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  • 1
    Electronic Resource
    Electronic Resource
    The use of metronidazole in management of methylmalonic and propionic acidaemias (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 792-796 
    Details
    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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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    The contribution of protein catabolism to metabolic decompensation in 3-hydroxy-3-methylglutaric aciduria (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 346-350 
    Details
    ISSN: 1432-1076
    Keywords: 3-Hydroxy-3-methylglutaric aciduria ; Protein ; Fat ; Stable isotope ; Decompensation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Leucine and protein metabolism were studied using stable isotope techniques in 6-year-old twins with 3-hydroxy-3-methylglutaric aciduria during acute metabolic decompensation. The decompensation was preceded by prolonged fasting in twin 1 and by an upper respiratory infection in twin 2. Twin 2 was also studied when well (control study). During infection, leucine oxidation (36 μmol/kg per hour), protein catabolism (6.0 g/kg per day) and urinary excretion of major leucine metabolites (104 μmol/kg per hour) were all increased compared with the control study (16 μmol/kg per hour, 4.7 g/kg per day and 28 μmol/kg per hour respectively). During fasting, leucine oxidation (18 μmol/kg per hour) was unchanged and protein catabolism (4.1 g/kg per day) was decreased despite substantially increased urinary metabolite excretion (87 μmol/kg per hour) compared with the control study. These results indicate that protein mobilisation and leucine oxidation played important roles in metabolic decompensation during infection but not during fasting. It is likely that the increased metabolite excretion during fasting arose primarily from fatty acid catabolism, indicating the importance of this substrate in metabolic decompensation in 3-hydroxy-3-methylglutaric aciduria.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02171564
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    In vivo propionate oxidation as a prognostic indicator in disorders of propionate metabolism (1990)
    Springer
    European journal of pediatrics 149 (1990), S. 408-411 
    Details
    ISSN: 1432-1076
    Keywords: Propionate ; Stable isotope ; Oxidation ; Methylmalonic acidaemia ; Propionic acidaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Biochemical markers such as plasma and urinary metabolite concentrations and in vitro enzyme activity are of limited prognostic value in the most common disorders of propionate metabolism, methylmalonic acidaemia (MMA) and propionic acidaemia (PA). In vivo propionate oxidation was compared with conventional prognostic measures as predictors of clinical severity in seven children with MMA and six with PA. Propionate oxidation was measured using a continuous infusion of [1-13C]propionate and was expressed as the rate of appearance of13CO2 as a percentage of the propionate infusion rate. Children with MMA (mean oxidation 51.2%, range 17.5–91.6,P〈0.05) and with PA (mean oxidation 36.3%, range 3.0–91.1,P=NS) oxidised substantially less propionate than controls (mean oxidation 81.9%, range 69.4–101.0,n=5). Percentage oxidation was a better predictor of the clinical severity score (r=0.75,P〈0.01) than was in vitro enzyme activity, plasma propionate or methylmalonate concentration or urinary metabolite excretion. Studies were repeated after an interval of 1–3 weeks in six of the subjects; the percentage oxidation in each subject was virtually unchanged between studies (coefficient of variation 8.6%). These results suggest that in vivo oxidation measurements using [13C]propionate are both reproducible and prognostically useful in disorders of propionate metabolism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02009661
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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