I have over the years received various opinions on the right regimen for dextrose IV in LCFAOD.
What is the consensus as to the more appropriate in LCFAOD eg LCHAD, VLCAD
It would be great to learn the INFORM scientists consensus on dextrose conc. and dosage – if there is one.
Most of us are on regiments, including myself, that state d10 at maintenance or 1.5x, maintenance: the UK crowd do similar via 2ml per kg per hr.
D10 at 1 – 1.5x maintenance / 2ml per kg per hr OR Use d20 to provide calories equivalent to BMR + PAL
Seems to be divide between whether to treat adults by providing enough calories to stop catabolism and prevent fasting VS providing high calories as priority.
Would have thought latter approach risks more stress e.g hyperglycemia, lyte derangements, ? Need for thiamine.
The European mainland countries’ scientists intend to work on guidelines.
Both INFORM and the American College of Genetics are currently working on guidelines to help standardize this practice.
You are correct that our number one concern during a metabolic episode is to induce an anabolic state. Typically, that means
some combination of oral or IV fluids. In the US, as you suggest, we use a quick and dirty calculation
to give 10% glucose at 1.5 x maintenance rate. Most patients do not become hyperglycemic with such and infusion and there
are no other real risks of the therapy. We always follow electrolytes and add them to the IV as needed. Thiamine has no role.
Hopefully, we’ll all get the guidelines done soon. The INFORM guideline will ultimately be available on the INFORM Families page.
I hope this helps.
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