• Unknown

• The intestinal wall is edematous, with wall thickening and strictures.
• Mesentery can be thickened, with enlarged lymph nodes.
• Normal bowel can exist in continuity with affected bowel (skip areas).
• Matting together of inflamed bowel and organs
• Abscesses/fistulas
• Inflammatory cells infiltrate all layers of the intestine.
• Epithelioid granulomas are found in 40% of biopsies of patients and are pathognomonic.

• First-degree relatives have a 5% to 25% higher risk than normal population.
• Family members of patients with Crohn disease have increased risk for both Crohn disease and ulcerative colitis.
• Offspring have an 8.9% and siblings an 8.6% of developing IBD.
• Concordance in monozygotic twins is 50%.

• The incidence rate in children is 10 per 100,000 in North American 10- to 19-year-olds.
• Forty percent of patients first present in childhood or adolescence.
• Males and females are equally affected in adulthood, but in childhood, there is a 2:1 male:female ratio.

• Toxic megacolon is a rare but serious complication.
• Obstruction (8%–40%) due to strictures, phlegmon, adhesions, giant polyposis, gallstones, lymphoma
• Abscess due to fistula and perforation
• Enteroenteric, enterovesical, enterovaginal, and enterocutaneous fistulas occur.
• Perianal disease affects 25% to 50% of patients.
• Malabsorption occurs in small bowel disease. The nutrient affected depends on the site of disease (e.g., B12 deficiency: terminal ileum; iron deficiency: duodenum).
• Incidence of adenocarcinoma is reported to be 4 to 20 times that of the general population.
• Massive hemorrhage is rare (1%). Rectal bleeding is common.
• Growth failure is frequent; final height is reduced and puberty is delayed in Crohn disease affecting prepubertal children.

• Ulcerative colitis
• Appendicitis
• Infection: Mycobacteria, Salmonella, Shigella, Campylobacter, Aeromonas, Yersinia, Clostridium difficile, Escherichia coli, Giardia, Cryptosporidium
• Hemolytic-uremic syndrome
• Henoch-Schönlein purpura
• Irritable bowel syndrome
• Peptic ulcer disease
• Constipation
• Autoimmune enteropathy, immunodeficiency
• Primary lactase deficiency
• Psychosocial disturbance

• Frequency of signs and symptoms
  • Weight loss, 85%
  • Diarrhea, 80%
  • Abdominal pain, 75%
  • Rectal bleeding, 50%
  • Growth failure, 35%
  • Nausea and vomiting, 25%
  • Rectal disease, 25%
  • Extraintestinal signs, 25%
  • Perianal disease, 15%
• Symptoms depend on the site of disease activity.
• The sites most often affected in decreasing frequency are terminal ileum, right colon, isolated colon, proximal small bowel, and gastroduodenum.

• Recurrent abdominal pain
• Diarrhea
• Weight loss
• Recent travel (enteric infections)
• Antibiotic use (C. difficile)
• Rectal bleeding
• Family history of IBD
• Appendectomy
• Growth failure: Careful charting of recent growth parameters, especially growth velocities from school or medical records, is essential.
  • Patients with small bowel disease have emesis, nausea, pain, and diarrhea. Diarrhea is secondary to malabsorption and bacterial overgrowth. Growth retardation is more frequent in these patients.
  • Patients with distal disease have symptoms of diarrhea, rectal bleeding, and urgency.
• Extraintestinal disease: arthritis, erythema nodosum, pyoderma gangrenosum, mouth ulcers, uveitis, hypercoagulable states, vasculitis, renal stones, amyloidosis, sclerosing cholangitis
• Extraintestinal symptoms:
  • Aphthous ulcers
  • Arthritis
  • Erythema nodosum
  • Pyoderma gangrenosum

• Decreasing height and weight percentiles over the past few years
• Abdominal examination: hyperactive bowel sounds, RLQ mass and tenderness
• Rectal examination: perirectal disease (tag, fissure, hemorrhoids, abscesses)

• Blood count: microcytosis, macrocytosis suggesting nutrient deficiency: iron, B12, folate, zinc levels
• ESR (disease activity)
• Electrolytes (hydration, renal function)
• Transaminases
• Alkaline phosphatase
• g-Glutamyl transpeptidase (hepatobiliary disease)
• pANCA Crohn disease (19% positive) and UC (80% positive)
• ASCA (anti-Saccharomyces cerevisiae antibody) Crohn disease (70% positive)
• Stool for occult blood and presence of white cells
• Stool cultures, C. difficile toxin A and B

• Consider plain abdominal radiograph in acute presentation.
• Barium upper GI and small bowel follow-through; this shows extent of disease in small bowel that is not accessible to endoscopy.
• Barium enema has been replaced by colonoscopy in acute colitis; useful in evaluation of complications such as strictures and fistulas.
• CT scan and ultrasound are useful tools for evaluating complications and disease severity.
• Endoscopy with multiple biopsy enables visualization and tissue diagnosis and is the test of choice for initial evaluation of colonic Crohn disease. Upper endoscopy with biopsy is indicated in patients with symptoms of gastroduodenal disease.

