inflammatory bowel disease in a pediatric population. J Pediatr Gastroenterol Nutr. Nov; 51(5):603-9. 2) Villa C, Pompili G, Franceschelli G, Munari A, Radaelli G, Maconi G, et al. Role of magnetic resonance imaging in evaluation of the activity of perianal Crohn’s disease. Eur J Radiol. Apr; 81(4):616-22. P-163 Compound Heterozygous IL10RA Mutations in Brothers with Very Early Onset Inflammatory Bowel Disease Julia Bracken, Darrel Dinwiddie, Kathy Christenson, Charles Roberts, Carol Saun- ders, Sarah Soden, Neil Miller, Stephen Kingsmore Childrens Mercy Hospital and Clinics, Kansas City, MO, USA BACKGROUND: Background: Interleukin 10 receptor (IL10R) mutations have been reported to cause aggressive inflammatory bowel disease (IBD) in children (1). Very Early Onset IBD (VEO-IBD) is known to be associated with severe disease, poor response to therapy and increased morbidity and mortality. CMH00165 and CMH00166 are brothers who both presented with early in life Crohn’s Disease (CD), thus far refractory to medical management. CMH00165 is a 4 year old male who presented at 12 months of age with perirectal abscess, failure to thrive, hematochezia and skin rash. CMH00166 is a 1 year old male who presented at 4 months of age with oral lesions, anemia, failure to thrive and skin rash. Immunol- ogy evaluation for the pair was unremarkable. Skin biopsy revealed neutrophilic dermatitis and GI mucosal biopsies confirmed diagnosis of Crohn’s disease. Medi- cal therapy including azathioprine, infliximab, methotrexate, adalimumab, and, finally tacrolimus has failed to adequately control disease. Poor therapeutic response resulted in corticosteroid dependence, two bowel resections in the older child, failure to thrive, nutritional insufficiency requiring intermittent paren- teral nutrition and repeated hospitalizations. METHODS: Methods: Given the severe and early onset phenotype of disease in a sibling pair, the family consented to exome sequencing at the Center for Pediatric Genomic Medicine (CPGM) at Children’s Mercy Hospital. DNA isolation was per- formed on blood specimens from the children and both unaffected parents. Exome enrichment was conducted with the Illumina TruSeq Exome v1 (62.2 meg- abase) kit and sequenced on an Illumina HiSeq 2000 to a depth of >8 gigabases per sample. Base calls(1 x 100 nucleotides) were generated with Illumina RTA 1.12.4.2 & CASAVA-1.8.2, aligned to the human genome reference NCBI 37 using Genomic Short-read Nucleotide Alignment Program (GSNAP), and variants were detected using the Genome Analysis Tool Kit (GATK)3. Variants were characterized with the CPGM’s Rapid Understanding of Nucleotide variant Effect Software (RUNES v1.0)3. RESULTS: Results: Both affected children were found to have compound heterozy- gous mutations in the Interleukin 10 Receptor A. Each parent was found to be a carrier of a single gene mutation. Variant 1: IL10RA, c.784C>T, p.Arg262Cys has been previously reported in VEO-IBD. Variant 2: IL10RA, c.349C>T, p.Arg117Cys is a novel variant affecting a conserved amino acid, and is predicted to be deleterious. CONCLUSION(S): Conclusions: We present a novel IL10RA variant and a case of compound heterozygosity in the IL10 receptor leading to early and aggressive IBD. This is the second family in which exome sequencing of an affected child or children and healthy parents was useful for molecular diagnosis of VEO-IBD4. Given previously reported success with allogeneic bone marrow transplantation in children with IL10R mutations2, this family will be presented with additional therapeutic options. P-164 Early Infliximab Trough Levels Predict Remission at One Year in Pediatric IBD Patients Casey Rosenthal, Gil Melmed, Bhavna Tripuraneni, Jennifer Gebbia, Silvia Callejas, Sharmayne Farrior, Shervin Rabizadeh, Marla Dubinsky Cedars Sinai Medical Center, Los Angeles, CA, USA BACKGROUND: Among patients with IBD, detectable trough concentrations of infliximab (IFX) during maintenance dosing are associated with response to ther- apy. Moreover the presence of anti-infliximab antibodies (ATI) increases drug clearance and loss of response. It is unknown whether IFX and ATI levels at the completion of induction dosing predicts long term efficacy of maintenance ther- apy. We determined whether week 14 trough IFX levels (IFX14) and ATI levels (ATI14) were associated with week 54 outcomes in pediatric IBD patients. METHODS: A prospective cohort of pediatric IBD patients receiving IFX were tested for IFX and ATI levels at both weeks 14 and 54 or at early termination. Pri- mary non-responders (NR) were patients who stopped drug before week 14 and early terminators were patients who stopped drug between weeks 14-54. Primary outcome was week 54 clinical remission (CR) : PCDAI <10 for Crohn’s disease or partial Mayo <2 points and no sub-score >1 for ulcerative colitis and the ab- sence of a dose or frequency intensification beyond 5 mg/kg q 8 weeks prior to week 54. Secondary outcomes were deep remission (DR): clinical remission with normal CRP, sustained durable