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Propionic acidemia
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==Management== Patients with propionic acidemia should be started as early as possible on a low protein diet. In addition to a protein mixture that is devoid of methionine, threonine, valine, and isoleucine, the patient should also receive [[L-carnitine|<small>L</small>-carnitine]] treatment and should be given antibiotics 10 days per month in order to remove the intestinal propiogenic flora. The patient should have diet protocols prepared for them with a "well day diet" with low protein content, a "half emergency diet" containing half of the protein requirements, and an "emergency diet" with no protein content. These patients are under the risk of severe hyperammonemia during infections that can lead to comatose states.<ref>Saudubray JM, Van Der Bergh G, Walter J : Inborn Metabolic Diseases Diagnosis and Treatment (2012)</ref> Liver transplant is gaining a role in the management of these patients, with small series showing improved quality of life.{{cn|date=October 2024}}
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