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HEMOGLOBIN A 1 IN GALACTOSEMIA, A POSSIBLE ROLE IN MONITORING DIETARY COMPLIANCE
Author(s) -
HOWARD N. J.,
MONAGHAN H.,
MARTIN J. M.
Publication year - 1981
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1981.tb05770.x
Subject(s) - galactosemia , galactokinase , medicine , lactose , endocrinology , galactose , transferase , hemoglobin , biochemistry , enzyme , biology , escherichia coli , gene
. Howard, N. J., Monaghan, H. and Martin, J. M. (Departments of Paediatrics and Physiology, University of Toronto and Research Institute, The Hospital for Sick Children, Toronto, Canada). Hemoglobin A, in galactosemia, a possible role in monitoring dietary compliance. Acta Paediatr Scand, 70:695,.–The finding of elevated HbA, 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 HbA 1 levels to normal in one and towards normal in the other. HbA 1 was measured in twelve treated subjects with transferase deficiency, seven carriers, and one child with galactokinase deficiency. Five transferase deficient children had elevated HbA 1 levels, and four of these agreed to a six week strict dietary exclusion of galactose and lactose. This trial resulted in a fall in HbA 1 levels in all subjects (mean 12.6 % to mean 9.2 %). These results suggest HbA 1 levels may be of value in assessing and ensuring dietary compliance in galactosemia.