OAc Concept

The dietary management dietary management consists of a diet low in Methionine, Valine, Threonine and Isoleucine (i.e. native protein), which are metabolic precursors of propionyl CoA. Such low protein diet requires the addition of protein supplements free from these amino acids.

Organic Acidemias / Acidurias

Organic acidemias are inherited metabolic disorders, related to the metabolic pathways of amino acids, carbohydrates, and fatty acids. The most common organic acidemias are propionic acidemia (PPA) and methylmalonic acidemia (MMA). Enzyme defects in degradation of propionyl CoA or methylmalonyl CoA lead to accumulation of these metabolites in the body. The severity of symptoms varies widely, ranging from mild neurological disturbances to metabolic crisis and even death.


Dr. Schär AG 

Winkelau 9
I - 39014 Burgstall / Postal

Tel. +39 0473 293 300
Fax +39 0473 293 399




Aminosäurenmischung | diätetische Behandlung von Phenylketonurie | erstes Lebensjahr | Erwachsene | glutenfrei | HPA | Hyperphenylalaninämie | Jugendliche | Kinder | Kleinkinder | Kohlenhydrate | Mineralstoffe | phenylalaninfreie | Proteinsupplemente | Phenylketonurie | PKU | Schwangere |Spurenelemente | Säuglinge | Vitamine


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