Lookup NU author(s): Dr Jane Salotti,
Dr Kevin Windebank,
Professor Judith Rankin
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BACKGROUND: The aetiology of Langerhans cell histiocytosis (LCH), a rare cancer-like disorder of the immune system, is largely unknown although a genetic component has been suggested based on familial cases, and reports of chromosome instability and genetic mutation. Associations between various cancers and congenital anomalies have been reported and although congenital anomalies have been noted in children with LCH only one study to date has reported their frequency. An association between congenital anomalies and LCH may suggest a common aetiological pathway, in particular, a genetic pathway. METHODS: Data from two coterminous registries in the same geographic region were used. All cases of LCH on the Northern Region Young Persons Malignant Disease Register diagnosed between 1985-2010 were cross-matched with live born cases of congenital anomaly registered by the Northern Congenital Abnormality Survey. RESULTS: 819,890 children and young people were born during 1985-2008. 13,799 (1.7%) had a congenital anomaly and 39 (0.005%) were diagnosed with LCH. Three LCH cases were identified amongst those with congenital anomalies, all three of whom had congenital heart disease. The relative risk of LCH for those with a congenital anomaly, compared with those without, was 4.74 (95% CI: 1.46-15.36, p=0.03). CONCLUSION: LCH was associated with congenital anomaly in a small but statistically significant number of patients, raising the possibility of a common genetic pathway in some cases.
Author(s): Salotti JA, Tennant PWG, Windebank K, Rankin J
Publication type: Article
Publication status: Published
Journal: Birth Defects Research Part A: Clinical and Molecular Teratology
Online publication date: 24/02/2015
Acceptance date: 05/12/2014
ISSN (print): 1542-0752
ISSN (electronic): 1542-0760
Publisher: John Wiley & Sons, Inc.
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