Document Detail


Sea-blue histiocyte syndrome with bone anomalies.
MedLine Citation:
PMID:  757536     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Two sisters, now 29 and 24 years old, are described. They presented a congenital storage of, most probably, phospholipids in the histiocytes of the sea-blue type or blue pigmentophages. The granules of these cells showed a PAS positivity and strong positivity for acid phosphatase, but there were negative also for non-specific esterase, naphthol-AS-D-chloracetate esterase and iron acid also for urine mucopolysaccharide. The two cases differed from all the described cases in the absence of hepatosplenomegaly and in the presence of bone changes resembling late spondyloepiphyseal dysplasia or atypical dysostosis multiplex.
Authors:
J Janele; V Brychnác; V Lodrová
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Haematologia     Volume:  12     ISSN:  0017-6559     ISO Abbreviation:  Haematologia (Budap)     Publication Date:    1978-1979
Date Detail:
Created Date:  1980-05-30     Completed Date:  1980-05-30     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0130266     Medline TA:  Haematologia (Budap)     Country:  HUNGARY    
Other Details:
Languages:  eng     Pagination:  215-20     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Acid Phosphatase
Adult
Bone and Bones / abnormalities*,  radiography
Esterases
Female
Histiocytes / enzymology*
Humans
Niemann-Pick Diseases / enzymology*
Periodic Acid-Schiff Reaction
Phospholipids
Syndrome
Chemical
Reg. No./Substance:
0/Phospholipids; EC 3.1.-/Esterases; EC 3.1.3.2/Acid Phosphatase

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