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Date: | Mon, 18 Nov 1996 21:29:40 -0800 |
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Human milk would be a perfect source of cobalamin, it contains
methylcobalamin, a readily usable form. The problem with this particular
infant is that he is missing one of the enzymnes that reduce cobalamin
from the dietary 3+ state to the usable 1+ state. This means that he
cannot use the cobalamin he takes in. One of the important functions of
cobalamin is that it is converted into succinyl co-enzyme A, which is
essential for the breakdown of isoleucine, methoionine, valine and
threonine. Without cobalamin, isoleucine and valine yield homocystine;
and methylmalonic acid cannot be converted into succinyl coA, and builds
up in the bloodstream. Thus these kids have both homocystinuria and
methylmalonic aciduria. Not a good mileau for a growing brain.
What I was wondering specifically was whether the methylcobalamin
in human milk would help to bypass the missing enzyme, and whether the
levels of the 4 above amino acids would be low enough for this baby to
get at least some human milk, like babies with phenylketonuria (PKU).
Any takers?
--
Catherine Watson Genna, IBCLC NYC [log in to unmask]
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