title: Variable outcome in infantile-onset inflammatory bowel disease in an Asian cohort.
abstract: OBJECTIVE
  Infantile-onset inflammatory bowel disease (IO-IBD) with the onset of disease before 12 mo of age, is a different disease entity from childhood IBD. We aimed to describe the clinical features, outcome and role of mutation in interleukin-10 (IL-10) and interleukin-10 receptors (IL-10R) in Asian children with IO-IBD.
  
  
  METHODS
  All cases of IO-IBD, defined as onset of disease before 12 mo of age, seen at University Malaya Medical Center, Malaysia were reviewed. We performed mutational analysis forIL10andIL10Rgenes in patients with presenting clinical features of Crohn's disease (CD).
  
  
  RESULTS
  Six [13%; CD = 3, ulcerative colitis (UC) = 2, IBD-unclassified (IBD-U) = 1] of the 48 children (CD = 25; UC = 23) with IBD have IO-IBD. At final review [median (range) duration of follow-up: 6.5 (3.0-20) years], three patients were in remission without immunosuppression [one each for post-colostomy (IBD-U), after standard immunosuppression (CD), and after total colectomy (UC)]. Three patients were on immunosuppression: one (UC) was in remission while two (both CD) had persistent disease. As compared with later-onset disease, IO-IBD were more likely to present with bloody diarrhea (100%vs55%,P= 0.039) but were similar in terms of an associated autoimmune liver disease (0%vs19%,P= 0.31), requiring biologics therapy (50%vs36%,P= 0.40), surgery (50%vs29%,P= 0.27), or achieving remission (50%vs64%,P= 0.40). No mutations in either IL10 or IL10R in the three patients with CD and the only patient with IBD-U were identified.
  
  
  CONCLUSIONS
  The clinical features of IO-IBD in this Asian cohort of children who were negative forIL-10orIL-10Rmutations were variable. As compared to childhood IBD with onset of disease after 12 mo of age, IO-IBD achieved remission at a similar rate.
authors.affiliation: Way Seah Lee, Ruey Terng Ng, Department of Paediatrics, University Malaya Medical Center, Kuala Lumpur 59100, Malaysia.
authors.forename: Way Seah
authors.initials: WS
authors.lastname: Lee
authors.affiliation: Way Seah Lee, Ruey Terng Ng, Department of Paediatrics, University Malaya Medical Center, Kuala Lumpur 59100, Malaysia.
authors.forename: Ruey Terng
authors.initials: RT
authors.lastname: Ng
authors.affiliation: Way Seah Lee, Ruey Terng Ng, Department of Paediatrics, University Malaya Medical Center, Kuala Lumpur 59100, Malaysia.
authors.forename: Koon-Wing
authors.initials: KW
authors.lastname: Chan
authors.affiliation: Way Seah Lee, Ruey Terng Ng, Department of Paediatrics, University Malaya Medical Center, Kuala Lumpur 59100, Malaysia.
authors.forename: Yu-Lung
authors.initials: YL
authors.lastname: Lau
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D044466:Asian People; D001688:Biological Products / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003082:Colectomy; D003093:Colitis, Ulcerative / Q000150:complications / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D003424:Crohn Disease / Q000150:complications / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D004252:DNA Mutational Analysis; D003967:Diarrhea / Q000453:epidemiology* / Q000209:etiology; D004750:Enteral Nutrition / Q000379:methods; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000453:epidemiology* / Q000209:etiology; D019693:Hepatitis, Autoimmune / Q000453:epidemiology* / Q000209:etiology; D006801:Humans; D007165:Immunosuppression Therapy; D007223:Infant; D007231:Infant, Newborn; D015212:Inflammatory Bowel Diseases / Q000150:complications / Q000453:epidemiology* / Q000235:genetics* / Q000628:therapy; D016753:Interleukin-10 / Q000235:genetics*; D008296:Malaysia / Q000453:epidemiology; D008297:Male; D009154:Mutation; D053701:Receptors, Interleukin-10 / Q000235:genetics*
chemical_list: D001688:Biological Products; C508609:IL10 protein, human; D053701:Receptors, Interleukin-10; D016753:Interleukin-10
keywords: Infantile-onset inflammatory bowel disease; Pediatric