International Committee for Standardization in Haematology: Recommended Screening Test for Glucose‐6‐Phosphate Dehydrogenase (G‐6‐PD) Deficiency

Severe hemolysis with normal blood count in a glucose-6-phosphate dehydrogenase deficient neonate

“…The reason that even a moderate degree of hemolysis may result in extreme hyperbilirubinemia may be due to diminished bilirubin conjugation found in G-6-PD deficient neonates compared with G-6-PD adequate controls. 18 Any factor further diminishing bilirubin conjugation, such as (TA) 7 promoter polymorphism associated with Gilbert syndrome 19 or prematurity, 15 may even further compromise the equilibrium. A moderate degree of hemolysis may release bilirubin into the blood stream without significantly lowering the Hb or Hct value and the rapid nature of the process may not allow for a reticulocyte response.…”

“…202, produced by Sigma Diagnostics, St Louis, MO, USA). The fluorescent test is the screening method preferred by the World Health Organization, 7 is precise in male hemizygotes 8 and has been shown to be accurate in male newborns. 9 Serum albumin was measured routinely by an automated analyzer (Vitros 5,1, Ortho-Clinical Diagnostics Inc., Rochester, NY, USA).…”

Differences Between Transcutaneous and Serum Bilirubin Measurements in Black African Neonates

“…Skin color was classified as previously reported in this population, 14 and recorded by the trained nurse assistants as shown in a color guide (Supplemental Fig 5). G6PD evaluation was based on the fluorescent blood spot for all infants by using methods described by Beutler et al, 17 as previously reported. 18 Lack of fluorescence after 10 minutes was indicative of deficient levels.…”

G6PD deficiency from lyonization after hematopoietic stem cell transplantation from female heterozygous donors

“…8 G6PD phenotype G6PD level was assayed by standard methods. 9 The median G6PD level in normal individuals was 11.7 (8.8-7.6) U/g hemoglobin, and in G6PD-deficient men 0.38 U/g hemoglobin. 6,7,9 As the G6PD mutants in the Chinese population do not give rise to congenital non-spherocytic hemolytic anemia, the G6PD enzyme levels are not expected to be affected by reticulocytosis.…”