Characteristics of Inflammatory Bowel Disease With Onset During the First Year of Life

Ruemmele, Frank M; Khoury, M. G. El; Talbotec, Cécile; Maurage, C.; Jf, Mougenot; Schmitz, J; Goulet, Olivier

doi:10.1097/01.mpg.0000237938.12674.e3

Cited by 112 publications

(91 citation statements)

References 35 publications

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“…These findings have been confirmed by the ESPGHAN registry [35]. Limited data exist on CD in children under age 1 year and, although CD is diagnosed in infants, this may represent a particular subgroup of patients [36].…”

Section: Paediatric CD Disease Coursesupporting

confidence: 62%

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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Section: Paediatric CD Disease Coursesupporting

confidence: 62%

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

Veereman-Wauters¹,

Cucchiara²

2008

TOPEDJ

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“…The most common diagnoses responsible for colonic inflammation in the first few months of life are either allergy or infectious diseases. Other causes can include early-onset inflammatory bowel disease 6 (IBD), autoimmune entheropathies (similar to immune polyendocrinopathy X-linked syndrome, X-linked inhibitor of apoptosis deficiency, and deficit in the IL-10/IL-10 receptor), chronic granulomatosis disease, glycogenosis 1b, carbohydrate-deficient glycoprotein syndrome, or Hermansky-Pudlak syndrome. 7 Enteropathy was severe in both patients.…”

Section: Figurementioning

confidence: 99%

Severe Early-Onset Colitis Revealing Mevalonate Kinase Deficiency

Levy

Arion²,

Berrebi

et al. 2013

Pediatrics

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Hyperimmunoglobulinemia D is the less severe form of mevalonate kinase deficiency (MKD) caused by recessive inherited mutation in the mevalonate kinase gene. Hyperimmunoglobulinemia D is characterized by febrile attacks, often associated with transient digestive manifestations, such as abdominal pain, diarrhea, and vomiting. Here we report for the first time 2 patients with MKD revealed by severe neonatal colitis. Both patients had chronic bloody diarrhea and failure to thrive; 1 patient since the age of 1 month and the other since the age of 12 days. Total parenteral nutrition was required. A marked elevation of acute phase reactants was present, and no evidence of infection was found. In patient 1, ileocolonoscopy revealed ulcerative colitis at the age of 5 months. Patient 2 suffered from enterocolitis and shock, associated with multiple bowel adhesions at age 5 weeks; the rectosigmoidoscopy showed aphtoid lesions of the sigmoid colon. Pathologic findings of colonic biopsies revealed a dense polymorph inflammatory infiltrate associated with deep ulcerations. Febrile attacks occurred 2 months after the onset of digestive symptoms in patient 1, and at onset of disease in patient 2. Genomic sequencing of the mevalonate kinase gene revealed compound heterozygous mutations in both patients. Anti-interleukin-1 agent produced longterm remission of all digestive features and laboratory parameters. This report emphasizes that MKD may be the cause of severe earlyonset inflammatory colitis, and must be considered by physicians, even in the absence of fever, after ruling out infections. Antiinterleukin-1 therapy may result in a dramatic improvement of MKD-related inflammatory bowel disease. Pediatrics 2013;132:e779-e783 AUTHORS:

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“…It is thought that inappropriate or exaggerated mucosal immune response to enteric infections may be involved in the initial etiology of some cases of IBD. In infants less than one year old who developed IBD, 50% had a prior bacterial infection requiring antibiotic therapy [29] . Although as intriguing as this finding is, more research is needed to determine if enteric infections cause IBD.…”

Section: Pathogenesismentioning

confidence: 99%

State-of-the-art of irritable bowel syndrome and inflammatory bowel disease research in 2008

McFarland¹

2008

WJG

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Irritable bowel syndrome (IBS) and inflammatory bowel disease (IBD) are two of the leading causes of chronic intestinal conditions in the world. This issue of World Journal of Gastroenterology (WJG ) presents a series of papers from world experts who discuss the current knowledge and opinions on these important conditions. Although great strides have been made in the diagnosis, treatment and pathology of IBS and IBD; much has yet to be explained. The etiologies and risk factors of these multifactorial conditions remain elusive. Specific diagnostic biomarkers need to be developed and safer treatments developed. The burden of IBS and IBD on the healthcare system is felt with repeated medical care visits and high costs. IBS and IBD patients can account for 30%-50% of office visits at gastroenterology services/clinics. Over one million people have IBD in the United States, with 30 000 new cases being diagnosed every year. One-quarter million people in the UK are afflicted with IBD. The cost of medical care in the United States for IBD is estimated to be $1.8 billion/year. IRRITABLE BOWEL SYNDROME (IBS)Incidence of IBS IBS is a global problem and is more common in women than men. In developed countries, the prevalence of IBS ranges from 3%-25% of adults [1][2][3][4] and in the United States, IBS affects 15 million adults [5] . In the United Kingdom, IBS affects 10%-15% of the adult population [6] . The cost of direct and indirect medical care for IBS reached over $200 billion dollars in the United States [5,7] . Diagnosis of IBSAs there are no biologic markers for IBS, the diagnosis is usually based on symptoms and exclusion of other known causes of intestinal distress [6] . Unlike IBD, IBS does not cause severe inflammation, ulcers or other structural damage that aids the diagnosis of IBD. IBS is a functional disorder characterized by abdominal bloating, flatulence, abdominal pain and bowel dysfunction. The varying nature of symptoms and lack of structural abnormalities presents a diagnostic challenge. There are three main types of disease phenotypes: diarrhea-predominant (IBS-D), constipation-predominant (IBD-C) or alternating diarrhea-constipation (IBS-A). The diarrhea-predominant type is more common (48%) in males, whereas constipation-predominant (39%) or alternating types (48%) are more common in women [1] . Several tools (for example, Rome Ⅲ, Manning criteria) have been developed to standardize the diagnosis of IBS. Most of the historic research has been focused on the pathophysiology of diarrhea, but constipation has not been as well described. The review by McCrea and colleagues in this issue summarizes our current knowledge about the physiology and pathology of constipation [8] . The prevalence of constipation ranges from 15%-25% in the general population and is more common in women than men and in ages over 70 years old [9] . The typical definition of constipation (less than three stools/week) may not be a sensitive measure for this condition, as individuals vary widely in their own bowel habits. ...

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Characteristics of Inflammatory Bowel Disease With Onset During the First Year of Life

Abstract: Very early onset IBD may reflect a subgroup of patients characterized by a particular sensitivity to modifications of the intestinal flora. Neonatal IBD was most often severe in presentation and evolution.

Cited by 112 publications

References 35 publications

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

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

Severe Early-Onset Colitis Revealing Mevalonate Kinase Deficiency

State-of-the-art of irritable bowel syndrome and inflammatory bowel disease research in 2008

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