Serum hexosaminidase and beta-glucuronidase activities in infants: effects of age and sex.
We investigated the effect of age and sex on the serum activity of hexosaminidase (HEX) and  -glucuronidase (BGLU) in 275 normal term infants aged 12 h to 12 months. Up to six weeks of life, HEX was significantly higher in boys (P<=0.023). During the age period of 1-26 weeks, BGLU was also higher in boys, but differences were significant only at 2-6 and 7-15 weeks (P<=0.016). The developmental pattern of HEX and BGLU was sex dependent. HEX activity increased in both sexes from 4-7 days of life, reaching a maximum of 1.4-fold the birth value at 2-6 weeks of age in boys (P<0.001) and a maximum of 1.6-fold at 7-15 weeks in girls (P<0.001). HEX activity gradually decreased thereafter, reaching significantly lower levels at 27-53 weeks than during the first three days of life in boys (P = 0.002) and the same level of this age interval in girls. BGLU increased in both sexes from 4-7 days of age, showing a maximum increase at 7-15 weeks (3.3-fold in boys and 2.9-fold in girls, both P<0.001). Then BGLU decreased in boys to a value similar to that observed at 4-7 days of age. In girls, BGLU remained elevated until the end of the first year of life. These results indicate a variation of HEX and BGLU activities during the first year of life and a sex influence on their developmental pattern. This observation should be considered in the diagnosis of GM2 gangliosidosis and mucopolysaccharidosis type VII.
Mabe
P
P
Unidad de Genética y Enfermedades Metabólicas, INTA, Universidad de Chile, Santiago, Chile. pmabe@uec.inta.uchile.cl
Beck
M
M
D000367:Age Factors; D000704:Analysis of Variance; D015415:Biomarkers / Q000097:blood; D005260:Female; D020143:Gangliosidoses, GM2 / Q000175:diagnosis / Q000201:enzymology; D005966:Glucuronidase / Q000097:blood* / Q000502:physiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D016538:Mucopolysaccharidosis VII / Q000175:diagnosis / Q000201:enzymology; D012737:Sex Factors; D001619:beta-N-Acetylhexosaminidases / Q000097:blood* / Q000502:physiology
D015415:Biomarkers; D005966:Glucuronidase; D001619:beta-N-Acetylhexosaminidases