title: Pediatric inflammatory bowel disease: specificity of very early onset.
abstract: The incidence of inflammatory bowel disease (IBD) has increased over the last 50 years. It is now recognized that several genetic defects can express an IBD-like phenotype at very early onset (<6 years).
  
  
  
  The aim of this review was to update knowledge concerning the specificity of IBD at onset <6 years, which can include conventional/standard IBD as well as monogenic IBD-like diseases. Expert commentary: We found that females are less prone than males to develop monogenic disorders, which have X-linked heritability in several cases. Furthermore, the Crohn's Diseases (CD) subtype seems to be suggestive of monogenic disorders while Unclassified IBD (IBDU) subtype is predominantly found in conventional/standard IBD at onset <6 years. Isolated colonic location is prevalent in both the subsets of IBD at onset <6 years if compared to IBD at later onset. Monogenic disorders require more aggressive medical and surgical treatments and can be complicated by the occurrence of lymphomas.
authors.affiliation: a Artificial Nutrition Unit and Hepato-Metabolic Disease Unit , Pediatric Hospital Bambino Gesu , Rome , Italy.
authors.forename: Teresa
authors.initials: T
authors.lastname: Capriati
authors.affiliation: b Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Sabrina
authors.initials: S
authors.lastname: Cardile
authors.affiliation: b Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Bronislava
authors.initials: B
authors.lastname: Papadatou
authors.affiliation: c Department of Pediatrics , University of Messina , Messina , Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Romano
authors.affiliation: b Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Daniela
authors.initials: D
authors.lastname: Knafelz
authors.affiliation: b Hepatology, Gastroenterology and Nutrition Unit , Bambino Gesù Children's Hospital , Rome , Italy.
authors.forename: Fiammetta
authors.initials: F
authors.lastname: Bracci
authors.affiliation: a Artificial Nutrition Unit and Hepato-Metabolic Disease Unit , Pediatric Hospital Bambino Gesu , Rome , Italy.
authors.forename: Antonella
authors.initials: A
authors.lastname: Diamanti
mesh_terms: D017668:Age of Onset; D000818:Animals; D002675:Child, Preschool; D003106:Colon / Q000276:immunology*; D020022:Genetic Predisposition to Disease; D006801:Humans; D015994:Incidence; D015212:Inflammatory Bowel Diseases / Q000453:epidemiology* / Q000235:genetics; D010641:Phenotype; D015995:Prevalence
keywords: IBD-like; Inflammatory bowel disease; early onset; pediatric; pediatric-onset