Glutaric acidemia type 1 (GA1)

Glutaric acidemia type 1 (GA1)

In this metabolic disorder, the amino acids, lysine and tryptophan, normal components of all animal and plant proteins, cannot be processed normally in the body. As a consequence, metabolic products can accumulate that are toxic for the brain. Children with the condition tend not to have any symptoms as a newborn. If left untreated, most children with the condition will have a larger head than normal and over time will suffer from developmental delay as well as certain movement disorders.Between the ages of 3 months and 3 years, acute metabolic crises occur, often triggered by minor infections, which can lead to permanent movement difficulties and disability.

GA1 can be well managed with a special diet and by taking l-carnitine supplements. To prevent metabolic crises, minor infections during the first years of life should be always be treated as an emergency.

 

 
Therapy: 
  • GA1 can be well managed with a special diet and by taking l-carnitine supplements.
  • To prevent metabolic crises, minor infections during the first years of life should be always be treated as an emergency. 
 
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