Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
1992-7-1
pubmed:abstractText
Magnetic resonance spectroscopy (MRS) is a useful tool for detecting in vivo metabolic status and in vitro analysis of chemical metabolites. We performed MRS on a 15 year-old mentally retarded male patient with recurrent myoglobinuria due to phosphoglycerate kinase (PGK) deficiency. Muscle histochemistry was normal, although electron microscopy showed a significant increase in glycogen content. Biochemical analysis using muscle specimens revealed profound reduction of PGK activity to 4.4% of the control mean. In vivo MRS using 31P revealed a significant accumulation of sugar phosphates after ischemic forearm exercise, although no significant rise of inorganic phosphate was demonstrated during exercise. 1H MRS using the perchloric acid extracts of the blood taken at the ischemic forearm test, revealed no rise of venous lactate but a significant reduction of alanine levels. These data suggest that, unlike the patients with a glycogenolytic block, our patient with a distal glycolytic block shows a significant accumulation of sugar phosphates. The reason of the reduction of alanine levels was uncertain. A compensatory mechanism for energy supply to the muscle tissue was considered.
pubmed:language
jpn
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
0029-0831
pubmed:author
pubmed:issnType
Print
pubmed:volume
24
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
244-9
pubmed:dateRevised
2008-12-25
pubmed:meshHeading
pubmed:year
1992
pubmed:articleTitle
[1H and 31P magnetic resonance spectroscopy study of a case of phosphoglycerate kinase deficiency].
pubmed:affiliation
Department of Pediatrics, Hamamatsu University School of Medicine.
pubmed:publicationType
Journal Article, English Abstract