remission (SDR): CR at every maintenance infusion (week 14-54) and week 54 IFX (IFX54) and ATI (ATI54) levels. Univariate analyses were used to determine associations between IFX14 and ATI14 and week 54 out- comes, as appropriate. Regression tree analysis was used to determine the opti- mal IFX14 cutoff level associated with remission. IFX and ATI ELISA testing were performed at Prometheus labs (San Diego, CA). RESULTS: A total of 38 patients (median age: 12.6 yrs.) were enrolled of whom 4 were primary NR. 2/4 primary NR had detectable ATI levels and 3/4 had undetect- able IFX levels before week 14. Of the 34 who entered maintenance at week 14, 3 patients discontinued IFX prior to week 54. Of the remaining 31 patients, 20 met criteria for both clinical and deep remission and 14 for SDR. ATI were detected in 3/ 34 (8%) at week 14 and 7/34 (20%) at week 54 or at early termination. 4/7 patients developed ATI after week 14. Week 14 IFX trough level was associated with CR (P ¼ 0.009), DR (P ¼ 0.009) and SDR (p ¼ 0.04). The optimal cut point for IFX trough level at week 14 to predict DR was 5.5 lmL (p ¼ 0.01). IFX14 was associated with IFX54 (P ¼ 0.02) and inversely associated with ATI54 (P ¼ 0.003). ATI14 was associ- ated with ATI54 (P ¼ 0.004) and inversely associated with IFX54 (P ¼ 0.06) but was not associated with any of the remission outcomes. CONCLUSION(S): Infliximab levels at week 14 are associated with clinical, deep and sustained durable remission at week 54, with a minimum trough level of 5.5 lg/mL strongly predictive of deep remission. Moreover, week 14 IFX trough levels correlate with both week 54 IFX and ATI levels. The presence of ATI at week 14 did not predict remission outcomes but did correlate with IFX and ATI levels at week 54. Assessment of infliximab levels at an early time point may be warranted to optimize dosing to maximize long term therapeutic benefit. Nursing Poster Presentations P-165 Learning from Transitional Care: Experiences and Recommendations of Young Adults Maureen Kelly, Ali Annaim, Paul Mihas, Michael Kappelman UNC-Chapel Hill, Chapel Hill, NC, USA BACKGROUND: There are few studies addressing the experience of transitional care in young adults with inflammatory bowel diseases (IBD). Most of the studies include patients with other chronic health problems, and are descriptive studies related to patient satisfaction regarding the experience, rather than studies based on clinical outcomes. Study Aim: To evaluate the experience of adolescents with IBD who have transitioned to adult care. METHODS: Former patients in the pediatric gastroenterology clinic at a tertiary care center were interviewed using a two-part telephone questionnaire. Twenty young adults completed a ten question scaled survey using a five point Likert scale. The responses were ranked from 1 to 5 (5 as the most positive response), and consisted of questions related to level of preparedness from the pediatric provider, independent research on the adult provider, level of preparedness before first appointment with the adult provider, communication, confidence in ability to manage disease independently, understanding of disease and plan before transition, satisfaction with experience, as well as preferred method of education. Eighteen of the participants also agreed to complete an open-ended questionnaire. This portion of the interview included questions related to the patient’s knowledge of their disease, medication, adherence, nutrition, self-man- agement skills, knowledge of reproductive health, attending college or starting a job, health insurance, ongoing adult support, and finding a new heath care provider. RESULTS: The young adults described an overall positive transition experience with a mean of 4 (very satisfied) on the Likert scale. The results of the open- ended questions revealed six overarching themes and better reflected the com- plexity of the transition process. These themes included: 1) reaction to transition spanning a wide range of experiences from hand holding to guided discovery to complete independence; 2) relationship with healthcare providers, including the ability to listen and discuss sensitive topics; 3) knowledge including information about the treatment plan and the transition process itself; 4) emotions ranging from anxiety about their diet to helplessness when hospitalized; 5) communica- tion including what occurs between the young adult and the pediatric provider, as well as between the pediatric and adult provider; and 6) recommendations which included the need to do research on their own during the transition pro- cess, not only on their medical condition, and resources available to them, but also on the background of their adult provider. CONCLUSION(S): Obtaining only quantitative data on the adolescent transitioning process is not sufficient to capture the experience. Many of the participants in this study remember what they consider a smooth transition, but even those individuals made recommendations to improve the process. Open-ended 2012 IBD Abstracts S81