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Background: Inflammatory bowel disease (IBD) is a chronic relapsing and remitting condition. The COVID-19 pandemic has severely disrupted provision of medical care across the world. IBD clinical nurse specialists (CNSs) played a pivotal role in the care of children with IBD during the pandemic national lockdown and in the recovery phase. This article aims to look at the impact of COVID-19 on the paediatric IBD service in one children's hospital and the effect on the IBD CNSs' workload. Method: A retrospective review of clinical notes and the service's IBD database from January 2019 to September 2020. Results: There was a significant increase in the number of email and telephone contacts to the IBD CNS team during lockdown. There was an increase in virtual clinics, and an increase in new IBD patients coming to the service, but a reduction in the number of face-to-face consultant clinics. Conclusion: COVID-19 has disrupted medical services to children with IBD and led to a reduction in face-to-face activities but has also led to a significant increase in virtual activities. CNSs have taken up a wider role to cover patient care during a time of both medical and nursing redeployment. [ABSTRACT FROM AUTHOR]

Chronic intestinal pseudo-obstruction is a rare disorder that affects the motility of the gastrointestinal tract. It results in acute or subacute ntestinal obstruction symptoms in the absence of mechanical lesion. It can ead to intestinal failure in children with significant strain on nutrition, growth, and development. There is no universally agreed protocol for management of chronic intestinal pseudo-obstruction in children, and there is wide variation in clinical practice. [ABSTRACT FROM AUTHOR]

Patients with a diverse spectrum of rare genetic disorders can present with inflammatory bowel disease (monogenic IBD). Patients with these disorders often develop symptoms during infancy or early childhood, along with endoscopic or histological features of Crohn’s disease, ulcerative colitis, or IBD unclassified. Defects in interleukin-10 signaling have a Mendelian inheritance pattern with complete penetrance of intestinal inflammation. Several genetic defects that disturb intestinal epithelial barrier function or affect innate and adaptive immune function have incomplete penetrance of the IBD-like phenotype. Several of these monogenic conditions do not respond to conventional therapy and are associated with high morbidity and mortality. Due to the broad spectrum of these extremely rare diseases, a correct diagnosis is frequently a challenge and often delayed. In many cases, these diseases cannot be categorized based on standard histological and immunologic features of IBD. Genetic analysis is required to identify the cause of the disorder and offer the patient appropriate treatment options, which include medical therapy, surgery, or allogeneic hematopoietic stem cell transplantation. In addition, diagnosis based on genetic analysis can lead to genetic counseling for family members of patients. We describe key intestinal, extraintestinal, and laboratory features of 50 genetic variants associated with IBD-like intestinal inflammation. In addition, we provide approaches for identifying patients likely to have these disorders. We also discuss classic approaches to identify these variants in patients, starting with phenotypic and functional assessments that lead to analysis of candidate genes. As a complementary approach, we discuss parallel genetic screening using next-generation sequencing followed by functional confirmation of genetic defects. [ABSTRACT FROM AUTHOR]