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 Radioljournal_title
Pediatric radiologyauthors
Chippington S,McHugh K,Vellodi Adoi
10.1007/s00247-008-0811-3subject
Has Abstractpub_date
2008-06-01 00:00:00pages
657-60issue
6eissn
0301-0449issn
1432-1998journal_volume
38pub_type
杂志文章abstract:BACKGROUND:Reversed blood flow has been reported in the superior mesenteric artery (SMA) in 92% of healthy term newborns at 2 h of age. By 24 h after birth the end-diastolic velocity became positive in all of the infants. OBJECTIVE:To characterize hemodynamic changes in the coeliac artery and superior mesenteric arter...
journal_title:Pediatric radiology
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journal_title:Pediatric radiology
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更新日期:2013-09-01 00:00:00
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更新日期:2007-01-01 00:00:00
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更新日期:2000-12-01 00:00:00
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pub_type: 杂志文章,评审
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pub_type: 杂志文章,已发布勘误
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journal_title:Pediatric radiology
pub_type: 杂志文章,评审
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更新日期:2007-11-01 00:00:00
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