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Early-Onset Inflammatory Bowel Disease

  • Neslihan Edeer Karaca
  • Guzide Aksu
  • Necil Kutukculer
Chapter

Abstract

Incidence of inflammatroy bowel disease (IBD) is growing among adults and children all over the world. Approximately 20% of patients with IBD are diagnosed before the age of 16 years

Early-onset, i.e. diagnosed before 5 years, and very-early-onset, i.e. diagnosed before 1 years, types of IBD (EO-IBD and VEO-IBD) have severe and therapy-resistant courses

The majority of VEO-IBD cases are caused by monogenic defects

Defects in IL-10 and IL-10 receptor, leading to defective STAT3 dimerization, have shown to cause severe dysregulation of the immune system, resulting in VEO-IBD with perianal disease

Key to a effective therapy lies in early diagnosis. Positive family history can lead to early diagnosis and curative treatment with HSCT should be emphaszied

Patients with IL-10/IL-10R defects usually present with early-onset enterocolitis, and severe perianal diseases such as fissures, abscesses or fistula, associated with other clinical features including chronic folliculitis, recurrent respiratory diseases and arthritis

Abscess formation, anal fissures, and enterocutaneous or rectovaginal fistulas can complicate EO-BID and frequently require surgical intervention such as our patient

Patients with IL-10/IL-10R- deficiency have the risk of developing B cell lymphoma

Early diagnosis of IL-10/IL-10R is crucial, especially in patients with early-onset enterocolitis, severe perianal diseases and other clinical features including chronic folliculitis and recurrent respiratory

Early HSCT has high positive impact in reducing morbidity and mortality in VEO-IBD patients

Keywords

Inflammatroy bowel disease IL-10 STAT3 VEO-IBD 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Neslihan Edeer Karaca
    • 1
  • Guzide Aksu
    • 1
  • Necil Kutukculer
    • 1
  1. 1.Ege University Faculty of Medicine, Department of Pediatric ImmunologyBornovaTurkey

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