فراوانی کمبود آنزیم گلوکز 6 - فسفات‌دهیدروژناز در نوزادان مبتلا به هیپربیلی‌روبینمی

  Jaundice as a clinical symptom of hyperbillirubinemia will appear generally when the billirubin level is more than 7mg/dl. This temporary elevation of billirubin is caused by hemolysis of erythrocytes. Among several causes of pathologic hyperbillirubinemia in newborns X-linked G6PD deficiency may be the most important. Iran in one of the high risk countries. Due to the risk of death and severe neurological damage, prompt determination of G6PD deficiency in newborns suffering from jaundice is of importance. Therfore we investigated the deficiency of the enzyme in newborns.   The population under study comprised 1500 newborns afflicted with hyperbillirubinemia who were born in the teaching hospitals of Iran University of Medical Sciences and Health. Services The G6PD test was done with blood containing EDTA by using two methods o fluoroscent “spot test” and reduction of NADP to NADPH with dichlorophenol. Of 1500 newborns afflicted with hyperbillirubinemia, 240 newborns had G6PD deficiency and of the 240 newborns, 200 were boys and 40 were girls. This indicated that in the population under study, 16% of them suffering from G6PD deficiency, and deficiency of the enzyme in boys was more frequent than in girls (P<0.001). G6PD deficiency was more frequent in the newborns whose parents had familial marriage (P = 0.001).   Total billirubin level in 43.4% of patients was 15 ± 1.04 mg/dl, direct billirubin level in 36% of patients was 1.25 ± 0.25 mg/dl and hemoglubin level in 36.4% of them was 17 ± 1.09 g/dl. Onset of hyperbillirubinemia in G6PD deficient newborns was mostly at first day after birth (68.8%). We propose the screening of G6PD deficiency in newborns is important.