Knew those genetics courses would come in handy some day.  MSUD (Maple sugar
urine disease) is a rare (1:200,000), autosomal recessive inborn error of
metabolism.  THe three amino acids affected are leucine, isoleucine and
valine.  Symptoms are maple sugar scented urine, seizures and developmental
problems.  Treatment is restriction of the three amino acids in question.
        Mature human milk contains from 1 to 6 micromoles/deciliter of these
three amino acids.  Sounds low to me.  You could compare these levels (the
individual levels are in Lawrence, p.110) with those in the abm manufactured
for this condition.  The infant could be monitored for blood levels  of
leucine, isoleucine and valine, and human milk decreased in favor of a food
free of these amino acids, if one exists, until a balance is reached.  This is
the same way PKU is handled in a breastfed infant.
Let us know what else you find out.
Catherine Watson Genna, IBCLC  NYC  [log in to unmask]