Pediatric inflammatory bowel disease

Background

The incidence of inflammatory bowel disease is increasing in the pediatric population worldwide.

Need and purpose of review

There is paucity of high quality scientific data regarding pediatric inflammatory bowel disease. Most of the guidelines are offshoots of work done in adults, which have been adapted over time to diagnose and treat pediatric patients. This is in part related to the small numbers in pediatric inflammatory bowel disease and less extensive collaboration for multicentric trials both nationally and internationally.

Methods

A literature search was performed using electronic databases i.e. Pubmed and OVID, using keywords: pediatric, inflammatory bowel disease, Crohn’s disease, Ulcerative colitis, epidemiology and guidelines. This article amalgamates the broad principles of diagnosing and managing a child with suspected inflammatory bowel disease.

Main conclusions

25% of the patients with inflammatory bowel disease are children and and young adolescents. The primary concern is its impact on growth velocity, puberty and quality of life, including psychosocial issues. Treatment guidelines are being re-defined as the drug armamentarium is increasing. The emphasis will be to achieve mucosal healing and normal growth velocity with minimal drug toxicity.

     

Progress in basic inflammatory bowel disease research

A modern approach to inflammatory bowel disease (IBD) research has been under way for little over one-half century, but only during the last two decades has progress accelerated and finally generated tangible results that have been translated into practical and better therapeutic strategies. The areas where progress has been more evident are those currently believed to be the key components of IBD pathogenesis, and include the environment, genetics, enteric microbiology, and immune reactivity. Progress in these different areas has been somewhat uneven, yielding a better understanding of the mechanisms behind gut inflammation and tissue injury rather than of specific etiological agents or predisposing factors. However, with the rapidly increasing utilization of novel methodological approaches like genetics, genomics, proteomics, and pharmacogenomics, it is reasonable to anticipate that the etiopathogenesis of IBD will be unveiled in the next couple of decades and more definitive, perhaps disease-modifying, approaches will be uncovered and implemented.     

Pediatric short bowel syndrome and subsequent development of inflammatory bowel disease: an illustrative case and literature review

Short bowel syndrome (SBS) in neonates is an uncommon but highly morbid condition. As SBS survival increases, physiologic complications become more apparent. Few reports in the literature elucidate outcomes for adults with a pediatric history of SBS. We present a case report of a patient, born with complicated gastroschisis resulting in SBS at birth, who subsequently developed symptoms and pathologic changes of inflammatory bowel disease (IBD) as an adult. The patient lived from age 7, after a Bianchi intestinal lengthening procedure, to age 34 independent of parenteral nutrition (PN), but requiring hydration fluid via G-tube. He was then diagnosed with IBD, after presenting with weight loss, diarrhea, and malabsorption, which required resumption of PN and infliximab treatment. This report adds to a small body of the literature which points to a connection between SBS in neonates and subsequent diagnosis of IBD. Recent evidence suggests that SBS and IBD have shared features of mucosal immune dysfunction and altered intestinal microbiota. We review current treatment options for pediatric SBS as well as multidisciplinary and coordinated transition strategies. We conclude that there may be an etiologic connection between SBS and IBD and that this knowledge may impact outcomes and approaches to care.     

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炎症性肠病(Inflammatoryboweldisease,IBD)包括溃疡性结肠炎(UlcerativeColitis,UD)和克罗恩病(Crohn’sdis ease,CD)。CD又称局限性肠炎(regionalenteritis)、节段性肠炎,也有称为肉芽肿性结肠炎(granulomatouscolitis)。19世纪30年代Crohn等人在美国医学会杂志上发表文章首次描述了该病的一些临床特征,到60年代中期Lockhart Mummery等人详细观察了本病的肠道特征,并率先提出与其它疾病肠道病变的鉴别要点,至1973年WHO专门委员会定名为Crohn's病。UD是1875年Wilks和Moxoh首先观察注意到该病的一些基本特征,到1903年Wilks和Boas…     

Update on paediatric inflammatory bowel disease

Inflammatory bowel disease in children is more prevalent than previously realized. Recent studies suggest an incidence in the UK of 5.3 per 100,000 in children under the age of 16 years, equivalent to around 700 new cases per annum, with Crohn's disease (CD) being at least twice as common as ulcerative colitis. The diseases appear to have a polygenic basis, with environmental factors providing the trigger of clinical disease in susceptible individuals. Endoscopy is vital for diagnosis, and new treatment modalities include the use of enteral nutrition as the sole therapy to induce and maintain remission, and the increasing early use of immunomodulatory agents such as azathioprine as steroid-sparing agents. Carefully timed, surgery is still important in both the acute and the chronic situation, particularly in the latter regard when growth faltering has not responded to medical therapy. Closer co-operation between specialist centres in the UK will hopefully provide an opportunity to conduct worthwhile multicentre research, in order to help tackle the many questions that still require a definite answer.     

Ultrasound assessment of the bowel: inflammatory bowel disease and conditions beyond

Ultrasound (US) is a versatile imaging study for the evaluation of the bowel in children. US imaging of the bowel can be used as the initial examination or in follow-up for many common pediatric diseases. In this article, we highlight our bowel US technique and describe how US can depict the features of a select group of bowel pathologies relevant to pediatric practice.     

