Crohnʼs Disease in Children 10 Years Old and Younger

Inflammatory bowel disease (IBD) is uncommon in children younger than 2 years of age. The criteria for differentiating IBD from other diseases with similar clinical presentation is unclear. We describe 16 patients who, between 1984 and 2004, received a histological diagnosis of IBD during the first two years of life. Six patients presented with histological Crohn's disease, eight with ulcerative colitis and two with indeterminate colitis. The median age at diagnosis was 125 days (range 1 day to 18 months) and the medium follow up was 89 months (range 65 days to 20 years). The disease appeared to be very severe: four children (25%) underwent total parenteral nutrition (TPN), two received colectomy (12.5%) and three patients died. Many of the patients required an aggressive, multidrug, immunosuppressive approach (azathioprine [AZA], Infliximab, thalidomide, cyclosporine A). One child presented with a hypogammaglobulinaemia without any specific immunodeficiency, while in the other patients, Wiskott-Aldrich syndrome (WAS) (4 cases) and chronic granulomatous disease (CGD) (2 cases) were identified. In 6/16 cases, allergic colitis was first considered; these cases initially underwent cow's milk protein-free diet as the only therapy before IBD was finally diagnosed. In conclusion, early IBD has a severe prognosis and often needs an aggressive therapeutic approach. Furthermore, an improper diagnosis of allergic colitis might cause an important diagnostic delay. Some severe immunodeficiencies, such as WAS and CGD, may represent a problem in terms of differential diagnosis and might be wrongly diagnosed as very early onset IBD.

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“…This is in accordance with several papers [8,9,14,23], even if some series report much higher rates [6,15,16,19].…”

Section: Discussionsupporting

confidence: 93%

IBD and IBD mimicking enterocolitis in children younger than 2 years of age

et al. 2008

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“…While two GWAS focused on pediatric IBD (3, 4), they included only a small sample size with age less than 10 years old at disease onset. Clinical heterogeneity within IBD, particularly based on VEO has long been known (5–7), and recent evidence suggests that this heterogeneity is explained by different mechanism-based disease subsets (8). Growing evidence now supports that these phenotypic differences seen among the childhood onset IBD reflect variations in genetics, composition of the microbiome and perhaps environmental risk factors (10, 11).…”

Section: Introductionmentioning

confidence: 99%

Role of Genetics in Pediatric Inflammatory Bowel Disease

Okou¹,

Kugathasan²

2014

Inflammatory Bowel Diseases

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Inflammatory bowel disease (IBD), encompassing Crohn’s disease (CD), ulcerative colitis (UC) and unclassified IBD (IBDU), is characterized by chronic intestinal inflammation, and has a multi-factorial etiology with complex interactions between genetic and environmental factors. The genetics of IBD are believed to be common and complex with over 163 associated genetic loci. However, the genetic contribution of the majority of these common loci is small and the effect sizes are low. Although childhood onset IBD represents only 10–25% of all IBD cases, in depth research into the genetic networks of pediatric IBD has revealed exciting new developments and unsuspected pathways. Recent pediatric studies have revealed an increasing spectrum of human monogenic diseases with high effect sizes/penetrance that can present with IBD or IBD-like intestinal inflammation. A substantial proportion of patients with these genetic defects present with very early onset of intestinal inflammation, with onset of IBD at less than 10 years of age. There is also considerable overlap with primary immunodeficiencies and very early onset IBD. This review summarizes the current understanding of the genetics of pediatric inflammatory bowel disease with a focus on the very early onset population, and discusses the promising results from the effort of finding missing heritability of IBD from studying pediatric population.

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“…Several studies have shown catch-up growth in children following intestinal resection [49,50]. In fact, 36% to 43% of paediatric patients will require surgery [51,52]. Preliminary data from the EPIMAD registry (population-based incidence cohort diagnosed from 1988-2002) demonstrated that the cumulative risk of surgery in children up to age 17 years with CD was 8%, 20% and 34% at 1, 3 and 5 years, respectively, post diagnosis [53].…”

Section: Treatment Options In Paediatric CDmentioning

confidence: 99%

The Management of Paediatric Crohn's Disease: Addressing Unmet Needs

Veereman-Wauters¹,

Cucchiara²

2008

TOPEDJ

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Paediatric Crohn's disease (CD) affects 5 in 100,000 children in the US and Europe and can result in growth retardation and delayed sexual development. Therefore, early diagnosis and treatment is critical, with the goal being maintenance of symptomatic remission and a change in disease course. Conventional treatment relies on aminosalicylate maintenance therapy with corticosteroids to control acute exacerbations and immunomodulators for steroid-resistant or frequently relapsing disease. Infliximab has demonstrated efficacy in moderately to severely active paediatric CD, with 88.4% patients in clinical response and 58.9% in clinical remission at week 10. Significant improvements in quality of life, height, reduction in corticosteroid use and mucosal healing were observed with infliximab. Traditional 'step-up' treatment strategy may be suboptimal because relapse and steroid dependency/resistance rates remain high. A 'top down' approach using biologic therapy earlier may suppress intestinal inflammation and promote prolonged and stable remission, but safety issues need to be considered.

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Crohnʼs Disease in Children 10 Years Old and Younger

Cited by 91 publications

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IBD and IBD mimicking enterocolitis in children younger than 2 years of age

IBD and IBD mimicking enterocolitis in children younger than 2 years of age

Role of Genetics in Pediatric Inflammatory Bowel Disease

The Management of Paediatric Crohn's Disease: Addressing Unmet Needs

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