Diurnal variability of glucose tetrasaccharide (Glc₄) excretion in patients with glycogen storage disease type <scp>III</scp>

Abstract

The extent and variability of Glc₄ excretion relative to creatinine was not correlated with cornstarch dose. A majority of collections showed low variability over 24 hours. These findings support the use of single time-point collections to evaluate Glc₄ in patients with GSD III treated with cornstarch. However, repeat sampling over short time-periods will provide the most accurate assessment of Glc₄ excretion, as intraday variability may be increased in patients with high Glc₄ excretion.