#Clinic reduces GA1 brain injury risk by 83% with therapies developed over 30 years
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“#Clinic reduces GA1 brain injury risk by 83% with therapies developed over 30 years”
Before the CSC’s founding in 1989, 90% of infants and young children with GA1 suffered a catastrophic form of acute neurological degeneration. The brain injury of GA1 leaves children mute, wheelchair-dependent, and fully disabled by generalized dystonia, and often results in complications such as scoliosis, hip dislocation, pulmonary aspiration, chronic pain, and untimely death.
Today, with the benefit of early diagnosis, dietary therapy, and an effective hospital protocol, only 7% of children born with GA1 suffer brain injury. Specifically, state newborn screening coupled with strict dietary management reduces the risk of brain injury 14-fold, and uninjured children with GA1 have normal growth, motor development, and cognitive function. Overall, early diagnosis of GA1 with lysine-free, arginine-enriched metabolic formula and emergency IV infusions during the first two years of life is safe and effective—preventing over 90% of brain injuries. The need for dietary and emergency IV therapies beyond early childhood is uncertain at this time.
30-year study identifies need of disease-modifying therapies for maple syrup urine disease
Kevin A. Strauss et al, Glutaric acidemia type 1: Treatment and outcome of 168 patients over three decades, Molecular Genetics and Metabolism (2020). DOI: 10.1016/j.ymgme.2020.09.007
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Clinic reduces GA1 brain injury risk by 83% with therapies developed over 30 years (2020, October 14)
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