• Eur. J. Pediatr. · Dec 1993

    Contribution of odd-numbered fatty acid oxidation to propionate production in neonates with methylmalonic and propionic acidaemias.

    • U Wendel, R Zass, and D Leupold.
    • Children's Hospital, Heinrich-Heine Universität Düsseldorf, Germany.
    • Eur. J. Pediatr. 1993 Dec 1; 152 (12): 1021-3.

    AbstractNewborns with disturbed propionate metabolism (propionic and methylmalonic acidaemias) accumulate during fetal life large amounts of odd-numbered long-chain fatty acids in adipose tissue (8% to 10% of total fatty acids vs 1% in normal controls) and other body lipids. During periods of acute catabolism, such as in the early days of life, mobilization and oxidation of odd-chain fatty acids from adipose tissue yield extensive amounts of toxic propionyl-CoA in the mitochondria and thus might contribute to the severe illness of the patients. Our data suggest that not only catabolism of protein but also that of adipose tissue has to be avoided in order to alleviate the neonatal illness of patients with propionic and methylmalonic acidaemias. It seems prudent to avoid prolonged fasting during the 1st year of life.

      Pubmed     Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…