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Introduction
Crohn's disease (CD) is a chronic inflammatory disorder, which can affect any part of the intestinal tract as well as extraintestinal tissue. Factors that contribute towards the pathogenesis of the disease are the host's genetic profile, immune system and environmental factors such as the gut microbiota. 1
CD occurs in all ages with recently reported paediatric figures from Scotland and South England suggesting an incidence of 4.75 and 5.85/100 000 people, respectively. 2 3 UK prevalence data are limited, but suggest a figure of 32 per 100 000 people. 4 Due to yet undefined environmental factors, the incidence and prevalence of CD is rising, in both adult and paediatric studies. 5 6
In this review, we aimed to update important new developments in the management of CD for paediatricians in the UK highlighting the key publications and guidelines published recently. 7 We also recognise that the BSPGHAN inflammatory bowel disease (IBD) guidelines published previously, now contain many recommendations that needed updated guidance, but without generation of a specifically new guideline. 8 9
Diagnosis
Key clinical symptoms of CD comprise diarrhoea, abdominal pain, growth failure and rectal bleeding with the first three being the most common in patients first presenting with CD. 10 CD often has an insidious onset, which may contribute to considerable diagnostic delay. CD can affect the entire intestinal tract, and transmural inflammation can lead to stricture formation and fistulisation between the gut and other abdominal organs as well as the skin. 11 Perianal inspection is imperative as significant perianal involvement (eg, inflamed fissures or skin tags, abscesses and fistulae) are seen at presentation in at least 15% of children with CD 12 (see figure 1 ). Extraintestinal manifestations in IBD can affect the skin, eyes, musculoskeletal and hepatobiliary systems. 13
Once IBD is suspected, patients should be fast tracked to specialist services (for relevant blood and stool tests see Fell et al 13a and figure 2 ). The revised Porto criteria provide detailed guidance on the diagnostic evaluation of paediatric patients with IBD 10 emphasising the necessity to perform upper gastrointestinal endoscopy and ileocolonoscopy with histology as well as small bowel imaging (see figure 1). 14
Once CD has been established, ongoing disease activity is monitored by clinical review and assessment....