Rosenthal A, Khaw K T, Shwachman H
Pediatrics. 1977 Jun;59(6):919-26.
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.
一项针对35例囊性纤维化患者的研究表明，肺部受累程度的加重与红细胞2,3 - 二磷酸甘油酸（2,3 - DPG）轻度但确切的增加以及血红蛋白对氧的亲和力降低有关。2,3 - DPG的主要调节因素是血液pH值、心输出量和全身氧运输。未观察到红细胞2,3 - DPG含量与动脉血氧张力之间存在显著关系。低磷血症可能阻止了红细胞2,3 - DPG含量更大幅度的增加。2,3 - DPG增加不足以及随之而来的血红蛋白 - 氧亲和力变化不足，再加上红细胞代偿反应不足，可能会对重症囊性纤维化患者的组织氧合产生不利影响。
