HEMOGLOBIN A1IN GALACTOSEMIA, A POSSIBLE ROLE IN MONITORING DIETARY COMPLIANCE

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Abstract
The finding of elevated HbA1 levels in 2 galactosemic infants of 24.1% and 23.8% (normal 6.3-8.2%) led to an assessment of the effects of dietary management in galactose-1-phosphate uridyl transferase deficiency and galactokinase deficiency. Exclusion of dietary galactose and lactose in the 2 propositi resulted in a reduction of HbA1 levels to normal in 1 and towards normal in the other. HbA1 was measured in 12 treated subjects with transferase deficiency, 7 carriers and 1 child with galactokinase deficiency. Five transferase deficient children had elevated HbA1 levels, and 4 of these agreed to a 6 wk strict dietary exclusion of galactose and lactose. This trial resulted in a fall in HbA1 levels in all subjects (mean 12.6% to mean 9.2%). HbA1 levels may be of value in assessing and ensuring dietary compliance in galactosemia.