title: Treatment of intractable ulcerating enterocolitis of infancy by allogeneic bone marrow transplantation.
abstract: OBJECTIVE
  Intractable ulcerating enterocolitis of infancy (IE) is an uncommon, autosomal-recessive, and devastating inflammatory bowel disorder that arises as a consequence of a poorly defined underlying immunologic disorder. Infants with IE suffer from recurrent severe oro-anal disease and an enterocolitis that is unresponsive to conventional immunosuppressive therapy and requires early pancolectomy to control the severity of the disease. Despite such aggressive treatment these individuals remain at high risk of Epstein-Barr virus-driven lymphomatous proliferations, including non-Hodgkin's lymphoma. The underlying genetic basis for this disease remains undefined. This report aims to describe the use of bone marrow transplantation as a treatment for this condition.
  
  
  METHODS
  This was a case series report.
  
  
  RESULTS
  We describe the successful treatment of IE by allogeneic bone marrow transplantation in 2 brothers, now aged 7 and 11 years, one of whom had developed an Epstein-Barr virus-related monomorphous B-lymphocyte lymphoproliferative disorder. This treatment has resulted in prolonged clinical remission in both boys and abrogated the need for aggressive immunosuppression.
  
  
  CONCLUSIONS
  Bone marrow transplantation can be used for the treatment of intractable ulcerating enterocolitis of infancy, which may support a role in other intractable inflammatory bowel conditions in the pediatric population.
authors.affiliation: Gastroenterology Unit, Great Ormond Street Hospital, London, United Kingdom. n.thapar@ich.ucl.ac.uk
authors.forename: Nikhil
authors.initials: N
authors.lastname: Thapar
authors.forename: Keith J
authors.initials: KJ
authors.lastname: Lindley
authors.forename: Fevronia
authors.initials: F
authors.lastname: Kiparissi
authors.forename: Mamoun A
authors.initials: MA
authors.lastname: Elawad
authors.forename: Michael
authors.initials: M
authors.lastname: Ashworth
authors.forename: Paul
authors.initials: P
authors.lastname: Veys
authors.forename: Hubert B
authors.initials: HB
authors.lastname: Gaspar
authors.forename: Susan M
authors.initials: SM
authors.lastname: Hill
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Milla
authors.forename: Neil
authors.initials: N
authors.lastname: Shah
mesh_terms: D016026:Bone Marrow Transplantation; D003082:Colectomy; D003106:Colon / Q000473:pathology; D000013:Congenital Abnormalities / Q000601:surgery / Q000628:therapy*; D020345:Enterocolitis, Necrotizing / Q000601:surgery / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male