIPAA group (p = 0.01). Thirteen patients (31.7%) had pouchitis: 4 NIPAA and 9 TIPAA (p = NS). Of the 41 patients, 11 required subsequent surgery; 2 patients (18.2%) received NIPAA and 9 (81.8%) received TIPAA (p = 0.20). Two TIPAA patients received a diverting ileostomy owing to chronic anal pain and failure to achieve continence. This study suggests children with medically refractory UC treated by NIPAA or TIPAA have similar outcomes. Minimal differences in overall outcome were noted following either approach. However, NIPAA may reduce reliance on antidiarrheals to achieve satisfactory defecation outcomes. III Retrospective comparative study. [ABSTRACT FROM AUTHOR]

5-Aminosalicylic acid preparations have been used as first-line drugs for treatment of ulcerative colitis (UC). However, some patients with UC present with exacerbation of symptoms because of allergy to mesalazine. Diagnosis of mesalazine allergy in active UC may be challenging because its symptoms mimic those of UC. Here we describe a 13-year-old boy with mesalazine allergy who achieved remission when his medication was changed from mesalazine to salazosulfapyridine. During his clinical course mesalazine was prescribed twice, and on each occasion exacerbation of the symptoms occurred. We considered a diagnosis of mesalazine allergy, and this was confirmed by a drug lymphocyte stimulation test; the result for salazosulfapyridine was negative. On the basis of criteria involving simple mucosal biopsy combined with endoscopy for predicting patients with UC who would ultimately require surgery, we considered that the UC in this case might be susceptible to steroid treatment, and we therefore treated the patient with salazosulfapyridine and prednisolone. Shortly afterwards, remission was achieved and the patient has remained in good condition on salazosulfapyridine alone. When treating patients with mesalazine, the possibility of allergy should always be borne in mind, especially when the clinical course is inconsistent with the results of biopsy. [ABSTRACT FROM AUTHOR]

Fecal microbiota transplantation (FMT) is a treatment designed to correct gut dysbiosis by administration of feces from a healthy volunteer. It is still unclear whether FMT for children with ulcerative colitis (UC) is effective or hazardous. Here we describe a young patient to have received FMT for UC. A three-year-old girl was admitted to our hospital with severe active UC, and treated with aminosalicylates and various immunosuppressive drugs. As remission was not achieved, we decided to try FMT before colectomy. We administered donor fecal material a total of six times by retention enema (×2) and via a nasoduodenal tube (×4) within 10 days. The patient developed abdominal pain and pyrexia after each FMT session. Analyses revealed the transferred donor fecal microbiota had not been retained by the patient, who ultimately underwent colectomy. The severity of the UC and/or timing of FMT may have partly accounted for the poor outcome. [ABSTRACT FROM AUTHOR]

Purpose We aim to investigate the postoperative outcomes, bowel habits and quality of life (QoL) of younger pediatric ulcerative colitis (UC) patients following surgical intervention compared to an older pediatric population. Methods Medical records of UC patients after colectomy with ileoanal reconstruction (2002–2013) at our institution were reviewed. Patients/parents completed a QoL, bowel habits and disease course questionnaire. Surgical outcomes, bowel habits and QoL were reported comparing the younger (≤ 11 years old, n = 26) to older (> 11 years old, n = 38) cohorts. Results The mean age at colectomy was 7.04 ± 0.63 years vs 14.71 ± 0.32 years in the two groups. Patients had a significant (P < 0.001) reduction in stooling frequency after surgery in both age groups and had favorable rates of fecal continence. The frequency of pouchitis and postoperative small bowel obstruction was similar in both cohorts. Dehydration was slightly increased in the younger population but not significant. Anastomotic leak and stricture rates were slightly reduced in younger patients. Postoperative QoL was favorable and similar regardless of age at surgery. Conclusions Colectomy with ileoanal anastomosis for young children (≤ 11 years old) with UC is without increased complications relative to older patients and maintains a postoperative QoL and stool patterns. [ABSTRACT FROM AUTHOR]

