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David Gray, Senior Specialist Dietitian at Guy’s and St Thomas’…
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Propionic acidaemia is a rare genetic disease characterised by incomplete metabolism of certain amino acids and, to a lesser degree, fats and cholesterol. The processing error causes propionic acid and other toxic substances to accumulate in the blood. Left untreated, these toxins damage the body’s organs especially the brain.
Propionic acidaemia varies in severity. Some individuals show only mild symptoms, while in others life-threatening complications such as stroke and coma occur. Progressive brain damage is a characteristic feature.
The amino acids that cause a problem in propionic acidaemia are isoleucine, valine, threonine and methionine. Amino acids are the building blocks of proteins. After eating proteins, the body ‘metabolises’ or breaks them down into amino acids. Animal proteins include dairy products, meat, eggs and fish. Proteins are also found in plants including soy, legumes, grains and nuts. The body uses the amino acids to make its own proteins essential for life – for example enzymes; structural proteins in muscles, hair, skin, cells and cartilage; proteins that generate movement in muscles; or those involved in cell functioning or immune responses. In periods of fasting or illness, the body often switches to use its own proteins, and stored fats, to generate energy. Leucine, isoleucine, valine and threonine are essential amino acids meaning that the body cannot make them. Therefore, these amino acids come from ingested protein or from the breakdown of previously ingested and stored proteins.
Propionic acidaemia has several other names. These include ‘propionic aciduria’ since high levels of propionic acid are also seen in the urine (-uria = in the urine); ‘propionyl-CoA carboxylase deficiency’ after the characteristic enzyme abnormality or ‘ketotic hyperglycinaemia’ describing the presence of keto acids and glycine in the blood (hyper = high; -aemia = in the blood). The disease shows many similarities to methylmalonic acidaemia.
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