Hemoglobin-oxygen equilibrium in cystic fibrosis.
A study of 35 patients with cystic fibrosis demonstrated that increasing severity of pulmonary involvement was associated with a mild but definite increase in erythrocyte 2,3-diphosphoglycerate (2,3-DPG) and a decrease in hemoglobin affinity for oxygen. The predominant regulators of 2,3-DPG were blood pH, cardiac output, and systemic oxygen transport. No significant relationship was observed between erythrocyte 2,3-DPG content and arterial oxygen tension. Hypophosphatemia may have prevented a greater increase in erythrocyte 2,3-DPG content. The inadequate increase in 2,3-DPG and consequent insufficient change in hemoglobin-oxygen affinity, coupled with an insufficient compensatory erythrocytic response, may adversely affect tissue oxygenation in patients with severe cystic fibrosis.
D000293:Adolescent; D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000097:blood* / Q000503:physiopathology; D004163:Diphosphoglyceric Acids / Q000097:blood; D004912:Erythrocytes / Q000032:analysis; D004920:Erythropoiesis; D005260:Female; D006439:Hemodynamics; D006454:Hemoglobins; D006801:Humans; D008297:Male; D010100:Oxygen / Q000097:blood*; D012119:Respiration
D004163:Diphosphoglyceric Acids; D006454:Hemoglobins; D010100:Oxygen