PEDIATRICS Vol. 59 No. 6 June 1977, pp. 919-926
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Hemoglobin-Oxygen Equilibrium in Cystic Fibrosis

Amnon Rosenthal M.D.1, Kon Taik Khaw M.D.1, and Harry Shwachman M.D.1

1 Department of Cardiology and the Division of Nutrition of the Department of Pediatrics, Children's Hospital Medical Center, Boston

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.

Submitted on June 25, 1976
Accepted on November 19, 1976