Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2013 Mar;43(5):589-92.
doi: 10.1007/s00247-012-2564-2. Epub 2012 Nov 24.

Characteristic scapular and rib changes on chest radiographs of children with ADA-deficiency SCIDS in the first year of life

David Manson  1 , Lauren Diamond, Kamaldine Oudjhane, Faisal Bin Hussain, Chaim Roifman, Eyal Grunebaum
Affiliations

Characteristic scapular and rib changes on chest radiographs of children with ADA-deficiency SCIDS in the first year of life

David Manson et al. Pediatr Radiol. 2013 Mar.

Abstract

Background: We describe radiographic changes in the ribs and scapulae seen in the first 6 months of life in children with ADA (adenosine deaminase) deficiency severe combined immundeficiency syndrome (SCIDS). We suggest that these changes are reversible with appropriate enzyme replacement therapy.

Objective: The purpose of this study was to describe characteristic rib and scapular radiographic changes in infants with ADA-deficiency SCIDS.

Materials and methods: This was a retrospective review of chest radiographs of nine children with ADA-deficiency SCIDS performed in the first year of life by two experienced pediatric radiologists. A control cohort of unaffected children was used for comparison.

Results: All children with ADA-deficiency SCIDS manifested unusual scapular spurring and anterior rib cupping. None of the control children manifested these changes.

Conclusion: Characteristic and reversible scapular and rib changes in the correct clinical setting should suggest an early diagnosis of ADA deficiency, prompting appropriate diagnostic and therapeutic measures.

PubMed Disclaimer

References

    1. Immunodefic Rev. 1990;2(3):175-98 - PubMed
    1. Clin Genet. 2003 Jun;63(6):522-9 - PubMed
    1. Pediatr Radiol. 1991;21(6):447-8 - PubMed
    1. J Pediatr. 1976 Nov;89(5):737-42 - PubMed
    1. Pediatr Radiol. 1997 May;27(5):447-51 - PubMed

Substances

Supplementary concepts

LinkOut - more resources