A 15-year-old female came for a second opinion to the general pediatrics clinic because of a 3 month history of abdominal pain described as more in the right upper quadrant but also left upper quadrant. The pain was described as crampy and would occur most days and also at night. She had 5-10 times/day stools that were described as loose without blood or mucous. Intermittently she had emesis. She was not able to participate in school or other activities because of the pain. She had a decreased appetite and had lost 3-5 pounds over the 3 months. The past medical history was negative. The family history was positive for possible inflammatory bowel disease (IBD) in the mother but she was not sure and didn’t take any medication. The review of systems showed no fever, cold intolerance, skin or visual changes, and no changes in flatulence or belching. She reported normal menses and no recent travel.
The pertinent physical exam showed an unhappy appearing teenager who said her pain was 7/10 but appeared comfortable. Her documented weight loss was 1.8 kilograms and other vital signs were normal. She complained of pain anywhere with palpation but no masses, organomegaly, ascites or rebound tenderness was noted. She had a dilated rectal vault and the stool was guiac negative. The work-up locally showed a hemoglobin of 11.3 g/dl, and platelets of 417 x 1000/mm2. She had normal electrolytes, blood urea nitrogen, creatinine, transaminases, bilirubin, albumin and total protein, lipase, amylase, urinalysis and erythrocyte sedimentation rate. An ultrasound and computer tomography examination of her abdomen and pelvis were normal. The diagnosis of possible inflammatory bowel disease and/or irritable bowel syndrome was made. The pediatrician felt with the history of no hematochezia over this time period that inflammatory bowel disease was less likely. However the patient did have a mild anemia and thrombocytosis which could be consistent with it and also the possible family history. She elected to start the patient on a high fiber diet and to refer to the pediatric gastroenterologist for possible endoscopy. Although the daughter was not excited about endoscopy, she thought that this was a good idea to be able to decide if she did have IBD and then could possibly start treatment. The patient’s clinical course showed she did have some improvement in her symptoms when she went to the gastroenterologist, and additional history revealed a cousin with Crohn’s Disease. Therefore the decision was made to perform endoscopy which was scheduled in 2 weeks.
Inflammatory bowel disease (IBD) is made up of two major diseases: Crohn’s Disease (CD) and Ulcerative Colitis (UC). CD is more common than UC in children. Extraintestinal manifestations are less common in children (6%) but increase to 25% in adulthood. Children with IBD have problems with growth and often have delayed puberty. Despite the delayed puberty, many may not reach predicted adult height.
- Crohn’s Disease
- 2.1-3.7 cases per 100,000 population
- Gender: Males > females until puberty then about the same rate
- Ileocolonic or colonic location predominance in children (more terminal ileum and less colonic in adults). Note bene: any area of the gastrointestinal tract can be affected from mouth to anus.
- Involves entire thickness of the gastrointestinal tract wall
- Skipped areas of colonic involvement are common
- Granulomas are seen clinically and histologically
- Nonstricturing, nonpenetrating colonic wall disease usually initially that over time becomes more stricturing/fistulizing and penetrating in many children
- Abdominal pain, diarrhea, hematochezia, malnutrition, growth failure, weight loss, demineralization of bone are common
- Genetics and cancer risk: Highly inheritable and has increased cancer risk
- Ulcerative Colitis
- 2 cases per 100,000 persons
- Gender: Males = females at all ages
- Pancolitis in children (more left-sided, proctitis in adults)
- Involves the mucosa only
- Continuous colonic involvement (no skipped areas)
- No granulomas
- Pancolitis with a shorter timespan to colectomy (if needed) in children than adults
- Hematochezia, abdominal pain and diarrhea are common. Weight loss, fatigue, fever, and problems with growth may also occur
- Genetics and cancer risk: Highly inheritable and has increased cancer risk
Serological testing for possible IBD has become available recently. Two recent studies that compared this panel to routine laboratory testing found better predictive values for routine laboratory testing (specifically hemoglobin, platelet count and erythrocyte sedimentation rate (ESR), one study also evaluated albumin). The authors of these papers recommend a complete blood count (evaluating for anemia and thrombocytosis) and ESR (evaluating for evidence of inflammation) as screening tests to help determine the necessity for more additional invasive/expensive testing.
It is important to note that children with IBD may have totally normal screening tests. In 2007, Mack et. al., showed that normal values were obtained for ESR (26% and 18%, for all patients and moderate/severe disease patients respectively), hemoglobin level (32% and 24%), platelet count (50% and 43%), and albumin (60% and 50%). Hypoalbuminemia may be seen in IBD. The frequency of having all four laboratory tests being normal was 9% for CD and 19% for UC. The authors point out that if all laboratory testing is normal that other diseases such as irritable bowel syndrome are more likely however, “…normal laboratory tests cannot be relied on as an adequate screening tool to exclude mild IBD….children with more-severe IBD only rarely have all 4 of the laboratory tests yield normal rests at presentation.” They also point out that hematochezia is a common compliant for many children diagnosed with IBD, and in UC hematochezia is more common the more severe the symptoms.
A differential diagnosis of gastrointestinal bleeding can be found here.
Questions for Further Discussion
1. What clinical indications warrant radiological testing or endoscopy?
2. What treatments are currently available for IBD?
3. Because of the increased risk of cancer, what screening is recommended?
4. What extraintestinal manifestations are common in IBD?
- Specialty: Gastroenterology
- Age: Teenager
To Learn More
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Mack DR, et al., Pediatric Inflammatory Bowel Disease Collaborative Research Group. Laboratory values for children with newly diagnosed inflammatory bowel disease. Pediatrics. 2007 Jun;119(6):1113-9.
Sabery N, Bass D. Use of serologic markers as a screening tool in inflammatory bowel disease compared with elevated erythrocyte sedimentation rate and anemia. Pediatrics. 2007 Jan;119(1):e193-9.
Sauer CG, Kugathasan S. Pediatric inflammatory bowel disease: highlighting pediatric differences in IBD. Gastroenterol Clin North Am. 2009 Dec;38(4):611-28.
Grossman AB, Mamula P. Crohn’s Disease. eMedicine.
Available from the Internet at http://emedicine.medscape.com/article/928288-overview (rev. 10/8/2009, cited 6/1/2010).
Markowitz JE, Mamula P. Ulcerative Colitis. eMedicine.
Available from the Internet at http://emedicine.medscape.com/article/930146-overview (rev. 9/11/2009, cited 6/1/2010).
Benor S, Russell GH, Silver M, Israel EJ, Yuan Q, Winter HS. Shortcomings of the Inflammatory Bowel Disease Serology 7 Panel. Pediatrics. 2010 May 3.
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital