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Hemoglobin oxygen affinity in patients with cystic fibrosis

PLoS ONE, ISSN: 1932-6203, Vol: 9, Issue: 6, Page: e97932
2014
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In patients with cystic fibrosis lung damages cause arterial hypoxia. As a typical compensatory reaction one might expect changes in oxygen affinity of hemoglobin. Therefore position (standard half saturation pressure P st) and slope (Hill's n) of the O dissociation curve as well as the Bohr coefficients (BC) for CO and lactic acid were determined in blood of 14 adult patients (8 males, 6 females) and 14 healthy controls (6 males, 8 females). While Hill's n amounted to approximately 2.6 in all subjects, Pst was slightly increased by 1mmHg in both patient groups (controls male 26.7±0.2, controls female 27.0±0.1, patients male 27.7±0.5, patients female 28.0±0.3 mmHg; mean and standard error, overall p<0.01). Main cause was a rise of 1-2 μmol/g hemoglobin in erythrocytic 2,3-biphosphoglycerate concentration. One patient only, clearly identified as an outlier and with the mutation G551D, showed a reduction of both Pst (24.5 mmHg) and [2,3-biphosphoglycerate] (9.8 μmol/g hemoglobin). There were no differences in BCCO, but small sex differences in the BC for lactic acid in the controls which were not detectable in the patients. Causes for the right shift of the O dissociation curve might be hypoxic stimulation of erythrocytic glycolysis and an increased red cell turnover both causing increased [2,3-biphosphoglycerate]. However, for situations with additional hypercapnia as observed in exercising patients a left shift seems to be a more favourable adaptation in cystic fibrosis. Additionally when in vivo PO values were corrected to the standard conditions they mostly lay left of the in vitro O dissociation curve in both patients and controls. This hints to unknown fugitive factors influencing oxygen affinity. © 2014 Böning et al.

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http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=84903380483&origin=inward; http://dx.doi.org/10.1371/journal.pone.0097932; http://www.ncbi.nlm.nih.gov/pubmed/24919182; https://dx.plos.org/10.1371/journal.pone.0097932.g003; http://dx.doi.org/10.1371/journal.pone.0097932.g003; https://dx.plos.org/10.1371/journal.pone.0097932; https://dx.plos.org/10.1371/journal.pone.0097932.t002; http://dx.doi.org/10.1371/journal.pone.0097932.t002; http://dx.plos.org/10.1371/journal.pone.0097932.g001; http://dx.doi.org/10.1371/journal.pone.0097932.g001; http://dx.plos.org/10.1371/journal.pone.0097932.g002; http://dx.doi.org/10.1371/journal.pone.0097932.g002; https://dx.plos.org/10.1371/journal.pone.0097932.t001; http://dx.doi.org/10.1371/journal.pone.0097932.t001; https://dx.plos.org/10.1371/journal.pone.0097932.t004; http://dx.doi.org/10.1371/journal.pone.0097932.t004; https://dx.plos.org/10.1371/journal.pone.0097932.t005; http://dx.doi.org/10.1371/journal.pone.0097932.t005; https://dx.plos.org/10.1371/journal.pone.0097932.g005; http://dx.doi.org/10.1371/journal.pone.0097932.g005; https://dx.plos.org/10.1371/journal.pone.0097932.g004; http://dx.doi.org/10.1371/journal.pone.0097932.g004; https://dx.plos.org/10.1371/journal.pone.0097932.t003; http://dx.doi.org/10.1371/journal.pone.0097932.t003; https://dx.doi.org/10.1371/journal.pone.0097932.t004; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.t004; https://dx.doi.org/10.1371/journal.pone.0097932.t001; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.t001; https://dx.doi.org/10.1371/journal.pone.0097932.t003; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.t003; https://dx.doi.org/10.1371/journal.pone.0097932.t005; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.t005; https://dx.doi.org/10.1371/journal.pone.0097932.g003; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.g003; https://dx.doi.org/10.1371/journal.pone.0097932.g001; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.g001; https://dx.doi.org/10.1371/journal.pone.0097932.g002; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.g002; https://dx.doi.org/10.1371/journal.pone.0097932.g005; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.g005; https://dx.doi.org/10.1371/journal.pone.0097932; https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0097932; https://dx.doi.org/10.1371/journal.pone.0097932.t002; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.t002; https://dx.doi.org/10.1371/journal.pone.0097932.g004; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0097932.g004; https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0097932&type=printable; http://dx.plos.org/10.1371/journal.pone.0097932.t004; http://dx.plos.org/10.1371/journal.pone.0097932; http://dx.plos.org/10.1371/journal.pone.0097932.g005; http://dx.plos.org/10.1371/journal.pone.0097932.t005; http://dx.plos.org/10.1371/journal.pone.0097932.g003; http://dx.plos.org/10.1371/journal.pone.0097932.t001; http://www.plosone.org/article/metrics/info:doi/10.1371/journal.pone.0097932; http://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0097932&type=printable; http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0097932; http://dx.plos.org/10.1371/journal.pone.0097932.t002; http://dx.plos.org/10.1371/journal.pone.0097932.g004; http://dx.plos.org/10.1371/journal.pone.0097932.t003; http://journals.plos.org/plosone/article?id=10.1371%2Fjournal.pone.0097932

Dieter Böning; Angela Littschwager; Matthias Hütler; Ralph Beneke; Doris Staab; Shree Ram Singh

Public Library of Science (PLoS)

Biochemistry, Genetics and Molecular Biology; Agricultural and Biological Sciences; Multidisciplinary

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