Glutaric Aciduria Type 1 (GA1) / Pyridoxine Dependent Epilepsy (PDE)


The ‘building blocks’ of protein are called amino acids. In GA1, due to the lack of an enzyme, the body cannot properly deal with two of the amino acids, lysine and tryptophan. People with GA1 therefore need to restrict the amount of protein they eat.

The management of GA1 may involve the use of protein substitutes. Protein substitutes for GA1 provide all the amino acids found in protein but no lysine and only a small quantity of tryptophan. As such, they provide a source of protein that is free from, or low in, the amino acids that cause problems in GA1. If used, protein substitutes are used alongside the individual’s allowance of regular dietary protein.

 

For Pyridoxine Dependent Epilepsy (PDE), despite adequate seizure control with pharmacological monotherapy, some patients with PDE suffer from significant developmental and intellectual issues.* For these patients, along with large doses of pyridoxine, best practice recommendations include a lysine restricted diet.* To maintain an adequate protein and micronutrient intake, lysine free protein substitutes are advised for patients with PDE managed on a low lysine diet.*

 

*References on file 

 


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Inborn Errors of Metabolism

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