scholarly journals COLORECTAL ADENOCARCINOMA DEVELOPING IN A YOUNG FEMALE WITH OVERLAP SYNDROME AND VERY EARLY ONSET-INFLAMMATORY BOWEL DISEASE: A CASE REPORT

2021 ◽  
Vol 27 (Supplement_1) ◽  
pp. S14-S15
Author(s):  
Prashanthi Kandavel ◽  
Victoria Shakhin ◽  
Mallory Chavannes ◽  
Christopher Gayer ◽  
Hillel Naon ◽  
...  
Keyword(s):  
Ulcerative Colitis ◽  
Inflammatory Bowel Disease ◽  
Bowel Disease ◽  
Early Onset ◽  
Colorectal Adenocarcinoma ◽  
Clinical Course ◽  
Overlap Syndrome ◽  
High Risk Population ◽  
Endoscopic Screening ◽  
Inflammatory Bowel

Abstract We present a 4-year-old female diagnosed with very-early onset inflammatory bowel disease (VEO-IBD), ulcerative colitis type, and overlap syndrome based on clinical presentation, blood and stool studies, diagnostic endoscopies and liver biopsy. Her clinical course was complicated by pervasive psychological stressors, non-compliance with medical therapy, and frequent provider changes. Initial treatment included Prednisone, 6-mercaptopurine and Ursodiol. Treatment was escalated to include multiple biological agents which were stopped prematurely due to non-adherence. She developed weight loss and growth stunting, necessitating exclusive enteral nutrition and then parenteral nutrition. Due to medically refractory disease, she underwent a total colectomy with end ileostomy at the age of 13. Pathology revealed stage III colorectal adenocarcinoma (CRC) with lymph node involvement and transmural inflammation diagnostic of Crohn’s disease (CD). Genetic testing of the resected colon noted a missense mutation of TP53. Our patient completed 6 cycles of extensive and targeted chemotherapy with no evidence of disease reccurrence. She recently underwent ileal pouch-anal anastomosis and creation of a diverting ileostomy. CRC is exceedingly rare in pediatric IBD (PIBD) patients, with less than 30 reported cases in the available literature1. Most cases occurred in males and patients with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC), where the risk of developing CRC is four times greater2. The risk of CRC increases with disease duration, with most PIBD patients developing CRC at 25 years of age1. Interestingly, mutations in TP53, a tumour suppressor gene, have been found to occur more frequently in patients with IBD-CRC compared to those with sporadic-CRC or IBD without dysplasia3. Mutations in TP53 may not only increase a patients general susceptibility to CRC but may also accelerate its development by contributing to excessive inflammation4. Our patient’s treatment refractory clinical course and transition in IBD diagnosis from UC to CD highlights the complexity of VEO-IBD management. While rare in pediatric patients, the development of CRC is a life-threatening risk and it should be considered in patients with prolonged and severely refractory IBD with concomitant autoimmune liver disease. Our patient’s young age at the time of CRC diagnosis emphasizes the need for vigilant yearly endoscopic screening and development of pediatric guidelines for dysplasia monitoring in this high risk population. Further research is needed to evaluate the role of genetic variant screening in CRC risk stratification. References: 1. Aardoom MA, et al. Inflamm Bowel Dis. 2018;24(4):732–741. 2. Soetikno RM, et al. Gastrointest Endosc. 2002;56(1):48–54. 3. Yaeger R, et al. Gastroenterology. 2016;151(2):278–287. 4. Cooks T, et al. Cancer Cell. 2013;23(5):634–46.

COLORECTAL ADENOCARCINOMA DEVELOPING IN A YOUNG FEMALE WITH OVERLAP SYNDROME AND VERY EARLY ONSET-INFLAMMATORY BOWEL DISEASE: A CASE REPORT

2021 ◽  
Vol 160 (3) ◽  
pp. S20
Author(s):  
Prashanthi Kandavel ◽  
Victoria Shakhin ◽  
Mallory Chavannes ◽  
Christopher Gayer ◽  
Hillel Naon ◽  
...  
Keyword(s):  
Inflammatory Bowel Disease ◽  
Case Report ◽  
Bowel Disease ◽  
Early Onset ◽  
Colorectal Adenocarcinoma ◽  
Young Female ◽  
Overlap Syndrome ◽  
Inflammatory Bowel

scholarly journals Natural History of  Very Early Onset Inflammatory Bowel Disease in North America: A Retrospective Cohort Study

