Document Detail

Frequent myocardial lesions in Shwachman's syndrome. Eight fatal cases among 16 Finnish patients.
MedLine Citation:
PMID:  6485783     Owner:  NLM     Status:  MEDLINE    
A total of 16 children with Shwachman's syndrome were studied over a period of 17 years. Eight cases were detected in autopsy at 6 to 15 months; all had died of cardiac failure due to myocardial lesions. The left ventricles showed necrosis of myofibres in large areas and the pancreas was atrophic and replaced by adipose tissue. The other eight patients included two siblings of deceased cases. Only one of these showed transient cardiac failure and no significant nutritional deficiencies were found. They had steatorrhoea due to failure of the exocrine pancreas and either constant (6 cases) or cyclic (2 cases) neutropenia. The steatorrhoea improved with age. Pyogenic infections, mainly otitis media were frequent during the first three years of life. Measurements of humoral and cell-mediated immunity were normal, but in addition to low numbers of neutrophils, the neutrophilic chemotaxis was depressed in all seven patients tested. Skin lesions, hepatic inflammation, and growth tended to improve with age. The family data of the patients is consistent with an autosomal recessive trait inheritance.
E Savilahti; J Rapola
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Acta paediatrica Scandinavica     Volume:  73     ISSN:  0001-656X     ISO Abbreviation:  Acta Paediatr Scand     Publication Date:  1984 Sep 
Date Detail:
Created Date:  1984-11-21     Completed Date:  1984-11-21     Revised Date:  2009-11-11    
Medline Journal Info:
Nlm Unique ID:  0000211     Medline TA:  Acta Paediatr Scand     Country:  SWEDEN    
Other Details:
Languages:  eng     Pagination:  642-51     Citation Subset:  IM    
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MeSH Terms
Agranulocytosis / complications*
Cardiomyopathies / etiology*,  mortality,  pathology
Celiac Disease / etiology
Exocrine Pancreatic Insufficiency / complications*,  genetics,  pathology
Infant, Newborn
Neutropenia / complications*

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