Distinct Phenotype of Early Childhood Inflammatory Bowel Disease

Paul, Thankam; Birnbaum, Adrienne; Pal, Deb K.; Pittman, Nanci; Ceballos, Clare; LeLeiko, Neal S.; Benkov, Keith

doi:10.1097/00004836-200608000-00004

Cited by 85 publications

(78 citation statements)

References 29 publications

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“…We have shown that involvement of the ileum is age dependent: children Ͻ8 years old at the time of diagnosis had significantly less involvement of the ileum and more isolated colonic disease than children Ͼ8 years at diagnosis, confirming previous studies. 7,8,25 In a large study of nearly 1400 North American early-onset patients, Heyman et al 7 demonstrated by multifactorial analysis that a colonic predominant phenotype exists in IBD diagnosed under the age of 8 years. Paul et al 8 studied 413 pediatric IBD patients and also demonstrated a greater tendency for very young patients to present with colonic disease.…”

Section: Discussionmentioning

confidence: 99%

“…This hypothesis has been supported to some extent by the limited available data suggesting that childhood-onset CD may be characterized by extensive intestinal involvement at presentation. [7][8][9] However, rigorous studies investigating the progression of intestinal involvement and behavior in childhood disease are not available to help explore this hypothesis further.…”

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confidence: 99%

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Definition of Phenotypic Characteristics of Childhood-Onset Inflammatory Bowel Disease

et al. 2008

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See Dubinsky MC et al on page 1105 in CGH;See editorial on page 1038. Background & Aims:Childhood-onset inflammatory bowel disease (IBD) might be etiologically different from adult-onset IBD. We analyzed disease phenotypes and progression of childhood-onset disease and compared them with characteristics of adult-onset disease in patients in Scotland. ; 43% vs 3%; P < .0001; OR, 23.36; 95% CI, 13.45-40.59) with less isolated ileal (L1; 2% vs 31%; P < .0001; OR, 0.06; 95% CI, 0.03-0.12) or colonic disease (L2; 15% vs 36%; P < .0001; OR, 0.31; 95% CI, 0.21-0.46). UC was extensive in 82% of the children at diagnosis, versus 48% of adults (P < .0001; OR, 5.08; 95% CI, 2.73-9.45); 46% of the children progressed to develop extensive colitis during followup. Forty-six percent of children with CD and 35% with UC required immunomodulatory therapy within 12 months of diagnosis. The median time to first surgery was longer in childhood-onset than adult-onset patients with CD (13.7 vs 7.8 years; P < .001); the reverse was true

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Definition of Phenotypic Characteristics of Childhood-Onset Inflammatory Bowel Disease

et al. 2008

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“…However, CD represented 60% of IBD cases in our VEO-IBD group, unlike previous studies that reported a predominance of UC over CD in VEO-IBD. 14,15,29,32 This could have arisen from the diagnostic criteria used, as well as from specific environmental factors and lifestyle in the study area. 33,34 Complete bowel investigations were obtained as often in the VEO-IBD group as in the EO-IBD group and the diagnostic criteria [definite or probable IBD cases] did not change over time.…”

Section: Discussionmentioning

confidence: 99%

Incidence and Phenotype at Diagnosis of Very-early-onset Compared with Later-onset Paediatric Inflammatory Bowel Disease: A Population-based Study [1988–2011]

Bequet¹,

Sarter²,

Fuméry³

et al. 2016

ECCOJC

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Background and Aims: Very-early-onset inflammatory bowel disease [VEO-IBD] is a form of IBD that is distinct from that of children with an older onset. We compared changes over time in the incidence and phenotype at diagnosis between two groups according to age at IBD diagnosis: VEO-IBD diagnosed before the age of 6 years, and early-onset IBD [EO-IBD] ]. Rectal bleeding and mucous stools were more common in patients with VEO-IBD, whereas weight loss and abdominal pain were more frequent in those with EO-IBD. Regarding CD, isolated colonic disease was more common in the VEO-IBD group [39% vs 14%; p = 0.003]. Conclusions:In this large population-based cohort, the incidence of VEO-IBD was low and stable from 1988 to 2011, with a specific clinical presentation. These results suggest a probable genetic origin for VEO-IBD, whereas the increase in EO-IBD might be linked to environmental factors.

