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Correlation between blood ammonia concentration and organic acid accumulation in isovaleric and propionic acidemia



Correlation between blood ammonia concentration and organic acid accumulation in isovaleric and propionic acidemia



Pediatrics 69(1): 115-117



In 7 patients with propionic acidaemia there was perfect correlation between blood ammonia concentration and serum propionic acid, whatever the onset of disease, with an increase of ammonia concentration of about 160 mu mol/mmol serum propionic acid. Usually ammonia concentrations were higher in the neonatal period (up to 1 mmol/litre) but that also corresponded to greater accumulation of propionic acid, attributed to the high protein catabolism in newborn infants.

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Accession: 000848802

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PMID: 7054746


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