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Dilated cardiomyopathy in a case of Shwachman-Diamond syndrome.
MedLine Citation:
PMID:  21486516     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
The Shwachman-Diamond syndrome is an autosomal recessive bone marrow failure syndrome with exocrine pancreatic insufficiency. Additional organ systems, such as the liver, heart and bone, may also be affected. We report a patient with a long history of cardiac failure and diagnosis of dilated cardiomyopathy with intermittent neutropenia. Periodic follow-up revealed progressive cardiac failure and pulmonary hypertension. A diagnosis of Shwachman-Diamond syndrome was made at the autopsy.
Authors:
Liliane Kopel; Paulo S Gutierrez; Silvia G Lage
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-4-13
Journal Detail:
Title:  Cardiology in the young     Volume:  -     ISSN:  1467-1107     ISO Abbreviation:  -     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-4-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9200019     Medline TA:  Cardiol Young     Country:  -    
Other Details:
Languages:  ENG     Pagination:  1-3     Citation Subset:  -    
Affiliation:
1Critical Care Unit, Department of Cardiology, Heart Institute, School of Medicine, University of São Paulo, São Paulo, Brazil.
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