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Pediatr Infect Dis J. 2005 Feb;24(2):179-81.

Infections in patients with Shwachman-Diamond syndrome.

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General Pediatrics Division, Massachusetts General Hospital for Children, Children's Hospital Boston, Boston, MA 02115, USA.


Shwachman-Diamond syndrome (OMIM 260400) is a multisystemic disorder characterized by pancreatic insufficiency, bone marrow dysfunction, skeletal abnormalities and immune dysfunction. Prompted by the case of a 13-year-old girl with Shwachman-Diamond syndrome who presented with pneumonia attributable to Pseudomonas aeruginosa, we review infectious complications of this disease. Pneumonia, recurrent otitis media and skin infections/abscesses constitute the majority of infections among these children.

[Indexed for MEDLINE]

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