Propionic acidemia (PA) and methylmalonic aicd (MMA) are branched-chain organic acidemias. An affected patient is at risk of acute deterioration if certain conditions occur. The authors are from Utrecht University in The Netherlands.

Episodes of acute decompensation start during early childhood.


Mechanism: catabolism


Triggers for a metabolic decompensation:

(1) acute trauma

(2) infection or fever

(3) excessive exercise

(4) any physiologic stress

(5) fasting

(6) vomiting

(7) relatively high protein intake

(8) medication

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