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  • 1
    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
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  • 2
    ISSN: 1432-1076
    Keywords: Key words Metabolic decompensation ; Double-labelled water ; Resting energy expenditure ; Preterm ; infant ; Methylmalonic acidaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The assessment of energy expenditure is valuable for the management of children with various conditions such as obesity and failure to thrive. Total daily energy expenditure (TDEE) includes resting energy expenditure (REE), energy expenditure during physical activity, dietary thermogenesis and growth. TDEE can be assessed by using the double-labelled water technique, but it has complex pitfalls and potential sources of errors and is impractical for everyday use. As REE is a substantial part of TDEE (65%–70%) and computerised indirect calorimeters have become recently available, this non-invasive, relatively cheap and easy to use technique is valuable for the assessment of short-term changes in energy metabolism. This can be used to assess REE of children with inborn errors of metabolism, whilst well and during episodes of metabolic decompensation and therefore to accurately determine energy intake.
    Type of Medium: Electronic Resource
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  • 3
    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
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 148 (1989), S. 344-348 
    ISSN: 1432-1076
    Keywords: Methylmalonic acidaemia ; Chronic renal failure ; Tubulo-interstitial nephritis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The renal function of 12 patients with non vitamin B12 responsive methylmalonic acidaemia has been investigated. Eight patients had reduced glomerular filtration rates, but the plasma creatinine concentration was only raised in those with values of less than 40ml/min per 1.73m2 surface area. The reduction in glomerular filtration was a function of the age and the severity of the disease. Plasma urate concentrations were increased in four patients but this may be secondary to the renal disease rather than its cause.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Environmental biology of fishes 28 (1990), S. 33-75 
    ISSN: 1573-5133
    Keywords: Sharks ; Rays ; Chimaeras ; Chondrichthyes ; Evolution ; Success ; Diversity ; Ecology ; Ecomorphotypes ; Reproductive modes ; Conservation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Synopsis Cartilaginous fishes, the sharks, rays and chimaeras (class Chondrichthyes), are a very old and successful group of jawed fishes that currently contains between 900 and 1100 known living species. Chondrichthyians show a high morphological diversity during most of their evolutionary career from the Paleozoic to the present day. They are relatively large predators which have remained a major, competitive element of marine ecosystems despite the varied rivalry of numerous other marine vertebrate groups over at least 400 million years. Although restricted in their ecological roles by morphology, reproduction and other factors, the living cartilaginous fishes are highly diverse and show numerous alternative life-history styles which are multiple answers to exploiting available niches permitted by chondrichthyian limitations. Chondrichthyians living and fossil can be divided into at least eighteen ecomorphotypes, of which the littoral ecomorphotype is perhaps the most primitive and can serve as an evolutionary origin for numerous specialist ecomorphotypes with benthic, high-speed, superpredatory, deep-slope and oceanic components. Reproductive modes in cartilaginous fishes are of six types, ranging from primitive extended oviparity through retained oviparity and yolk-sac viviparity (previously ovoviviparity) to three derived forms of viviparity. Reproductive modes are not strongly correlated with ecomorphotypes and with the phylogeny of living elasmobranchs. The success and importance of cartilaginous fishes is largely underrated by marine biologists and by the public, and requires new and ‘heretical’ emphasis to overcome the present inadequacies of chondrichthyian research and the problems of overexploitation that cartilaginous fishes face.
    Type of Medium: Electronic Resource
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