P-085: Resolution of factor XI (FXI) deficiency in refractory ulcerative colitis (UC) after surgery: a case report

Abstract

s of the 3rd International Symposium on Pediatric Inflammatory Bowel Disease S425 (perianal disease, ileal and/or colonic stenosis with or without abscess with respective resections). In multivariate analysis, no association with age, gender, inflammatory and serologic markers was detected. Conclusion: IBD is rare in our country, however extensive forms predominate and rates of surgery are high, especially in CD (40% by the final visit), with important resections, reflecting the severity of IBD. Family history and positivity of ANCA and ASCA are low; therefore, other factors have to be involved in this aggressive phenotype. P-084 Eosinophilic gastroenteropathy from diet to colectomy A. Karoliny1 *, J.B. Kovacs1, A. Nagy1, N. Lasztity1, K. Meichelbeck1, L. Karsza1, G. Hegyi1, B. Lestar2, G. Veres3, M. Lorincz1. 1Pal Heim Children’s Hospital, Budapest, Hungary, 2Hungarian Defence Forces Med. Center, Dept. of Surgery, Budapest, Hungary, 31st Dept. of Pediatrics, Semmelweis University, Budapest, Budapest, Hungary Background: Incidence of eosinophilic gastrointestinal disorders is increasing worldwide, however, precise pathomechanism, diagnostic criteria and therapy, in severe cases, is not well known. While there are well documented publications and guidelines about the clinical manifestation and treatment of eosinophilic oesophagitis the incidence, clinical manifestation and treatment of primary eosinophilic gastroenterocolitis is less well known. Case report: A 4-year-old male patient was presented with massive haematochesia and diarrhoea. Upper and lower endoscopy showed gastritis, duodenitis and ulcerative pancolitis, respectively. Histology confirmed eosinophilic gastroenteropathy (>100 eosinophils/HPF in duodenum and colon). 5-ASA, systemic corticosteroid, leukotriene receptor antagonist and azathioprine treatment combined with elimination diet did not achieve remission. Due to further deterioration and increased transfusion demand nasogastric feeding with amino acid based formula was introduced. After two years of unsuccessful conventional therapy infliximab was given, but due to severe bloody diarrhoea total colectomy was needed. After 12 months of follow-up, the patients is symptom free, growing well with no hematochezia. None of the upper and lower endoscopies, biopsies, and colectomy specimens showed any signs of Inflammatory Bowel Disease (IBD), but high number of eosinophils. Conclusion: IBD and eosinophilic colitis may share a common phenomenon resulting differencial diagnostic difficulties. Here, we presented a patient with severe form of eosinophilic gastroenteritis causes life-threatening hematochezia. Biological treatment and colectomy for this indication is rarely indicated. P-085 Resolution of factor XI (FXI) deficiency in refractory ulcerative colitis (UC) after surgery: a case report A. Angelakopoulou1 *, K. Lindley2, M. Mathias2, S. McCartney3, A. Windsor3, F. Kiparissi1. 1Great Ormond Street Hospital NHS Trust, London, United Kingdom, 2Great Ormond Street Hospital, London, United Kingdom, 3University College London Hospital NHS Trust, London, United Kingdom Introduction: Coagulation abnormalities in acute inflammatory conditions such as sepsis are well recognised. Chronic inflammatory conditions, such as Inflammatory Bowel Disease, have the potential to induce a coagulopathic or procoagulant state with potential devastating consequences. Aim: To report the resolution of severe FXI deficiency in severe UC post colectomy and restorative surgery. Methods: We present the case of a 14-year-old male with severe UC, FXI deficiency and an acute, thrombotic cerebrovascular event. Results: The patient presented with two-week history of headache, vomiting, altered consciousness and left leg weakness, one year after diagnosis of severe UC refractory to combination immunosuppressive therapy. MRI changes in keeping with Right Middle Cerebral Artery ischaemic stroke. Cerebral/visceral angiography excluded systemic vasculitis. Investigations revealed APTT of 67.3s (26 38s) and low FXI (13 IU/dl; 50 150). Platelet count showed a drop post stroke (lowest 76) with subsequent gradual recovery. Congenital FXI deficiency was excluded by patient genetic and parental FXI testing. Lifelong aspirin anticoagulation was commenced. No neurological sequelae were observed. UC remained uncontrolled despite escalation to non-conventional immunosuppression; he underwent subtotal colectomy and ileostomy formation with good results. Lowest FXI levels were documented pre-operatively (8 IU/dl) and treated with FXI concentrate. FXI showed spontaneous normalisation within 2 years of surgery (FXI >50 IU/dl) with no further complications. Conclusion: Clinicians should recognise the haematological implications of UC, especially in cases refractory to medical therapy. In our patient, FXI deficiency was secondary to uncontrollable gastrointestinal inflammation and normalised after definitive surgical treatment. P-086 Total colectomy in pediatric ulcerative colitis: a cohort study from a tertiary center in Greece K. Dimakou, S. Fessatou, M. Maragoudaki, I. Panayotou, E. Roma-Giannikou*, G. Chouliaras. University of Athens, “Aghia Sophia” Children’s Hospital, Athens, Greece Introduction: Pharmacological interventions may induce and maintain clinical remission in children with ulcerative colitis (UC). Nevertheless a significant proportion of these patients may, ultimately, undergo total colectomy (TC) due to refractory disease. Aim: To assess the observed rate of colectomy in pediatric UC in Greece. Patients were classified according to period of diagnosis (1981 1989, 199