نتایج جستجو برای: propionic acidemia
تعداد نتایج: 4552 فیلتر نتایج به سال:
BACKGROUND Chronic pain is a complex phenomenon resulting from biological, psychological and social factors, and the use of patient-centred care (PCC) appears to be a promising avenue for its treatment. Various methods have been used for measuring PCC in nurses and physicians (caregivers); however, methodological problems have been raised following the observation of real clinical encounters or...
We isolated bacteriophages active against Propionibacterium freudenreichii from 16 of 32 swiss cheese samples. Bacteriophage concentrations ranged from 14 to 7 x 10(5) PFU/g, depending on the sample and the sensitive strain used for detection. Only a few strains, 8 of the 44 strains of P. freudenreichii in our collection, were sensitive. We observed that multiplication of bacteriophages occurre...
The reaction of propionyl isothio-cyanate with valine was found to give the title compound, C(8)H(15)NO(3), instead of the expected thio-urea product. The whole mol-ecule is non-planar and the carbonyl group is cis to the methyl-butanoic acid group across the C-N bond. Inter-molecular O-H⋯O and N-H⋯O hydrogen bonds build up a two-dimensional network developing parallel to (100).
The structure of the side chain of the 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-C29 dicarboxylic bile acid occurring in body fluids of infants with coprostanic acidemia was investigated by means of mass spectrometry and nuclear magnetic resonance spectroscopy. The findings identified this bile acid as 3 alpha, 7 alpha, 12 alpha-trihydroxy-27a, 27b-dihomo-5 beta-cholestane-26,27b-dioic acid ...
Kocuria kristinae is a catalase-positive, coagulase-negative, Gram-positive coccus found in the environment and in normal skin and mucosa in humans; however, it is rarely isolated from clinical specimens and is considered a nonpathogenic bacterium. We describe a case of catheter-related bacteremia due to K. kristinae in a young adult with propionic acidemia undergoing periodic hemodialysis. The...
United States legislatures are debating whether to use tandem mass spectrometry to expand the roster of inherited disorders tested in newborn screening programs. The debate is hampered because published financial data comparing charges associated with late vs early diagnosis are not readily available. We provide pilot financial data comparing late diagnosis vs presumptive diagnosis and early ma...
Propionic acidemia (PA) is a rare autosomal recessive disorder of metabolism caused by deficient activity of the mitochondrial enzyme propionyl-CoA carboxylase. The clinical manifestations are metabolic acidosis, poor feeding, lethargy, vomiting, osteoporosis, neurological dysfunction, pancytopenia, developmental retardation and cardiomyopathy. Liver transplantation has recently been considered...
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