A Clinical Analysis Of Glucose-6-Phosphate Dehydrogenase Deficiency In Neonates

Objective:To summarize the clinical characteristics of glucose-6-phosphate dehydrogenase (G6PD) deficiency in neonates and follow up the activity of G6PD.Methods:Retrospective analysis was done in the group of 43 neonates who were diagnosed as G6PD deficiency in children's hospital of Chongqing medical university from Jan 2008 to Jun 2008. Study the level of bilirubin,the morbidity and invasion-time of jaundice according to sex, the deficiency extent of the enzymatic activity and whether complicating ABO incompatibility. Follow up the activity of G6PD and detect G6PD gene.Reasults:1.39 cases developed hyperbilirubinemia in the 43 G6PD deficiency neonates, accounted for 90.7%. 4 cases developed bilirubin encephalopathy, accounted for 9.3%. The peek time when G6PD deficiency neonates develop jaundice is in 2~4 days after birth.2.In the G6PD deficiency neonates, it didn't show much difference in the activity of G6PD and the level of bilirubin between male and female(P>0.05);the level of bilirubin and the invasion-time of jaundice didn't take a notable difference among the three different enzymatic activity deficiency teams(P>0.05).3.It didn't make any difference in the activity of G6PD and the level of bilirubin, whether complicating ABO incompatibility.4.We made a follow-up analysis in 19 cases, accounted for 44.19%, with 4 cases abnormal. There was a great difference in the activity of G6PD before and after the follow-up(P<0.05).Conclusion:1.The characteristics of neonatal G6PD deficiency are:the disease occurs more frequently in male neonates than in female neonates. The male neonates tend to have high degree of deficiency. The level of bilirubin didn't take a notable difference among the three different enzymatic activity deficiency teams.2.It didn't make any difference in the level of bilirubin, whether complicating ABO incompatibility.3.There are 2 types:temporary type and permanent type in neonatal G6PD deficiency, and most of them are temporary.4.The morbidity of bilirubin encephalopathy is high in the G6PD deficiency neonates .5.It is advised that the detection of G6PD activity should be routinely carried out in neonatal screening in Chongqing, and subgroups should be divided according to enzymatic activity.