Abstract

Serum activity of hexosaminidases A and B has been measured to identify patients with Tay-Sachs and Sandhoff's diseases, and may also be affected in other clinical conditions including neonatal necrotizing enterocolitis (NEC). Data utilizing serum HEX as a marker for NEC is difficult to interpret because normal developmental patterns have not been characterized. We have measured serum activity of total HEX and Hex B in 61 neonates of 27 to 40 weeks gestation who did not have NEC. These infants were followed from birth until 4 to 8 weeks of age. The infants were divided into 2 gestational age groups (≤34 weeks and >35 weeks) since enzyme activities were similar within each of these 2 groups. Total serum HEX activity, increased with postnatal (PN) age and was greater in the older gestational age group (ANOVA p<0.01). Factors associated with increased total serum HEX include enteral feeding, parenteral nutrition and unexplained hematochezia. Serum HEX activity in infants with clinical conditions that may influence serum activity of this enzyme must be compared to that in normal infants of similar gestational and postnatal ages.

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