Galactosemia medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dayana Davidis, M.D. [2]
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Overview
Medical Therapy
The only proven to be efficient treatment for galactosemia is elimination of all galactose containing foods, this would mean limiting all dairy products to zero and all traces of dairy in other foods.
Immediate stopping of all galactose containing foods in individuals with GALT enzyme activity of less than 10% and whose red blood count or RBC galactose-1-phosphate is greater than 10mg/dl. All infant formulas should be replaced with soy formulas that contain sucrose, fructose and non galactose polycarbohydrates. Elimination of galactose containing foods should be a lifelong treatment.
After early life and childhood, milk becomes a less important part of life and hence managing the galactose free diet, however at this stage it was noted that despite avoidance of exogenous galactose, the production of endogenous galactose results in an increase of the enzyme gal-1-P. It is due to ingestion of foods contaminated with some dairy such as breads, biscuits, soups, cereals etc.
Individuals with the Duarte variant form of galactosemia do not need to be strict about galactose elimination, however they do need to avoid milk and ingest moderate amounts of galactose.
Continued monitoring of the accumulation of toxic RBC gal-1-P and urinary galactitol should be carried throughout life. If sudden increases are detected, other tests and evaluations should be carried especially for infection and sepsis. Ophtalmologic examination, developmental evaluation and speech development focusing should be done