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Inflammatory bowel disease in children.

Globally, the number of children and adolescents afflicted by inflammatory bowel disease has been increasing in recent years. This is true for parts of the world where the rates of inflammatory bowel disease have historically been high, such as Western Europe and North America, but also more recently in places like Japan and Asia, which have traditionally reported low numbers of patients with inflammatory bowel disease.

Although the reasons for this rise are not clear, the reality is that medical practitioners and parents alike need to be better informed about how inflammatory bowel disease may present in a young person, and the myriad repercussions that such a diagnosis may have.

Inflammatory bowel disease consists of Crohn's disease, ulcerative colitis and inflammatory bowel disease-unclassified. Crohn's and colitis are differentiated by the pattern and location of gastrointestinal tract (or gut) inflammation while inflammatory bowel disease-unclassified is a diagnosis applied in cases when a distinction between Crohn's and colitis cannot be made initially.

Inflammatory bowel disease is a life-long condition for which there is no cure. It is marked by periods of relative good control (remission) interspersed between flare-ups of the disease (relapses). The aim of management of inflammatory bowel disease is to achieve and then maintain good control and to prevent relapses and to prevent potential long-term complications.

While the exact cause of inflammatory bowel disease is unclear, it is understood that the disease occurs in genetically predisposed people when a dysregulated immune response is mounted to an interaction between elements travelling through the bowel (for instance, certain types of bacteria), and the lining of the gut. This cascade of events results in inflammation that, in Crohn's, can occur anywhere between the mouth and the anus.

In colitis, inflammation is limited to the large intestine (colon). In Crohn's, inflammation can involve the entire depth of the gut wall whereas in colitis the inflammatory changes are more superficial.

Typically, inflammation in colitis extends continuously from the rectum for a variable distance around the large bowel. The inflammation in Crohn's is, however, typically not continuous. Instead it is characterized by "skip lesions"--stretches of normal gut punctuated by areas of inflammation.

Approximately a quarter of inflammatory bowel disease diagnoses are made in those under the age of 20 years. While sharing some similarities, Inflammatory bowel disease in children differs from adult-onset disease in many important ways. Typically, the distribution of Crohn's differs in children, with extensive involvement commonly seen, along with many children having areas in the upper parts of the gut (e.g., the stomach) involved. Similarly, most children with colitis have involvement of the entire colon, with very few having disease in just the last few centimeters of the gut (known as proctitis).

These patterns are very different in adults with Crohn's or colitis. Children with colitis also commonly have more aggressive disease course, with higher rates of surgical intervention. Furthermore, children may have different genetic profiles than adults, and may respond differently to therapies.

The site of the gut that is involved mostly determines symptoms of inflammatory bowel disease in children. Colitis typically presents with bloody diarrhea, whereas Crohn's is often accompanied by abdominal pain, diarrhea and weight loss. However, a child with Crohn's involving just the colon will present more like someone with colitis.

A sign of particular concern that only affects children with inflammatory bowel disease is the plateauing of growth. Weight loss is common, but many will also have impairment of height gain while some will have delayed onset of puberty. Other manifestations of inflammatory bowel disease include mouth ulcers, lip swelling or changes around the anus such as skin tags, fissures or abscesses. Inflammatory bowel disease can also present with problems outside the gut: these include joint pain or swelling, skin rashes or eye pain.

It is crucial that a diagnosis of inflammatory bowel disease is made without unnecessary delay in children, as failure to do this may have negative long-term impact on growth and pubertal development.

The first step towards making the diagnosis is having an awareness of the disease and how it may present. Then, if faced with a child who may have inflammatory bowel disease, it is imperative that other, more common, causes for gut symptoms (such as infections or celiac disease) are properly excluded.

Following a thorough history and physical examination, the first step in investigations should include blood and stool tests. If a degree of suspicion remains, endoscopic investigation is undertaken along with biopsies taken from the various areas of the gut. In children, upper and lower gastrointestinal endoscopy is undertaken together to fully assess these areas of the gut. In almost all instances, radiological investigations are also arranged to evaluate the areas of the gut unable to be viewed endoscopically (namely the length of the small bowel).

Once a diagnosis is made, children with inflammatory bowel disease are optimally cared for by a multi-disciplinary team headed by a pediatric gastroenterologist. Other core team members should include a specialist inflammatory bowel disease nurse, a pediatric dietitian, as well as a psychologist and a pediatric surgeon whose services can be called upon as required.

The two phases of treatment for inflammatory bowel disease are, firstly, induction of remission (getting the disease under control) and then therapies to maintain remission (keep control). The preferred method to induce remission in children with Crohn's is Exclusive enteral nutrition. This nutritional approach involves the use of a liquid formula diet provided for a period of up to eight weeks, with complete exclusion of usual food elements. This treatment provides high rates of remission, high rates of healing of the bowel wall, and avoids side-effects that may occur secondary to drug therapies such as steroids.

Antibiotics may be an option in those with mild Crohn's, whereas biological therapies may be required in those with severe disease. Furthermore, some children with Crohn's will require an operation to establish remission. In colitis, aminosalicylates are often used initially in mild disease with steroids reserved for more severe cases.

Maintenance therapies may include ongoing supplementary nutrition, the aminosalicylates, or immunosuppressive drugs (such as azathioprine and methotrexate). In those children requiring a biological drug (e.g., infliximab or adalimumab) to induce remission, this agent may then be continued as a maintenance therapy. Many children will require ongoing therapy through childhood to maintain control of their Inflammatory bowel disease.

Drug therapy makes up just one facet of the management of children with inflammatory bowel disease. Nutritional guidance should be offered even during the maintenance phase to ensure that the affected child is growing normally. Psychosocial support is a crucial element in helping children accept and adjust to their life-long diagnosis. Peer support initiatives may also be very beneficial. In intractable cases, surgery can sometimes offer a lifeline.

While inflammatory bowel disease remains chronic and incurable, optimal management of inflammatory bowel disease should lead to an otherwise-motivated, well-supported young person living a fulfilling life, with normal growth and achievement of life goals.

Advances in the understanding of the causes of inflammatory bowel disease will continue to lead to the development of new therapies that promise to further enhance the outcomes of Inflammatory bowel disease.

By Robert N. Lopez, MD and Andrew Day MD

Dr. Lopez is a pediatric gastroenterology trainee currently based in Christchurch, New Zealand.

Professor Day is an academic paediatric gastroenterologist based in Christchurch, NZ. Day has extensive clinical and research interests in inflammatory bowel disease in children and adolescents.
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Author:Lopez, Robert N.; Day, Andrew
Publication:Pediatrics for Parents
Date:May 1, 2014
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