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a Hôpital Robert Debré, Paris, France:
Centre for Clinical Investigation, b Biochemistry Laboratory, c National Institute for Health and
Medical Research, Paris, France
Correspondence to: Dr Guy Touati, Centre d'Investigation Clinique, Hôpital Robert Debré, 48 Bd Sérurier, 75019 Paris, France.
Accepted 4 September 1996
OBJECTIVE
To assess the prevalence of in vivo
detectable abnormalities of lactate metabolism in mitochondrial disorders.
DESIGNRetrospective study in a metabolic
investigation unit.
PATIENTS28 patients with a respiratory chain
disorder identified from biochemical or genetic analyses, or both, and
133 age matched controls. Controls were children in whom causes of
secondary hyperlactataemia and/or disorders, affecting the energy
pathways could be excluded.
METHODSLactate and pyruvate were measured in
blood, together with other intermediary metabolism indices, before and
one hour after four meals each day. Lactate and creatinine in a 24 hour
urine sample collected at the same time were analysed. When basal
hyperlactataemia was not evident, an intravenous glucose or pyruvate
loading test was performed as a provocative test.
RESULTSAbnormal lactate metabolism was found in
25 of 28 patients thus demonstrating the potential usefulness of these
investigations in the diagnosis of mitochondrial diseases. Moderate
lactate accumulation was present in relatively mild disease, associated
with a mitochondrial DNA mutation and combined respiratory complexes
deficiency. By contrast, high lactate concentrations were observed in
very young children, with severe disease, isolated complex deficiency,
and no apparent mitochondrial DNA defect.
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