Further Outpatient Care
Evaluate patients with galactokinase deficiency on a regular and frequent schedule to assess dietary intake and to perform eye examinations.
Inpatient & Outpatient Medications
Medication is unnecessary.
Complications
Dietary indiscretions resulting in small, frequent doses of galactose encourage initial or recurrent cataract formation.
Prognosis
The prognosis is excellent. The disorder has no implications for any organ other than the lens. Problems can be eliminated with proper care.
Patient Education
Affected individuals must learn to master the dietary restrictions required to eliminate galactose ingestion.
Patients should undergo an annual slit-lamp examination to detect cataract formation.
For excellent patient education resources, visit eMedicineHealth's Eye and Vision Center. Also, see eMedicineHealth's patient education article Cataracts.
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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.
