Characteristics of inflammatory bowel disease with an onset before eight years of age: A multicenter epidemiological survey in <scp>J</scp>apan

Maisawa, Shunichi; Sasaki, Makoto; Ida, Shinobu; Uchida, Keiichi; Kagimoto, Seiichi; Shimizu, Toshiaki; Yoden, Atsushi

doi:10.1111/jgh.12071

Cited by 25 publications

(39 citation statements)

References 26 publications

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“…Among children with very early onset Crohn's disease, there is a marked preponderance of cases with isolated colonic disease, for example, 76.5% before age 5103 and 42% before age 8 104. Among younger cases, there is a strong male preponderance, for example, 1.6:1 across all Crohn's disease presenting <5103 and some of this is accounted for by X linked single gene disorders.…”

Section: Isolated Colonic Crohn's Disease In Childhood and Single Genmentioning

confidence: 99%

Recent advances in clinical practice: a systematic review of isolated colonic Crohn's disease: the third IBD?

Subramanian

Ekbom

Rhodes

2016

Gut

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Section: Isolated Colonic Crohn's Disease In Childhood and Single Genmentioning

confidence: 99%

Recent advances in clinical practice: a systematic review of isolated colonic Crohn's disease: the third IBD?

Subramanian

Ekbom

Rhodes

2016

Gut

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“…A large proportion of patients with very early-or early-onset IBD (symptoms before 10 yr of age 13 ) remain molecularly unclassified. 14,15 Early detection of such diseases and identification of the underlying causative genetic aberration(s) may improve the treatment strategies and enable further understanding of the pathogenic mechanisms underlying IBD.…”

mentioning

confidence: 99%

Atypical Manifestation of LRBA Deficiency with Predominant IBD-like Phenotype

Serwas

Kansu

Santos-Valente

et al. 2015

Inflammatory Bowel Diseases

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“…1,2 Up to 25% of patients first present during childhood or adolescence, 3 with early-onset (EO) (0-5 yr) and very early-onset (VEO) diseases (0-2 yr) increasingly reported. [4][5][6][7] Although data on EO forms are scattered, they seem to be characterized by a unique phenotype, extensive and aggressive, which affect the management of this age group. 5 Moreover, prior reports suggest that EO-IBD differ epidemiologically and are distinguished by a predominant colonic involvement and a greater impact of genetic predisposition, compared with older age of onset.…”

mentioning

confidence: 99%

Phenotype and Disease Course of Early-onset Pediatric Inflammatory Bowel Disease

Aloi

Lionetti

Barabino

et al. 2014

Inflammatory Bowel Diseases

127

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BACKGROUND:\ud Early-onset (EO) pediatric inflammatory bowel diseases (IBD) seem to be more extensive than those with a later onset. To test this hypothesis, we examined the phenotype and disease course of patients with IBD diagnosis at 0 to 5 years, compared with the ranges 6 to 11 and 12 to 18 years.\ud METHODS:\ud Anatomic locations and behaviors were assessed according to Paris classification in 506 consecutive patients: 224 Crohn's disease, 245 ulcerative colitis, and 37 IBD-unclassified.\ud RESULTS:\ud Eleven percent of patients were in the range 0 to 5 years, 39% in 6 to 11 years, and 50% in 12 to 18 years. Ulcerative colitis was the most frequent diagnosis in EO-IBD and in 6- to 11-year-old group, whereas Crohn's disease was predominant in older children. A classification as IBD-unclassified was more common in the range 0 to 5 years compared with the other groups (P < 0.005). EO Crohn's disease showed a more frequent isolated colonic (P < 0.005) and upper gastrointestinal involvement than later-onset disease. Sixty-two percent of the patients in the 0 to 5 years range had pancolonic ulcerative colitis, compared with 38% of 6 to 11 years (P = 0.02) and 31% of 12-18 years (P = 0.002) range. No statistical difference for family history for IBD was found in the 3-year age groups. Therapies at the diagnosis were similar for all children. However, at latest follow-up, a significantly higher proportion of younger children were under steroids compared with older groups (P < 0.05). Surgical risk did not differ according to age.\ud CONCLUSIONS:\ud EO-IBD exhibits an extensive phenotype and benefit from aggressive treatment strategies, although surgical risk is similar to later-onset disease. A family history for IBD is not common in EO disease

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Characteristics of inflammatory bowel disease with an onset before eight years of age: A multicenter epidemiological survey in Japan

Abstract: Our results suggest that, in Japan, the pathogenesis of IBD in infants and children may differ from that in Western countries, and that the characteristics of early childhood-onset IBD are distinct from those of school age-onset IBD.

Cited by 25 publications

References 26 publications

Recent advances in clinical practice: a systematic review of isolated colonic Crohn's disease: the third IBD?

Recent advances in clinical practice: a systematic review of isolated colonic Crohn's disease: the third IBD?

Atypical Manifestation of LRBA Deficiency with Predominant IBD-like Phenotype

Phenotype and Disease Course of Early-onset Pediatric Inflammatory Bowel Disease

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