Premium
Neonatal hyperbilirubinaemia in heterozygous giucose‐6‐phosphate dehydrogenase deficient females
Author(s) -
Meloni Tullio,
Forteleoni Gavino,
Dore Angelo,
Cutillo Stefano
Publication year - 1983
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1983.tb02017.x
Subject(s) - glucose 6 phosphate dehydrogenase , dehydrogenase , glucose 6 phosphate dehydrogenase deficiency , bilirubin , medicine , enzyme , jaundice , phenobarbital , endocrinology , biology , chemistry , biochemistry
S ummary . Glucose‐6‐phosphate dehydrogenase (G6PD, D‐glucose 6‐phosphate: NADP oxidoreductase, E.C. 1.1.1.49) activity and the percentage of G6PD deficient erythrocytes was determined in 50 girls heterozygous for G6PD deficiency, 25 of whom had had hyperbilirubinaemia at birth and 25 who had normal bilirubin levels. The enzymatic activity was 2 32 ±0·87 l.U./g Hb in the first group and 3·31 ±0·92 l.U./g Hb in the second group. The percentage of G6PD deficient erythrocytes was 54.1 ±15.3 and 65.3 ±0.87, respectively. The level of enzymatic activity exceeded 4 I.U./g Hb and the percentage of G6PD deficient cells fell below 40% in only one of the subjects who had developed hyperbilirubinaemia. Levels of enzymatic activity below 4 I.U./g Hb, or percentages of G6PD deficient erythrocytes higher than 40% can therefore be considered to be associated with a high risk of developing neonatal hyperbilirubinaemia. In our opinion, these babies should receive prophylactic treatment with phenobarbital, as do G6PD deficient Mediterranean males.