• Prednisone can control intestinal inflammation. Dosage is 1 to 2 mg/kg/d oral prednisone (maximum, 60 mg). Initially, patient is treated for 4 to 6 weeks and tapered 5 mg weekly to the lowest amount tolerated.
• Sulfasalazine, a biologically active congener of sulfapyridine and 5-aminosalicylate, the latter being antiinflammatory. It may be useful in prophylaxis in adult disease. Dose is 50 to 75 mg/kg/d t.i.d (maximum, 4 g/d). Folate (1 mg/d) supplementation is also used.
• There are many ASA preparations used according to their sites of efficacy to correspond to diseased bowel:
  • Asacol (terminal ileum, colon) 30 to 50 mg/kg/d (maximum 4.8 g/d for active disease and 3.2 g/d to maintain remission)
  • Pentasa (duodenum, jejunum, ileum, colon), 50 to 60 mg/kg/d (maximum, 4 g/d for active disease and 3 g/d to maintain remission)
  • Dipentum (colon)
  • Rowasa, 4-g enemas and 500-mg suppositories
• Azathioprine, 2.0 to 2.5 mg/kg/d, and its metabolite 6-mercaptopurine, starting with 1.0 to 1.5 mg/kg/d to maintain white cell count greater than 4×10⁹/L and platelets greater than 100×10⁹/L, can maintain remission and is steroid-sparing. It is also useful for treating fistulas.
• Cyclosporine: unclear utility in Crohn disease in children; nutritional management is as follows:
  • Bowel rest with parenteral nutrition
  • Elemental diet reported to be effective in inducing remission in active disease
  • To correct growth failure, an increase in caloric intake is recommended and can be given as overnight nasogastric feeds if oral supplements are not tolerated. An elemental formula is recommended.
• Antibiotics
  • Metronidazole and Ciprofloxacin are useful for intestinal and perianal disease.
• Topical hydrocortisone is useful in localized left-sided disease and is available in liquid and foam enemas. Absorption is dependent of degree of inflammation and length of exposure, but up to 75% may be absorbed. Poorly absorbed topical steroids, such as budesonide, are not yet available.
• cA₂ (anti-TNF): recently FDA-approved medication. Initial use will be for patients unresponsive to the above medical therapies (5 mg/kg IV infusion). Potentially will be given every 2 to 3 months if necessary.

• The morbidity of this disease is very high. The majority of patients experience recurring disease.
• In adults, 55% of patients will have mild-to-severe disease at any one time, with the remainder in remission.
• Patients with colonic disease seem to suffer more from extraintestinal disease and are more refractory to treatment.
• Exacerbation of disease in children often follows intercurrent viral illness, such as EBV and adenovirus.
• The cause of poor growth is multifactorial, including anorexia, malabsorption, increased energy expenditure, and prolonged corticosteroid use.
• Most patients have good general health in between disease and go on to lead productive lives.
• Death is a rare complication (2.4% in a large series).
• After 5 and 20 years of disease, the probability of survival was 98% and 89% of expected survival, respectively.
• In terms of risk of neoplasia, incidence of adenocarcinoma of the rectum and colon is reported to be 4 to 20 times that of the general population.
• There are no good population base studies looking at the incidence of lymphoma in IBD. One study of 2,000 patients found that 1 in 300 Crohn disease patients develops lymphoma.

Q: Will my child have this disease forever?
A: There are many different clinical presentations of Crohn disease. Some people will have only the initial attack and then are symptom-free, but usually an individual will have episodes of recurrences and remissions. Presently, research is being done to identify the genetic factors in Crohn disease; once the genes can be identified, a cure will be possible.

Q: What is the cause of Crohn disease?
A: Both genetic and environmental factors are important in the development of Crohn disease. Possible environmental factors include aseptic environment in the first few years of life, lack of breast feeding, frequent use of antibiotics or aspirin, and diet.

Q: Where can I learn more about Crohn disease?
A: The Crohn and Colitis Foundation of America (CCFA) is a nonprofit organization dedicated to the care of people with Crohn disease and ulcerative colitis.

Q: What new therapies will be used in the near future?
A: Biologic agents, which is a type of therapy that uses our recently improved knowledge of the immune system, either down-regulate inflammatory mediators or up-regulate immunomodulatory mediators. It is hoped that this new class of therapies will greatly improve our care of people with IBD.

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