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  • 1995-1999  (2)
  • DSC  (1)
  •  Inborn errors of metabolism  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 156 (1997), S. 382-383 
    ISSN: 1432-1076
    Keywords: Key words Malonyl coenzyme A decarboxylase deficiency  ;  Cardiomyopathy  ;  Inborn errors of metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A new case of mitochondrial malonyl coenzyme A decarboxylase deficiency is described. The patient presented with an initial episode of metabolic acidosis, seizures, hypoglycemia, and cardiac failure at 2 months of age which slowly resolved. Subsequent evaluations at 4 years of age for developmental delay revealed a prominent elevation of malonic acid in urine. Malonyl carnitine was also elevated. The activity of malonyl CoA decarboxylase in cultured fibroblasts was 7% of normal. Conclusion Malonyl CoA decarboxylase deficiency may result in inhibition of fatty acid oxidation, which may account for the cardiomyopathy.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1435-1536
    Keywords: Key words Poly(ethylene-co-5.4 mol% 3 ; 5 ; 5-trimethylhexyl methacrythlate) ; poly(dimethyl-siloxane) ; miscibility ; permeability ; dynamic elastic measurement ; DSC
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Notes: Abstract  Blends of poly(ethylene- co-5.4 mol% 3,5,5-trimethylhexyl methacrylate) (PE-TMHM) with poly(dimethylsiloxane) (PDMS) were prepared in the PDMS content range from 0 to 20%. The miscibility was studied for PE-TMHM–PDMS blends by DSC, dynamic mechanical and microscopic spectroscopy, and the gas permeability was measured for O2, N2 and CO2 as function of PDMS content. PE-TMHM and PDMS were partially miscible with each other below 20 wt% of PDMS content. The permeability coefficients (P) for O2, N2 and CO2 were increased by the blending of PDMS to PE-TMHM. The change of P for O2 with PDMS content well reflected the partially miscible phase separation behavior.
    Type of Medium: Electronic Resource
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