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
Permalink
