Variation of urinary excretion of aspartylglucosamine and associated clinical findings in aspartyglucosaminuria.

The urinary excretion of aspartylglucosamine (AADG), the main accumulating glycoprotein degradation product in aspartylglucosaminuria (AGU), was studied in 40 patients at various stages of the disease. Only slight variation was found when the amount of AADG excreted by ten AGU patients under 10 years of age was compared with AADG excretion of older patients at a clinically advanced stage of the disease. The 24h AADG excretion of the younger patients was 354 mg compared with 441 mg in the group of ten patients over 20 years of age. Clinical symptoms were unrelated to AADG excretion.