Pathology of inflammatory bowel disease

Inflammatory bowel disease in childhood refers to ulcerative colitis, Crohn's disease, and colitis of an indeterminate type. Their gross and microscopic features are discussed along with the differential diagnosis from other childhood conditions associated with bloody diarrhea.     

Development and subsequent refinement of the inflammatory bowel disease questionnaire: a quality-of-life instrument for adult patients with inflammatory bowel disease

BACKGROUND: Health-related quality of life (HRQOL) describes the physical, social, and emotional attitudes and behavior of people in relation to health status. By the 1980s, HRQOL was noted to be impaired in chronic diseases but inflammatory bowel disease, had not been extensively described. METHODS: This review summarizes the results of several studies describing the development and application of a disease-specific HRQOL instrument, the Inflammatory Bowel Disease Questionnaire (IBDQ). RESULTS: An initial study described how patients with inflammatory bowel disease identified 150 problems they had experienced in four domains: bowel, systemic, emotional, and social function. Almost half of patients underreported impairment until encouraged by a reminder list. Thirty-two questions were included in the final questionnaire and were scored on a 7-point scale from I (worst) to 7 (best) for a range of possible scores from 32 to 224. Physicians' and spouses' global assessments correlated poorly with patient-reported HRQOL. Subsequent validation of the IBDQ suggested a strong correlation with disease severity (r = -0.5; p < 0.001) and a test-retest reliability of 0.7. Mean score changes of 16 to 30 points have been linked to changes in therapy. Statistically significant differences also occur between active and inactive disease. Results of four clinical trials have included the IBDQ as a measure of outcome. A self-administered version and a shortened version of the IBDQ have also been validated. CONCLUSIONS: The IBDQ is a valid, reliable, and sensitive measure that can be meaningfully applied in clinical trials. The short IBDQ may also be useful in clinical research and office practice.     

Pediatric "PSC-IBD": a descriptive report of associated inflammatory bowel disease among pediatric patients with psc

BACKGROUND: Inflammatory bowel disease (IBD) in adults with primary sclerosing cholangitis (PSC) is characterized by pancolonic involvement, a high frequency of rectal sparing, and an increased risk of pouchitis and colorectal neoplasia. The clinical features of IBD in pediatric patients with PSC have not been well described. The aim of this study was to characterize the frequency, clinical features, and natural history of IBD in pediatric patients diagnosed with PSC. METHODS: A retrospective chart review was performed for all patients 18 years of age or younger diagnosed with PSC seen at the Mayo Clinic between 1975 and 1999. Endoscopic and histologic features and surgical and postsurgical outcomes were recorded. RESULTS: Fifty-two children with PSC were identified. Forty-three patients (84%) were also diagnosed with IBD. In 36 of 43 cases, there was a sufficient diagnostic evaluation to allow a detailed review. Thirty-two of 36 patients (89%) had ulcerative colitis and 4 of 36 patients (11%) had Crohn's disease. In 4 of 36 patients (11%), IBD was asymptomatic. Although the most frequent endoscopic presentation of IBD was universal colitis, endoscopic rectal sparing was frequently noted (27% of colonoscopic studies). Of the four patients diagnosed with Crohn disease, in none did perianal, fistulizing, or stricturing disease develop. Proctocolectomy was performed in six patients (17%); three operations were performed for dysplasia. Pouchitis complicated four of the five ileal pouch-anal anastomoses procedures. CONCLUSIONS: Among pediatric patients (1) PSC without IBD is uncommon; (2) asymptomatic IBD may be associated with PSC; (3) because the time to dysplasia may be accelerated, once the diagnosis of IBD is made in the setting of PSC, heightened endoscopic surveillance may be indicated; (4) pouchitis occurs frequently in these patients.     

Nutritional management of inflammatory bowel disease

Malnutrition and growth failure are frequent complications of inflammatory bowel disease in childhood owing to inadequate dietary nutrient intakes, excessive intestinal losses, malabsorption, and increased nutrient requirements. Aggressive nutritional therapy is indicated for primary and supportive management of disease activity, drug nutrient interactives, individual nutrient abnormalities, and the overall complications of inflammatory bowel disease, malnutrition, and growth failure. The prevention of nutritional disorders in inflammatory bowel disease is accomplished by monitoring anthropometric and biochemical indices and by instituting appropriate enteral or parenteral nutritional therapy when indicated.     

Very early onset inflammatory bowel disease

Very early onset inflammatory bowel disease (VEO-IBD) represents a unique and growing subset of patients with inflammatory bowel disease (IBD). Some VEO-IBD patients present with immunodeficiency and possess loss of function genetic mutations involving immune pathways that cause their IBD. A search for Mendelian causes of IBD is likely most beneficial when the presentation involves extra-intestinal autoimmunity or involves intestinal histopathology that is atypical for IBD. While a subset of these young patients will have highly aggressive courses (and likely present with immunodeficiency), the majority of patients with VEO-IBD appear to have disease courses similar to that of their older counterparts. Most notably, many of these young children will require long courses of immunosuppression simply as a result of the profoundly early presentation—thus increasing their long-term risks of cancer and opportunistic infections.