IBD Case of the Month:Pediatric Diagnosis of IBD Developed by the CCFA Nursing Initiatives Committee Author: Kristin Madden, NP University of Nebraska Medical Center Children’s Hospital & Medical Center
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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.
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.
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?
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 pyodermagangrenosum. 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?
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
Previous Workup • Radioallergosorbenttest (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
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
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
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
Laboratory Results • CBC – unremarkable • ESR 53 (h), CRP 0.7 • AST - 345/ALT – 925 (h) • GGT – 446 (h) • ANA – negative • SMA – 48 (+) • IgG – 1405 (h) • ANCA – 1:80 (+) • Fecal Calprotectin1207 (h) • Acute Hepatitis Panel (-) • Stool Culture, C-diff and Adeno (-) • AFP – 4 • CPK – 116 • Urine organic/serum amino (-) • Scopes (pathology) – Cecum, transverse, descending and ascending colon with focal acute colitis. • Liver biopsy (pathology) – Dense portal inflammation with cholangiolar proliferation. Cholangitis. Portal fibrosis with bridging fibrosis Stage III/IV.
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.
What is your plan of care? • Treat colitis with Sulfasalazine 10mg/kg TID (maintenance dose for >2 years is 30-50 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
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.
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