Monitoring - PA/MMA

The precursor amino acids (isoleucine, valine, threonine and methionine) do not accumulate in plasma in these disorders to it is not possible to measure levels to determine the accurate natural protein intake levels.¹

Management of the diet induces the risk of prescribing too high or too low levels of natural protein with consequences of inadequate growth, vomiting and metabolic decompensation.¹

Monitoring of nutritional status and suggested frequency^1,2,3
Laboratory monitoring	Plasma amino acids: Valine, Isoleucine, Threonine and Methionine
Diet history	Each clinic visit
Growth (weight, length/height, BMI)	Each clinic visit
Clinical examination (eg hair, skin)	Each clinic visit
Albumin, pre-albumin	Each clinic visit
Bone health (Calcium, phosphorus, alkaline phosphatase, magnesium, parthyroid hormone, vitamin D)	Annually, more frequently in case of chronic kidney disease
FBC, zinc, selenium, ferritin, folic acid, vitamin B12	Annually

References:

Dixon M, MacDonald A, White F, Stafford J. Disorders of Amino Acid Metabolism, Organic Acidaemias and Urea Cycle Disorders. In: Clinical Paediatric Dietetics. 4^th ed: Shaw V. West Sussex: John Wiley & Sons Limited, 2014:381-525: 19914636
Baumgartner et al. Proposed guidelines for the diagnosis and management of methylmalonic and propionic academia. Orphanet Journal of Rare Diseased, 2014;9:130.
Bernstein