PP57 MORTALITY AND MORBIDITY OF PAEDIATRIC LIVER TRANSPLANTATION PERFORMED AT EARLY AGES

cerevisiae (ASCA) and anti-neutrophil cytoplasmic (ANCA) antibodies were not increased. Abdominal ultrasound was normal. Computerized bowel tomography showed an abnormal terminal ileum and cecum with thickened bowel wall. These findings were confirmed by a duoble-contrast barium enema. The esophagogastroduodenoscopy was normal. Colonoscopy and videocapsule endoscopy showed intestinal multiple mucosal ulcerations, which were compatible with Crohn’s disease. The histopathology of the colon and terminal ileum showed inflammation extending through the muscolaris propria, with a predominance of plasma cells, lymphocytes, and eosinophils, but a paucity of neutrophils and no granuloma (probably caused by the impaired neutrophil extravasation). On the basis of clinical history, endoscopy, and extraintestinal manifestations (arthritis, ulcerative stomatitis), a Crohn’s disease was diagnosed and a course of prednisone (2 mg/kg/day) and mesalamine (70 mg/kg/day) was started and ibuprofen was discontinued. One month later the patient experienced a recurrence with abdominal pain, diarrhea, arthritis and worsening anemia. Thus prednisone and mesalamine were reducted and terapy with Infliximab was started. After the first infusion, her bowel and extraintestinal manifestations completely cleared up. After 6 months of treatment the child is still in clinical remission and no complications have appeared. To the best of our knowledge, gastrointestinal problems in LAD-1 consists of a few case reports including Hirschsprung disease, necrotizing enterocoitis, and chronic enterocolitis. The only previous report of IBD-like illness in LAD-1 was in a severely affected patient with a complitely resolution after bone marrow transplantation. In conclusion, disorders of leukocyte adhesion should be included in the differential disgnosis of pediatric Crohn’s disease, particularly in the presence of recurrent infections. Therapy with Infliximab appears to be effective in these patients.