Medical Care
Treatment may be provided on an outpatient basis.
Cataracts that do not regress or disappear with therapy may require hospitalization for surgical removal.
Surgical Care
Cataracts may require surgical removal.
Consultations
See the list below:
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Biochemical geneticist
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Nutritionist
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Ophthalmologist
Diet
Diet is the foundation of therapy. Elimination of lactose and galactose sources suffices for definitive therapy.
Because soy formulas are typically poor calcium sources, ensure adequate calcium intake through dietary supplements.
Many lactose-free foods are known to contain free galactose; counsel from a skilled nutritionist may help a patient avoid these items.
Under kosher dietary law, all foods labeled kosher must also be identified as meat, dairy, or pareve (neither meat nor dairy). Thus, shopping for foods from the kosher meat and pareve food categories exclusively can simplify purchasing food.
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UDP-galactose synthesis and galactosemia. The most common form of galactosemia is due to a deficiency of galactose-1-phosphate uridyltransferase (GALT). This enzyme normally uses galactose-1-phosphate derived from dietary galactose. In the absence of GALT, galactose-1-phosphate accumulates, along with excessive galactose and its oxidative and reductive products galactitol and galactonate (not shown). UDP-galactose synthesis may also be impaired in the absence of GALT but not completely because UDP-galactose-4′-epimerase (GALE) can form UDP-galactose from UDP-glucose and can supply the donor to galactosyltransferases required for normal glycoconjugate biosynthesis.