2020 ◽  
Author(s):  
Basavaraj Kerur ◽  
Eric I Benchimol ◽  
Karoline Fiedler ◽  
Marisa Stahl ◽  
Jeffrey Hyams ◽  
...  
Keyword(s):  
Ulcerative Colitis ◽  
Inflammatory Bowel Disease ◽  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Bowel Disease ◽  
Early Onset ◽  
Bowel Surgery ◽  
Age Of Diagnosis ◽  
History Of ◽  
Inflammatory Bowel

Abstract Background The incidence of very early onset inflammatory bowel disease (VEOIBD) is increasing, yet the phenotype and natural history of VEOIBD are not well described. Methods We performed a retrospective cohort study of patients diagnosed with VEOIBD (6 years of age and younger) between 2008 and 2013 at 25 North American centers. Eligible patients at each center were randomly selected for chart review. We abstracted data at diagnosis and at 1, 3, and 5 years after diagnosis. We compared the clinical features and outcomes with VEOIBD diagnosed younger than 3 years of age with children diagnosed with VEOIBD at age 3 to 6 years. Results The study population included 269 children (105 [39%] Crohn’s disease, 106 [39%] ulcerative colitis, and 58 [22%] IBD unclassified). The median age of diagnosis was 4.2 years (interquartile range 2.9–5.2). Most (94%) Crohn’s disease patients had inflammatory disease behavior (B1). Isolated colitis (L2) was the most common disease location (70% of children diagnosed younger than 3 years vs 43% of children diagnosed 3 years and older; P = 0.10). By the end of follow-up, stricturing/penetrating occurred in 7 (6.6%) children. The risk of any bowel surgery in Crohn’s disease was 3% by 1 year, 12% by 3 years, and 15% by 5 years and did not differ by age at diagnosis. Most ulcerative colitis patients had pancolitis (57% of children diagnosed younger than 3 years vs 45% of children diagnosed 3 years and older; P = 0.18). The risk of colectomy in ulcerative colitis/IBD unclassified was 0% by 1 year, 3% by 3 years, and 14% by 5 years and did not differ by age of diagnosis. Conclusions Very early onset inflammatory bowel disease has a distinct phenotype with predominantly colonic involvement and infrequent stricturing/penetrating disease. The cumulative risk of bowel surgery in children with VEOIBD was approximately 14%–15% by 5 years. These data can be used to provide anticipatory guidance in this emerging patient population.

Features of very early-onset inflammatory bowel disease: the experience of the Federal Pediatric Center

2021 ◽  
Vol 19 (3) ◽  
pp. 5-13
Author(s):  
P.V. Shumilov ◽  
◽  
A.E. Shchigoleva ◽  
Keyword(s):  
Ulcerative Colitis ◽  
Inflammatory Bowel Disease ◽  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Primary Immunodeficiency ◽  
Bowel Disease ◽  
Early Onset ◽  
Activity Index ◽  
Response To Therapy ◽  
Inflammatory Bowel

Objective. To clarify the incidence of monogenic IBD-like diseases and the features of clinical course and response to therapy of major types of inflammatory bowel diseases (IBD) among children under the age of 6 with manifestation of the disease. Patients and methods. The study included 135 children under the age of 6 with manifestation of IBD; in the comparison group, there were 128 children after the age of 6 with manifestation of IBD (97 children with ulcerative colitis (UC) and 31 children with Crohn’s disease (CD)) who were observed for at least 1 year. All children underwent a standard examination, including calprotectin and antineutrophil antibodies testing, determination of activity by the Pediatric Ulcerative Colitis Activity Index (PUCAI) or the Pediatric Crohn’s Disease Activity Index (PCDAI), depending on the nosology. Children with the onset of IBD under 6 years of age underwent a genetic testing using Primary Immunodeficiency Panel by next-generation sequencing. All children were analyzed for efficacy of therapy during catamnestic observation. Results. It was revealed that in the study group the incidence of monogenic IBD-like diseases was 6.7%, of UC – 71.1%, of CD – 22.2%. Major types of IBD with very early onset differed little in their clinical, endoscopic and laboratory features from the forms with manifestation at an older age. In most cases, both CD (57%) and UC (71%) were characterized by low activity. Very earlyonset CD was characterized by isolated localization of the colon (53%, p = 0.037) and a non-stenotic and non-penetrating behaviour of the disease (60% of cases). The leading clinical symptoms were diarrhea (67%) and blood in the stool (63%, p = 0.04). Very early-onset UC was characterized by total lesion of the colon (84%, p = 0.001) and the development of anemia (48%, p = 0.01). Among children with very early-onset UC, the percentage of glucocorticosteroid-dependence and glucocorticosteroid-resistance was high, but anti-TNFα therapy was prescribed late. Conclusion. It is advisable to observe children with very early-onset IBD in federal multidisciplinary clinics, where there is experience in managing patients with this pathology. Key words: inflammatory bowel disease, very early onset, Crohn’s disease, ulcerative colitis, primary immunodeficiency, treatment, children

