Hemoglobin A1 in galactosemia, a possible role in monitoring dietary compliance.

The finding of elevated HbA1 levels in two 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 two propositi resulted in a reduction of HbA1 levels to normal in one and towards normal in the other. HbA1 was measured in twelve treated subjects with transferase deficiency, seven carriers, and one child te uridyl transferase deficiency and galactokinase deficiency. Exclusion of dietary galactose and lactose in the two propositi resulted in a reduction of HbA1 levels to normal in one and towards normal in the other. HbA1 was measured in twelve treated subjects with transferase deficiency, seven carriers, and one child te uridyl transferase deficiency and galactokinase deficiency. Exclusion of dietary galactose and lactose in the two propositi resulted in a reduction of HbA1 levels to normal in one and towards normal in the other. HbA1 was measured in twelve treated subjects with transferase deficiency, seven carriers, and one child with galactokinase deficiency. Five transferase deficient children had elevated HbA1 levels, and four of these agreed to a six week 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%). These results suggest HbA1 levels may be of value in assessing and ensuring dietary compliance in galactosemia.