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

Thapar N,Shah N,Ramsay AD,Lindley KJ,Milla PJ

doi

10.1097/01.mpg.0000159622.88342.bc

subject

Has Abstract

pub_date

2005-05-01 00:00:00

pages

582-8

issue

5

eissn

0277-2116

issn

1536-4801

pii

00005176-200505000-00010

journal_volume

40

pub_type

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