Document Detail

Respiratory aspects of Shwachman's syndrome in adults.
MedLine Citation:
PMID:  2731608     Owner:  NLM     Status:  MEDLINE    
Shwachman's Syndrome is a rare disorder which causes considerable morbidity in childhood and can be confused with cystic fibrosis. Six patients (4 males; mean age 27 yrs) are described in order to illustrate the clinical picture of the syndrome in adults. Recognized features persisting in later life include pancreatic insufficiency and bone marrow dysfunction (all patients), short stature (5/6), advanced dental caries (4/6), and impaired glucose tolerance (2/6). Chest radiograph was normal in all patients whilst lung function tests showed mild restriction in three and obstruction in two. All patients were cyclically neutropenic (nadir count 2.1 x 10(9).l-1). Four of the six patients were thrombocytopenic and three had persistent immunoglobulin deficiencies. Neutrophil chemotaxis was abnormal in four patients and the nitro blue tetrazolium test was abnormal in a fifth. Two patients suffered from recurrent respiratory infections, one of whom died from bone marrow aplasia. Although Shwachman's Syndrome may be less troublesome in adults than in children, many of the abnormalities persist into adult life and may continue to cause diagnostic difficulty.
J Wiggins; D M Geddes
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The European respiratory journal     Volume:  2     ISSN:  0903-1936     ISO Abbreviation:  Eur. Respir. J.     Publication Date:  1989 Mar 
Date Detail:
Created Date:  1989-07-18     Completed Date:  1989-07-18     Revised Date:  2013-05-23    
Medline Journal Info:
Nlm Unique ID:  8803460     Medline TA:  Eur Respir J     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  285-8     Citation Subset:  IM    
Brompton Hospital, London.
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MeSH Terms
Body Height
Bone Marrow Diseases / physiopathology*
Pancreatic Diseases / physiopathology*

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