Pediatric Crohn Disease 

  • Author: Andrew B Grossman, MD; Chief Editor: Carmen Cuffari, MD   more...
 
Updated: Apr 27, 2011
 

Background

Crohn disease (CD), or Crohn's disease, is a chronic inflammatory bowel disease. Once considered rare in the pediatric population, Crohn disease is recognized with increasing frequency among children of all ages. Approximately 20-30% of all patients with Crohn disease present when they are younger than 20 years. With its increasing recognition, Crohn disease has become one of the most important chronic diseases that affect children and adolescents.

In addition to the common GI symptoms of diarrhea, rectal bleeding, and abdominal pain, children often experience growth failure, malnutrition, pubertal delay, and bone demineralization. Other problems unique to the pediatric population include the paucity of controlled clinical trials and the psychological issues that occur in children and adolescents with Crohn disease. The unique problems encountered in the pediatric population necessitate a medical approach that promotes clinical improvement and reverses growth failure with minimal toxicity.

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Pathophysiology

The pathogenesis of Crohn disease is multifactorial. After a triggering event occurs in a genetically susceptible individual, an altered immune response leads to chronic inflammation of the intestine. Although the etiology of the precipitating event is unknown, luminal bacteria or specific antigens are thought to be involved.

Chronic inflammation from T-cell activation leading to tissue injury is implicated. After activation by antigen presentation, unrestrained responses of helper lymphocytes type 1 (Th1) predominate in Crohn disease because of defective regulation. Th1 cytokines, such as interleukin (IL)-12 and tumor necrosis factor (TNF)-alpha stimulate the inflammatory response. Inflammatory cells recruited by these cytokines release nonspecific inflammatory substances, including arachidonic acid metabolites, proteases, platelet activating factor, and free radicals, which result in direct injury to the intestine.

The macroscopic findings at the time of endoscopy and/or colonoscopy or surgery include various degrees of edema, erythema, ulceration, friability, thickening of the bowel wall and mesentery, and extension of fat over the serosal surface of the intestine (see image below).

Colonoscopic image of a large ulcer and inflammatiColonoscopic image of a large ulcer and inflammation of the descending colon in a 12-year-old boy with Crohn disease.

Skipped areas of inflammation anywhere in the upper or lower GI tract are characteristic of Crohn disease, in contrast to the continuous diffuse colonic inflammation found with ulcerative colitis (UC). Microscopic findings on intestinal mucosal biopsy consist of chronic inflammation with architectural distortion (see image below).

Histologic features of chronic colitis with crypt Histologic features of chronic colitis with crypt atrophy and branching, and lymphocytic infiltrate. Hematoxylin-eosin staining. Courtesy of Dr E. Ruchelli.

Granulomas (see image below) are sometimes noted on biopsy findings in Crohn disease but never in UC; their presence can be useful in distinguishing between these 2 entities.

Colonic granuloma in a patient with Crohn disease.Colonic granuloma in a patient with Crohn disease. Hematoxylin-eosin staining. Courtesy of Dr E. Ruchelli.
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Epidemiology

Frequency

United States

Over the past few decades, the incidence of inflammatory bowel disease (Crohn disease in particular) has greatly increased. The age-specific rate in North America for children aged 10-19 years is estimated to be approximately 3.5 cases per 100,000 population. The only prospective pediatric epidemiologic study from North America showed that the rate of Crohn disease in Wisconsin was 4.56 cases per 100,000 population, or twice that of UC.[1]

International

The rate of Crohn disease in Europe and Canada is 2.1-3.7 cases per 100,000 population, and rates are somewhat higher in northern regions than southern regions. Crohn disease is rare in Africa, Asia, and South America.

Mortality/Morbidity

Death from Crohn disease is extremely rare in children and adolescents. Severe and complicated Crohn disease may result in prolonged hospitalizations, surgeries, growth failure, malnutrition, pubertal delay, and poor quality of life.

Race

Crohn disease is more common in whites than in blacks and is rare in Asian and Hispanic children. Rates are higher in people of Jewish descent, particularly Ashkenazi Jews and Jews of middle European origin compared with Sephardic or eastern European Jews.

Sex

The rate of Crohn disease in women is 1.1-1.8 times higher than that in men. There is a reverse pattern with pediatric inflammatory bowel disease, with a higher rate in boys than in girls. In the United States, the pediatric male-to-female ratio in 2003 was 1.6:1.

Age

The rate of Crohn disease reaches its first peak in the second and third decade of life. The second, smaller peak occurs in adults aged 60-80 years. Approximately 25% of all cases of inflammatory bowel disease are diagnosed before age 20 years.

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Contributor Information and Disclosures
Author

Andrew B Grossman, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine

Andrew B Grossman, MD is a member of the following medical societies: American Gastroenterological Association, Crohns and Colitis Foundation of America, and North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Coauthor(s)

Petar Mamula, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology and Nutrition, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine

Petar Mamula, MD, is a member of the following medical societies: American Academy of Pediatrics, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Specialty Editor Board

Robert Baldassano, MD  Director, Center for Pediatric Inflammatory Bowel Disease, Division of Gastroenterology and Nutrition, Associate Professor, Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania

Robert Baldassano, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Stefano Guandalini, MD  Director, University of Chicago Celiac Disease Program, Section Chief of Gastroenterology, Hepatology and Nutrition; Professor, Department of Pediatrics, University of Chicago Comer Children's Hospital

Stefano Guandalini, MD is a member of the following medical societies: American Gastroenterological Association, European Society for Paediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF  Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD  Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

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Colonoscopic image of a large ulcer and inflammation of the descending colon in a 12-year-old boy with Crohn disease.
Histologic features of chronic colitis with crypt atrophy and branching, and lymphocytic infiltrate. Hematoxylin-eosin staining. Courtesy of Dr E. Ruchelli.
Colonic granuloma in a patient with Crohn disease. Hematoxylin-eosin staining. Courtesy of Dr E. Ruchelli.
Image obtained during upper GI series with a small-bowel follow-through shows narrowing and irregularity in the distal ileum in a 16-year-old male adolescent with Crohn disease.
MRI of an inflamed terminal ileum in a 10-year-old girl with Crohn disease.
MRI of a small abscess on the right side of the anal sphincter in a 9-year-old boy with Crohn disease.
Table. Characteristics Differentiating Crohn Disease and Ulcerative Colitis
Characteristic
Crohn diseaseUlcerative Colitis
DistributionEntire GI tractColon only, although gastritis recognized
Skip lesionsContinuous involvement proximally from rectum
PathologyFull thicknessMucosa only
Granulomas (30%)No granulomas
RadiologyEntire GI tractColon only
Skip lesionsContinuous involvement proximally from rectum
Fistulas, abscesses, fibrotic stricturesMucosal disease only
Cancer riskIncreasedEstimated 1% per year starting 10 years after diagnosis
Presentation
Crohn DiseaseUlcerative Colitis
BleedingCommonVery common
ObstructionCommonUncommon
FistulaCommonNone
Weight lossCommonUncommon
Perianal diseaseCommonRare
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