Red cell 2,3-diphosphoglycerate levels in children with hereditary haemolytic anaemias.
The role of red cell 2,3-diphosphoglycerate (2,3-DPG) in increasing the availability of haemoglobin oxygen in neonatal jaundice and hereditary haemolytic anaemias was investigated. Measurements of 2,3-DPG were carried out on 58 normal children and six normal adults, 18 full-term newborns with neonatal jaundice and 57 cases (51 children and six adults) with hereditary haemolytic anaemias. In normal children and adults, with a mean haemoglobin of 12.69 g/dl, mean 2,3-DPG was 14.90 mumol/g Hb. In jaundiced newborns with a mean haemoglobin of 16.04 g/dl mean 2,3-DPG levels were 14.51 mumol/g Hb, i.e. normal. 2,3-DPG levels were increased in patients with beta-thalassaemia major, alpha-thalassaemia, sickle-cell disease, favism, hereditary spherocytosis and in heterozygotes for beta-thalassaemia with increased haemoglobin F. In heterozygotes for beta-thalassaemia with increased haemoglobin A2 only and in sickle cell trait 2,3-DPG levels were normal.
D000293:Adolescent; D000743:Anemia, Hemolytic / Q000097:blood / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D004163:Diphosphoglyceric Acids / Q000097:blood*; D005236:Favism / Q000097:blood; D005260:Female; D005319:Fetal Hemoglobin; D006441:Hemoglobin A; D006447:Hemoglobin H; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007567:Jaundice, Neonatal / Q000097:blood; D008297:Male; D013103:Spherocytosis, Hereditary / Q000097:blood; D013789:Thalassemia / Q000097:blood
D004163:Diphosphoglyceric Acids; D006441:Hemoglobin A; D005319:Fetal Hemoglobin; D006447:Hemoglobin H