title: Long-term outcome of intractable ulcerating enterocolitis of infancy.
abstract: BACKGROUND
  Chronic enterocolitis is rare in infancy and accounts for less than 0.5% of all newly diagnosed inflammatory bowel disease (IBD) in the UK. Presentation at this young age is usually indicative of underlying immunodeficiency/immunodysregulation. A group of such infants suffer intractable ulcerating enterocolitis of infancy (IE) in which there is a pan-enteritis with marked oro-anal involvement and deep flask like mucosal ulcers throughout the colon.
  
  
  METHODS
  Retrospective review of presenting features, treatment and long-term outcome in a series of 8 children with typical IE.
  
  
  RESULTS
  The 8 children were aged between 1 and 4 weeks at onset (median 2 weeks, mean 2.3 weeks), of which 7 were followed up for 2-22 years (median 7.5 years, mean 11 years). All 8 children had an intractable disease course requiring a colectomy for control of symptoms. The median age at colectomy was 1.7 years (range 4 months-4 years). Three children developed a generalised lymphadenopathy due to uncontrolled EBV-related lymphoid proliferations (ages 4, 12, 18). These comprised a monomorphous B-lymphoycte lympho-proliferative disorder, a large pleomorphic follicular lymphoma, and a high grade pleomorphic B cell non-Hodgkin's lymphoma.
  
  
  CONCLUSIONS
  Infants with IE have a high risk of developing lymphomatous proliferations that appears to be related to the underlying immunodysregulation. Use of aggressive immunosuppression and acquisition of EBV infection appears to accelerate this process; hence we advocate early colectomy in confirmed cases. In children with IE screening for EBV and vigilance for abnormal lymphoid proliferations is paramount.
authors.affiliation: Gastroenterology Unit, Institute of Child Health and Great Ormond Street Hospital, London, UK. n.thapar@ich.ucl.ac.uk
authors.forename: Nikhil
authors.initials: N
authors.lastname: Thapar
authors.forename: Neil
authors.initials: N
authors.lastname: Shah
authors.forename: Alan D
authors.initials: AD
authors.lastname: Ramsay
authors.forename: Keith J
authors.initials: KJ
authors.lastname: Lindley
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Milla
mesh_terms: D000293:Adolescent; D000328:Adult; D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D003082:Colectomy; D003093:Colitis, Ulcerative / Q000150:complications / Q000188:drug therapy* / Q000601:surgery*; D016572:Cyclosporine / Q000627:therapeutic use; D004760:Enterocolitis / Q000150:complications / Q000188:drug therapy* / Q000601:surgery*; D020031:Epstein-Barr Virus Infections / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D007231:Infant, Newborn; D008228:Lymphoma, Non-Hodgkin / Q000453:epidemiology; D008232:Lymphoproliferative Disorders / Q000453:epidemiology; D008297:Male; D013256:Steroids / Q000627:therapeutic use; D013792:Thalidomide / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007136:Immunoglobulins; D007166:Immunosuppressive Agents; D013256:Steroids; D013792:Thalidomide; D016572:Cyclosporine; D001379:Azathioprine