Methylmalonic Acidaemia and Propionic Acidaemia

What is Methylmalonic Acidaemia and Propionic Acidaemia?

Methylmalonic Acidaemia (MMA) and Propionic Acidaemia (PPA) are disorders of propionate metabolism.

The disease can present itself in a variety of forms from a severe presentation to a much milder form.  Symptoms will vary from individual to individual and include drowsiness, rapid breathing and vomiting.  A spell in hospital is often necessary to stabilise the infant/child and the hospital team will give you information and guidance on how to manage your child’s condition.

The aim of dietary management is to limit production of propionate by restricting methionine, threonine, valine and isoleucine by means of a low protein diet. Protein requirements are met by supplementing the diet with a methionine, threonine, valine and isoleucine free amino acid mixture.

Carnitine is used as it may be effective in encouraging urinary propionyl-carnitine excretion and preventing carnitine depletion. Metranidazole is often prescribed to limit propionate production by gut bacteria.

Emergency regimes are required in periods of decompensation and catabolism.

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