1. IBD Case of the Month: Pediatric Diagnosis of IBD
Developed by the Crohn’s & Colitis Foundation Nurse & Advanced Practice Committee
Author: Kristin Madden, NP
University of Nebraska Medical Center
Children’s Hospital & Medical Center

2. Instructions
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3. Objectives
Identify ‘red flag’ symptoms and how to order labs/diagnostics to lead you to diagnosis.
Identify radiologic, laboratory, and more invasive methods of testing for diagnosis of IBD.

4. Introduction/Background
November 2011: a 21 month old female presents to clinic with chief complaint of hematochezia.
History includes 6-8 weeks of loose stools (4-5x/day) with visible mucus and bright red blood. No recent laboratory tests have been conducted.

5. What additional information will be helpful?
What is the family history?
Is there important birth history?
Is the review of systems revealing of additional factors?

6. Review of Systems (ROS) What is important & why
General: pertinent negatives – no recent travel, no recent antibiotics. This is important to evaluate as we need to consider infectious etiologies to the presenting symptoms.
Skin: no eczema, no erythema nodosum or pyoderma gangrenosum. These findings are supportive evidence for allergic vs inflammatory or autoimmune diseases.
ENT: Determine if there are any additional chronic disease processes or mouth sores that could support Crohn Disease.
Respiratory: Any chronic cough, asthma or pneumonias that would indicate aspiration or compromised immune system?
Cardiovascular: Rule out chronic disease of heart, hypertension, etc.
GU: Rule out anatomical issues or urinary reflux.
Muscular/Skeletal: Is there hypotonia, developmental delay or syndromic appearances?
Hematologic/Lymphatic: Easy bruising/bleeding present (liver disease)? Any enlarged lymph nodes?
Neurologic: Headaches or irritability present?
Endocrine: Are there current Autoimmune diseases present increasing risk for GI Autoimmune Disease?

7. Physical Exam Vitals: Temp – 36.5, Pulse – 109, Resp – 30, BP – 94/69
Growth: Head Cir – 45.7, Height – 77.6, Weight – 9.75, Weight for Length – 35.75%
General: alert, no distress
Head: normocephalic
Eyes/Ears/Nose/Throat: sclera clear, conjunctiva pink, nose clear, throat clear, without oral lesions
Neck: supple, no masses
Lungs: clear to auscultation bilaterally
CV: regular rate and rhythm, no murmur, equal pulse and cap refill<3 sec
Abdomen: soft, nondistended, nontender, no organomegaly, normal bowel sounds, no masses/hernia/guarding. Liver edge palpable 2-3 cm below right costal margin
Skin: No eczema and no skin rash noted.
Musculoskeletal: No reported joint pain or stiffness

8. Previous Workup
Radioallergosorbent test (RAST) positive for cows milk allergy
Complete blood count (CBC) – normal/no anemia
Liver enzymes (AST/ALT) – 614/832; elevated
Erythrocyte Sedimentation Rate (ESR) – 64; elevated inflammatory marker
C-reactive Protein (CRP) – 11.1; elevated inflammatory marker
Fecal occult blood – positive

9. Do you have red flags/cause for concern based on physical exam & previous workup?
No concern
Only minimal concern
Significant concern
Major concern indicating need for admission

10. Do you have a Differential Diagnosis?
Autoimmune hepatitis or Primary Sclerosing Cholangitis
Celiac Disease
Constipation
Crohn's Disease
Functional Abdominal Pain
Immune Deficiency
Infection
Irritable Bowel Syndrome
Metabolic Disease
Milk +/- soy protein allergy
Ulcerative Colitis

11. What would be ordered for workup?
Allergy testing
Blood work
Capsule endoscopy
CT enterography or MR enterography
Liver Biopsy
pH probe
Upper endoscopy and colonoscopy
Stool studies
Upper GI
Nothing

12. Laboratory Results
Scopes (pathology) – Cecum, transverse, descending and ascending colon with focal acute colitis.
CBC – unremarkable
ESR 53 (h), CRP 0.7
AST - 345/ALT – 925 (h)
Liver biopsy (pathology) – Dense portal inflammation with cholangiolar proliferation. Cholangitis. Portal fibrosis with bridging fibrosis Stage III/IV.
GGT – 446 (h)
ANA – negative
SMA – 48 (+)
IgG – 1405 (h)
ANCA – 1:80 (+)
Fecal Calprotectin 1207 (h)
Acute Hepatitis Panel (-)
Stool Culture, C-diff and Adeno (-)
AFP – 4
CPK – 116
Urine organic/serum amino (-)

13. What is your Diagnosis?
Autoimmune liver disease based on liver biopsy results: Dense portal inflammation with cholangiolar proliferation. Cholangitis. Portal fibrosis with bridging fibrosis Stage III/IV.
Cholangitis can be seen in Autoimmune or Primary Sclerosing Cholangitis.
Inflammatory Bowel Disease – likely ulcerative colitis based on pathology: Cecum, transverse, descending and ascending colon with focal acute colitis. Prefer to see both chronic and acute inflammation. But will start treatment based on these results.

14. What is your plan of care?
Treat colitis with Sulfasalazine 10mg/kg TID
(maintenance dose for >2 years is mg/kg/day)
Prednisolone 1 mg/kg BID
Azathioprine 1 mg/kg (after obtaining TPMT enzyme activity level +/- genetics)
Follow clinical response to treatment and laboratory response to treatment

15. Summary
In this case study it is important to complete a workup and not be distracted by the young age of the patient or the history of milk protein allergy as an explanation for blood in the stool.
If workup had not been completed it could have been easy to miss colitis and liver disease.

16. Thank you!
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