Gastrointestinal system diseases significantly affect physical and emotional development of child and vary from simple absorbsion disturbance to severe malabsorbsion. Early diagnosis and treatment play important role because direct affect of these diseases on growth development. Many of these diseases damage enterocytes, enteric nerve system cells, smooth muscle cells (myocytes) or interstitial Cajal cells (ICCs). Gastrointestinal system diseases usually have multifactorial and polygenic inheritance except some diseases such as cystic fibrosis and familial polyposis coli. Recent years, new genetic methods, next generation sequencing and exom sequencing, have started to illuminate genetic etiology of polygenic diseases. Inherited diseases related chronical problems in childhood period are important because of many of them require hospitalization. Genetic tests may help to certain diagnosis and prediction of the clinical problems of an inherited disease. Recently, many medical therapy options have been tried according to genetic etiology. In next years, when genetic etiology of gastrointestinal diseases explore, personalised medical therapy might be performed. In this review we aimed to update information about frequently seen pediatric gastrointaestinal diseases and genetics reasons of these diseases which are heterogenous and multifactorial. By this way, some informations were given for planning some gene panels which can be beneficial for diagnosis, and useful notions were emphasized for genetic counselling. [ABSTRACT FROM AUTHOR]

Objectives: We aimed to evaluate the frequency of extraintestinal manifestations (EIM) in children with inflammatory bowel disease (IBD) and the correlation of EIM with disease activity index, disease type and disease age., Methods: Records of patients who were under the 18 years of age and followed up with the diagnosis of IBD were included in the study. The demographic characteristics of the patients, the age of the patients, the sex of the patients, the type of disease and the age of the disease were recorded. When patients were enrolled in the study disease activity indexes were calculated by examining the physical examination and laboratory values of the patients in their last visits, by using the Pediatric Ulcerative Colitis Activity Index (PUCAI) in Ulcerative Colitis (UC) and the Pediatric Crohn's Disease Activity Index (PCDAI) in Crohn's Disease (CD)., Results: It was conducted with a total of 44 patients, 40.9% (n=18) females and 59.1% (n=26) males diagnosed with IBD. The ages of the patients participating in the study ranged from 8 to 19 and the mean was found to be 14.64±3.19 years. According to the type of disease; 27.3% of the patients were CD and 72.7% were UC. When the disease activity is examined; 37.2% were in remission, 37.2% were mild, 16.3% moderate and 9.3% severe. The incidence of EIM in females is 77.8% and 65.4% in males. The incidence of EIM is 75% in CD and 68.8% in UC. Hepatobiliary involvement was detected in 41.5% (n=17) of the patients, joint involvement in 29.5% (n=13), osteopenia in bone in 16.3% (n=7), osteoporosis in 7% (n=3), ocular involvement (uveitis) in 2.3% (n=1) and skin involvement (erythema nodosum) in 2.3% (n=1)., Conclusion: EIM are common in childhood IBD patients. It should be kept in mind that EIM are as common in UC as CD., Competing Interests: None declared., (©Copyright 2023 by The Medical Bulletin of Sisli Etfal Hospital.)

Approximately half of the children with ulcerative colitis (UC) have refractory, relapsing or steroid-dependent disease. UC in children is more extensive than in adults, presents more often with severe attacks and carries a more aggressive disease course. Therefore, although a step-up approach is usually recommended in UC, aggressive therapy will often be indicated in children since steroid dependency should never be tolerated. It is vital to ensure that in every resistant case, the symptoms are truly related to the inflammatory disease activity and not to other conditions such as poor adherence to treatment, infections, adverse reactions to drugs, irritable bowel syndrome, lactose intolerance, celiac disease and bacterial overgrowth. The clinician should be ready to escalate therapy in a timely manner but only after ensuring optimization of current treatments. Optimization may include, among others, appropriate dosage, utilization of assays that determine thiopurine, calcineurin inhibitors and anti-tumor necrosis factor levels, introduction of combination therapy when indicated (enemas and immunomodulators) and a long enough time for treatment to become effective. Colectomy is always a valid option and should be discussed before major treatment escalations. Experimental therapies can be considered when all else fails and the family prefers to avoid colectomy. The management of refractory and relapsing disease is particularly challenging in children, and this review summarizes the available evidence to guide treatment decisions in this setup. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]