Splenic nodules in paediatric Gaucher disease treated by enzyme replacement therapy.

Abstract:

BACKGROUND:The natural history of focal splenic lesions in paediatric Gaucher disease (GD) is unknown and these lesions are thought to persist despite enzyme replacement therapy (ERT). OBJECTIVE:To assess the prevalence, evolution and resolution of splenic nodules in a cohort of paediatric Gaucher patients treated with ERT. MATERIALS AND METHODS:The US findings in 37 children with GD were retrospectively reviewed. A total of 28 children underwent serial abdominal US examinations as part of their initial assessment and during routine follow-up. All patients received ERT. RESULTS:Six children (21%) had splenic nodules on US examination, either at presentation or on follow-up examination. In all six patients, the nodules had resolved on follow-up imaging, with resolution taking 17 months to 4 years 8 months. Two sets of siblings developed nodules that resolved over a similar time period. CONCLUSION:Disappearance of focal splenic lesions in children with GD during follow-up has not been previously reported. The development of new focal splenic lesions in children with GD whilst on ERT has not been previously documented.

journal_name

Pediatr Radiol

journal_title

Pediatric radiology

authors

Chippington S,McHugh K,Vellodi A

doi

10.1007/s00247-008-0811-3

subject

Has Abstract

pub_date

2008-06-01 00:00:00

pages

657-60

issue

6

eissn

0301-0449

issn

1432-1998

journal_volume

38

pub_type

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