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“…For CD, an earlier age of onset and a higher frequency of exclusively colonic disease was shown in familial cases [41][42][43][44][45]. The familial form of AS is associated with a milder disease phenotype [46], and high heritability of disease severity was shown [47,48].…”

Section: Familial Clustering Of Spondyloarthro-pathy and Inflammatorymentioning

confidence: 99%

Genetics of Spondyloarthropathies and Inflammatory Bowel Disease: Searching for Common Susceptibility Factors

Laukens¹,

Vos

2008

CRR

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Ileocolonoscopic evidence for subclinical gut inflammation is found in a subpopulation of spondyloarthropathy (SpA) patients. The prevalence of microscopic intestinal lesions is even higher and can be classified as either an acute or a chronic type of inflammation. The latter condition is associated with an increased risk of developing overt inflammatory bowel disease (IBD), especially Crohn's disease (CD), over time. Evidence for genetic predisposition in both SpA and IBD is strong, and has resulted in the identification of several linked chromosomal loci and putative candidate genes. The regular co-existence of SpA and IBD within the same family suggests a common genetic component. Interestingly, comparison of genome-wide linkage and association data reveals thirteen disease-associated chromosomal regions that are shared between SpA and IBD. This should convince geneticists to examine genes within these regions as potential susceptibility genes for the development of both SpA and IBD. Significant association of such shared genetic determinants was established for NOD2 (16q), the major histocompatibility complex I allele HLA-B27 (6p) and recently also the interleukin 23 receptor (1p). Transgenic animals in which tumor necrosis factor alpha or HLA-B27 is overexpressed suffer both joint and gut abnormalities resembling human SpA/CD pathology, providing additional evidence for a common genetic predisposition for the onset of joint and gut inflammation. In view of a hypothetical pathway leading to intestinal and articular inflammation in SpA and IBD, we review and compare genome-wide linkage and genetic association data obtained for SpA and IBD.Keywords: Spondyloarthropathy, Crohn's disease, genetic susceptibility, NOD2, HLA-B27, IL23R.Spondyloarthropathy (SpA) and inflammatory bowel disease (IBD) are chronic inflammatory conditions in which the initiating events are complex. In SpA, the axial skeleton, the sacroiliac joints and, to a lesser degree, peripheral joints become inflamed, whereas in IBD involving Crohn's disease (CD) and ulcerative colitis (UC), the intestine is the primary site of inflammation. The estimated prevalence in Western countries is 0.3-2% for SpA and 0.1-0.3% for IBD. The unity between SpA and IBD is illustrated by a strong clinical as well as molecular overlap. The first symptoms present themselves in early adulthood, usually before the age of 30, but early childhood and late onset disease occur sporadically. Medical treatment is mainly based on diminishing the inflammation and maintaining the remission states. Spondyloarthropathy is a heterogeneous group generally divided into 5 disease categories: ankylosing spondylitis (AS), reactive arthritis (ReA), psoriatic arthritis (PsA), SpA associated with IBD (SpA-IBD) and undifferentiated SpA. In the SpA-IBD group, patients suffer from clinically overt CD or UC. However, the gut is also an important site of inflammation in patients belonging to the other SpA disease categories. In ileocolonoscopic studies of SpA patients, histological ...

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Distinct Phenotype of Early Childhood Inflammatory Bowel Disease

Cited by 85 publications

References 29 publications

Definition of Phenotypic Characteristics of Childhood-Onset Inflammatory Bowel Disease

Definition of Phenotypic Characteristics of Childhood-Onset Inflammatory Bowel Disease

Incidence and Phenotype at Diagnosis of Very-early-onset Compared with Later-onset Paediatric Inflammatory Bowel Disease: A Population-based Study [1988–2011]

Genetics of Spondyloarthropathies and Inflammatory Bowel Disease: Searching for Common Susceptibility Factors

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