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Transorgan fluxes in a Porcine model reveal a central role for liver in acylcarnitine metabolism

Severe PGBH does not respond to any dietary or medical intervention Step 4: Obtain a mixed meal tolerance test or a provocation meal test If dietary changes and an alpha-glucosidase inhibitor do not prevent postprandial hypoglycemia from recurring, then confirmation of PGBH is needed, using a mixed meal tolerance test or a provocation meal test

Symptomatology can be absent and liver and/or neurologic function decline may be the only diagnostic clue of infection

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