Ulcerative colitis

2011 ◽  
Author(s):  
R. Mark Beattie ◽  
Anil Dhawan ◽  
John W.L. Puntis
Keyword(s):  
Ulcerative Colitis ◽  
Inflammatory Bowel Disease ◽  
Family History ◽  
Bowel Disease ◽  
Clinical Course ◽  
Younger Child ◽  
Clinical Presentations ◽  
History Of ◽  
Inflammatory Bowel ◽  
Index Cases

Clinical presentations 310Investigation 311Clinical course 311Management 31225% of inflammatory bowel disease presents in childhood, 1/3 as ulcerative colitis. Presentation can occur at any age and ulcerative colitis is the commonest cause of inflammatory bowel disease in the younger child. Family history of Crohn's disease or ulcerative colitis is common in index cases....

scholarly journals Lifestyle Issues in Inflammatory Bowel Disease – Smoking

1994 ◽  
Vol 8 (7) ◽  
pp. 422-427 ◽  
Author(s):  
Cecilia Benoni
Keyword(s):  
Ulcerative Colitis ◽  
Inflammatory Bowel Disease ◽  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Protective Effect ◽  
Bowel Disease ◽  
Clinical Course ◽  
Clinical Effects ◽  
Increased Risk ◽  
Inflammatory Bowel

During the pa t decade, smoking habit has been identified as a major exogenous factor in inflammatory bowel disease (IBD). It is associated not only with the development of the disease but al o with the clinical course in established disease. IBD combines absolute opposites as smoking is associated with Crohn’s disease and nonsmoking or former smoking with ulcerative colitis. The first reports of a negative association between smoking and ulcerative colitis were based on independent, clinical observations; from those studies a positive association was found between smoking and Crohn’s disease. Epidemiological studies that followed consistently showed that smokers have a reduced risk of ulcerative colitis and an increased risk of Crohn’s disease and that exsmokers have an increased risk of ulcerative colitis. In ulcerative colitis, but not in Crohn’s disease, a dose-response pattern has been demonstrated. Changes in clinical course, in disease severity and extension, and in recurrence rate indicate substantial clinical effects of smoking with a protective effect of smoking in ulcerative colitis and an aggravating effect in Crohn’s disease. There are also indications of smoking’s effects on changes in IBD epidemiology and sex distribution. The biological explanation to the finding is unknown. Smoking may aggravate Crohn’s disease by vascular effects. Theories on the protective effect in ulcerative colitis include effects on immune and inflammatory response, on mucus and on intestinal permeability. Possibly, beneficial effects in ulcerative colitis are exerted by nicotine but further studies are needed. Due to overall negative effects of smoking, IBD patients should not smoke. It seems, however, reasonable to give individual advice in patients with ulcerative colitis who have experienced a beneficial effect of ·making considering both current health status and life situation.

scholarly journals Liver Transplantation, IBD Clinical Course, Is There a Link? A Systematic Review and Meta-Analysis Protocol

2020 ◽  
Author(s):  
GholamReza Sivandzadeh ◽  
Manoosh Mehrabi ◽  
Ali Reza Safarpour ◽  
Hadis Ashrafizadeh ◽  
Abbas Ali Keshtkar
Keyword(s):  
Systematic Review ◽  
Ulcerative Colitis ◽  
Inflammatory Bowel Disease ◽  
Liver Transplantation ◽  
Publication Bias ◽  
Bowel Disease ◽  
Clinical Course ◽  
Meta Analysis ◽  
Effect Model ◽  
Inflammatory Bowel

