Document Detail

Erythrocyte transketolase activity in suspected cases of Leigh's disease, or subacute necrotising encephalomyelopathy.
MedLine Citation:
PMID:  7436444     Owner:  NLM     Status:  MEDLINE    
Erythrocyte transketolase activity and the effect of adding thiamine pyrophosphate (% thiamine pyrophosphate effect) were measured in 111 subjects suspected to suffer from Leigh's disease (subacute necrotising encephalomyelopathy). From clinical evidence these subjects were divided into five groups: (1) necropsy-proved cases of subacute necrotising encephalomyelopathy, (2) cases positive for urinary thiamine pyrophosphate: adenosine triphosphate phosphotransferase inhibitor, (3) clinically likely cases of subacute necrotising encephalomyelopathy (patients still alive, or on whom no necropsy was performed), (4) cases diagnosed as diseases other than subacute necrotising encephalomyelopathy (control group), (5) cases for which no diagnosis had been made. Comparison of erythrocyte transketolase activities with and without added thiamine pyrophosphate and of the % thiamine pyrophosphate effect for each group compared with the control group showed no statistically significant differences from normal values for any of these parameters. Similarly, there were no differences between the two sexes in transketolase activity, and no correlation between transketolase activity and age. These results indicate that erythrocyte transketolase activity is not altered in subacute necrotising encephalomyelopathy and is unlikely to be of value for the diagnosis of Leigh's disease.
A McBurney; D Leigh; H McIlwain
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Archives of disease in childhood     Volume:  55     ISSN:  1468-2044     ISO Abbreviation:  Arch. Dis. Child.     Publication Date:  1980 Oct 
Date Detail:
Created Date:  1981-01-16     Completed Date:  1981-01-16     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  0372434     Medline TA:  Arch Dis Child     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  789-94     Citation Subset:  AIM; IM    
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MeSH Terms
Brain Stem*
Encephalomalacia / blood,  enzymology*,  genetics
Erythrocytes / enzymology*
Thiamine Pyrophosphate
Transketolase / antagonists & inhibitors,  blood*
Reg. No./Substance:
154-87-0/Thiamine Pyrophosphate; EC

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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