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Crohn's Disease
Tuesday, 02 September 2003
Wednesday, 13 July 2005
Ileitis, Regional Enteritis, Granulomatous Ileocolitis

What

Crohn’s disease is a form of inflammatory bowel disease and is characterized by chronic inflammation in any part of the digestive tract. The symptoms of the disease can be variable, depending on the location of the disease and the severity of the inflammation. The most commonly affected region is the ileum (the area where the small and large intestine meet).

Who

Crohn’s disease can occur in children and adults, but adolescents and young adults are at the greatest risk. The incidence of new cases of Crohn’s disease is about 5 to 10 per 100,000 individuals per year. About 20 to 25 percent of these cases are diagnosed in children under the age of 18 years, but this disease rarely occurs in children younger than 5 years of age. Males and females are equally affected and there are certain racial or ethnic groups that are more frequently affected. People of Jewish descent, particularly Ashkenazi Jews, have an increased risk. Crohn’s disease appears to be most prevalent in North America, northwestern Europe, and the United Kingdom.

Signs and Symptoms

The most common symptoms are abdominal pain, poor appetite, weight loss, and diarrhea (may not be present). There may be blood in the stools and the child may feel tired and weak. Poor growth, an especially important symptom, is almost always present and delayed sexual maturation may occur and may be noticeable before intestinal symptoms develop. Perianal lesions (problems around the anus), anemia, and weight loss may be present as well. Symptoms in areas other than the intestines may be present, but are far less common. These include arthritis, recurrent fevers, recurrent oral ulcers, inflammation of the lips, clubbing of the digits, raised red lesions seen mostly on the shins, and an ulcerating rash. If not adequately controlled, the inflammation in the intestines can cause holes to form there and scarring may occur, which can cause serious infections or obstruct food and air from moving through the intestines.

Possible Causes

Crohn’s disease is caused by complex interactions between genetic factors and environmental factors. Humans are made up of cells that form tissues and organs in the body. Each cell has 23 pairs of chromosomes within it, numbered from 1 to 22 with the last pair being sex chromosomes that determine gender, that hold DNA which tells the body how to develop and function correctly. Each chromosome pair is made up of one chromosome from the mother’s egg and one from the father’s sperm. On chromosome 16, a gene (IBD1) has been discovered that explains the genetic component of Crohn’s disease. If one copy of this gene is mutated in some way, the child has a 2.6 fold increase in risk of developing this disease. If both copies of this gene are mutated, the child has a 40-fold increase in risk of developing Crohn’s disease. This gene could be mutated and passed down from one or both parents or could have a sporadic mutation. This disease also shows genetic anticipation, meaning that the disease tends to develop at an earlier age and with increased severity with each generation that is affected in the family. Research is being done to determine if other genes are involved as well. Having the mutated gene alone does not explain the occurrence of Crohn’s disease, which has led to the belief that environmental factors also play a role. Infection by bacteria, increased numbers of normal bacteria in the body, and smoking are all risk factors for developing Crohn’s disease. It is believed that, with the combination of the genetic and environmental factors, an autoimmune response occurs, which is a malfunction in the immune system that causes it to attack the body’s own healthy cells in the gastrointestinal system. This causes inflammation, ulceration, and thickening of the gastrointestinal tract.

Diagnosis

While there is no single test that can confirm the suspicion of Crohn’s disease, several tests can be done to determine the diagnosis, usually by both the general pediatrician and a pediatric gastroenterologist. A full history and physical exam will be done to establish risk factors and presenting symptoms, such as an abdominal mass or tenderness as well as other symptoms described above. Usually a stool culture will be done to rule out infection immediately. A blood test can be done to look for specific markers that people with Crohn’s disease tend to have in the blood. X-rays may be done to examine the intestines. However, the primary procedure for diagnosis is endoscopy and colonoscopy, which involves threading a thin flexible tube with a tiny camera inside of it into the intestine from the mouth or the anus, respectively. This procedure allows the physician to see the intestines and look for inflammation or abnormalities. A biopsy of the intestines can also be taken during this procedure to look for microscopic signs of Crohn’s disease.

Treatment

Treatment for Crohn’s disease is a multidisciplinary effort and can involve the primary pediatrician, a pediatric gastroenterologist, other pediatric subspecialists, a surgeon, a nutritionist, a gastrointestinal nurse specialist, and a variety of other people. The goal of treatment is to decrease inflammation, relieve symptoms, optimize growth, normalize pubertal development, facilitate normal social development, and avoid long-term disease-related complications. Growth is the measure of success of therapy. The diet should include abundant protein with enough calories to restore weight and support growth. Milk, alcohol, hot spices, and fiber from raw fruits and vegetables may worsen symptoms and should be avoided if this happens. If the child is not receiving enough nutrition, a tube may be temporarily placed in the stomach or through the nose into the stomach to supply nutrients. Mineral losses are common and may cause deficiency in iron, zinc, calcium, magnesium, and phosphorus. These should be treated appropriately. Medications may be used to help control the inflammation. These may include corticosteroids, antibiotics (sulfasalazine, mesalamine, metronidazole), and immunomodulatory agents (azathioprine, 6-mercaptopurine, methotrexate, cyclosporine, tacrolimus, infliximab). A combination of medications may be used to limit the dosage required and thus the side effects of any one medication. If no other forms of treatment are effective, surgery may be done to remove the diseased segments of the intestine. Despite all the different forms of treatment, Crohn’s disease is not currently curable but research is being done in the hope of reaching this ultimate goal.

Prognosis

The prognosis for each individual with Crohn’s disease varies. However, death from Crohn’s disease is extremely rare in children. Symptoms tend to flare up but then disappear for periods of time. Having Crohn’s disease puts individuals at a greater risk for small bowel or colon cancer later in life. Therefore, children with Crohn’s disease should probably undergo colonoscopy for cancer screening beginning about 8 to 10 years after the diagnosis of Crohn’s disease occurs. With proper treatment, most individuals with Crohn’s disease can lead normal lives.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

About Crohn’s Disease: The Basics A great website with concise information, description of the anatomy, and links to ongoing research studies. National Digestive Diseases Information Clearinghouse (NDDIC): Crohn’s Disease An informative website with a description of medications, links to support groups and other information with some specific to children. Crohn’s and Colitis Foundation of America A comprehensive website with links to events and current research. Crohn’s/Colitis Homepage A good website with links to support groups and information about specific medical institutions. Teens with Crohn’s Personalized chat group/website for those interested in sharing first-hand experiences. Healing Well.Com http://www.healingwell.com/ibd/ Crohn’s resource center for parents and patients.

Google Search for Crohn's Disease

References and Sources

Clinical manifestations and diagnosis of Crohn's disease in children and adolescents www.utdol.com/application/topic.asp?file=pedigast/12528&type=P&selectedTitle=1~97 Overview of the management of Crohn's disease in children and adolescents www.utdol.com/application/topic.asp?file=pedigast/12773&type=P&selectedTitle=2~97 Walker AW et al (2004). Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. 4th ed. Ontario: BC Decker Inc. Balfour Sartor R, Sandborn W J (2004). Kirsner’s Inflammatory Bowel Diseases. 6th ed. London: Saunders Bayless TM, Hanauer SB (2001). Advanced Therapy of Inflammatory Bowel Disease. Ontario: BC Decker Inc.