Abstract BackgroundPrimary sclerosing cholangitis (PSC) is an uncommon chronic and progressive cholestatic liver disease. There is a robust association between PSC and Inflammatory Bowel Disease (IBD), usually Ulcerative Colitis (UC). According to the review of literature, the incidence of de novo IBD after solid organ transplantation (SOT) is found to be higher than general population. Considering lacking of any systematic review and pursuing debate on the clinical course and risk factors of IBD activity after Liver Transplantation (LT), the present study will be performed with a focus investigation on the correlation of IBD clinical course with liver transplantation. MethodsIn this systematic review, the electronic databases including PubMed/MEDLINE, Scopus, WoS (Clarivate Analytics), Embase (Embase.com), and ProQuest will be searched. Our search strategy (i.e. The eligibility criteria) covers prospective and retrospective observational studies that evaluated the clinical course of ulcerative colitis or/and Crohn’s disease after liver transplantation with no language limitation published between 1970.01.01 and 2020.03.30. The selection phase, data extraction and quality assessment will be independently implemented by two authors. In case of any disagreement between the authors, the issue will be resolved by consensus; if not resolved, the opinion of a third expert will be asked. We will use one of the following two models: Random Effect Model or Fixed Effect Model according to the severity of methodological heterogeneity and forest plot will present the combination of data obtained from all finally included studies, to show the separated and combined frequency and their corresponding 95% CIs. Statistical heterogeneity will be evaluated by the Q-statistic test and I2 statistics. Funnel plot for assessing the potential reporting bias, Begg's and Egger's tests for meaningful results of the publication bias, and the Fill & Trim method for corrected publication bias will be used. DiscussionThis systematic review and meta-analysis study will clarify the correlation of IBD clinical course with liver transplantation. Because of the importance of inflammatory bowel disease, if the future study reveals consistent results, it will be clinically beneficial for physicians and other health care professionals to better manage inflammatory bowel disease after liver transplantation.Systematic review registrationPROSPERO, CRD42020179412.

scholarly journals Very Early-Onset Inflammatory Bowel Disease (VEO-IBD) Presenting with Recurrent Leukocytoclastic Vasculitis Preceded by Streptococcal Pharyngitis

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Ashley Fonseca ◽  
Julee Sunny ◽  
Lina M. Felipez
Keyword(s):  
Ulcerative Colitis ◽  
Inflammatory Bowel Disease ◽  
Clostridium Difficile ◽  
Bowel Disease ◽  
Early Onset ◽  
Leukocytoclastic Vasculitis ◽  
Streptococcal Pharyngitis ◽  
Clostridium Difficile Toxin ◽  
Inflammatory Bowel ◽  
Multiple Episodes

Inflammatory bowel disease (IBD) that presents in children <6 years of age is known as very early-onset IBD (VEO-IBD). Extraintestinal manifestations in IBD, such as erythema nodosum (EN), pyoderma gangrenosum (PG), and, less likely, leukocytoclastic vasculitis (LV), are more commonly present in Crohn’s disease. Association between LV and ulcerative colitis (UC) is not commonly seen. We report a case of a 6-year-old female with a VEO-IBD UC phenotype presenting with multiple episodes of leukocytoclastic vasculitis, each preceded by streptococcal pharyngitis. Prior to the diagnosis of VEO-IBD, a skin biopsy was obtained and had shown leukocytoclastic vasculitis with a negative IgA stain. Initial laboratory results were remarkable for leukocytosis and increased anti-strep O and anti-DNase B titers. Gastrointestinal panel PCR demonstrated Clostridium difficile toxin A/B. Treatment for LV consisted of methylprednisolone IV 20 mg for four days with a weaning schedule of prednisolone for two weeks and naproxen 250 mg BID for three days. Clostridium difficile was treated with metronidazole 250 mg TID for ten days. She remained stable for three years until she presented with continuous bloody stools, newly onset chest pain, and shortness of breath. Computed tomography angiogram (CTA) was normal. Stool calprotectin was elevated at 658 mcg/gm. Abdominal magnetic resonance enterography (MRE), esophagogastroduodenoscopy, and colonoscopy confirmed a VEO-IBD ulcerative colitis phenotype. She was started on infliximab 10 mg/kg every four weeks after infliximab titers, and antibodies were obtained. Currently, the patient remains on clinical and biochemical remission, with no recent LV episodes or recurrence of streptococcal pharyngitis. Our patient is unique as no case report has been published with multiple episodes of leukocytoclastic vasculitis in association with a VEO-IBD UC phenotype.

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