ISSN:
1432-1076
Keywords:
Methylmalonic acidaemia
;
Propionic acidaemia
;
Metronidazole
;
Gut bacteria
;
Propionate
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Gut bacteria have been implicated as an important source of propionate in children with inborn errors of propionate metabolism. We have investigated the value of oral metronidazole (10–20 mg/kg per day) in five children with methylmalonic acidaemia (MMA) and four with propionic acidaemia (PA). Urinary excretion of propionate metabolites fell significantly during the treatment in all subjects, the mean decrease being 41% (range 12–76,P〈0.01), while mean plasma propionate was reduced from 45.0 μmol/l to 25.1 μmol/l (P〈0.05). Substantial reduction of the gut bacterial population was confirmed by lactulose breath hydrogen tests and by stool culture, and stool propionate concentration was reduced in most subjects. Clinical improvement was noted in three children. These results suggest that long-term antimicrobial therapy may offer significant clinical benefit to children with inborn errors of propionate metabolism.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01957284
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