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letter
. 1988 May;80(5):509–514.

Lung Function in Sickle Cell Hemoglobinopathy Patients Compared With Healthy Subjects

Roscoe C Young Jr, Raylinda E Rachal, Carl A Reindorf, Earl M Armstrong, Octavius D Polk Jr, Robert L Hackney Jr, Roland B Scott
PMCID: PMC2625764  PMID: 3418733

Abstract

Previous studies of lung function tests performed on patients with sickle cell disease have shown a restrictive ventilatory defect, usually a diffusion defect, and mild hypoxia at rest. The present study was undertaken to explain the pathophysiology of these changes and to extend these studies to include functional measurements not reported previously.

Lung function studies were performed at rest and during treadmill walking on 66 patients with sickle cell anemia and on 16 healthy control subjects. Patients had restrictive ventilatory defects, decreased lung compliance, and uneven ventilation-perfusion ratios. These abnormalities caused an increased alveolar-arterial oxygen tension difference that caused hypoxemia. The diffusion defects were because of the sickle cell disease.

Carboxyhemoglobin levels were increased in patients with sickle cell disease. This increase may be caused by a combination of factors, including increased cigarette smoking, hemolysis, and preferential survival of red blood cells that contain carbon monoxide and which do not sickle. During treadmill walking, the patients with sickle cell disease showed a decreased work tolerance caused by impaired oxygen delivery. The anaerobic threshold is reached sooner in patients with sickle cell disease and may also account for the limitations in work capacity of these patients.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. ALLISON A. C. Genetic factors in resistance to malaria. Ann N Y Acad Sci. 1961 Jun 7;91:710–729. doi: 10.1111/j.1749-6632.1961.tb31102.x. [DOI] [PubMed] [Google Scholar]
  2. BECKLAKE M. R., GRIFFITHS S. B., McGREGOR M., GOLDMAN H. I., SCHREVE J. P. Oxygen dissociation curves in sickle cell anemia and in subjects with the sickle cell trait. J Clin Invest. 1955 May;34(5):751–755. doi: 10.1172/JCI103129. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. BURROWS B., KASIK J. E., NIDEN A. H., BARCLAY W. R. Clinical usefulness of the single-breath pulmonucy diffusing capacity test. Am Rev Respir Dis. 1961 Dec;84:789–806. doi: 10.1164/arrd.1961.84.6.789. [DOI] [PubMed] [Google Scholar]
  4. Bromberg P. A., Jensen W. N. Arterial oxygen unsaturation in sickle cell disease. Am Rev Respir Dis. 1967 Sep;96(3):400–407. doi: 10.1164/arrd.1967.96.3.400. [DOI] [PubMed] [Google Scholar]
  5. COBURN R. F., BLAKEMORE W. S., FORSTER R. E. Endogenous carbon monoxide production in man. J Clin Invest. 1963 Jul;42:1172–1178. doi: 10.1172/JCI104802. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. COMROE J. H., Jr, FOWLER W. S. Lung function studies. VI. Detection of uneven alveolar ventilation during a single breath of oxygen. Am J Med. 1951 Apr;10(4):408–413. doi: 10.1016/0002-9343(51)90285-9. [DOI] [PubMed] [Google Scholar]
  7. Dinakara P., Blumenthal W. S., Johnston R. F., Kauffman L. A., Solnick P. B. The effect of anemia on pulmonary diffusing capacity with derivation of a correction equation. Am Rev Respir Dis. 1970 Dec;102(6):965–969. doi: 10.1164/arrd.1970.102.6.965. [DOI] [PubMed] [Google Scholar]
  8. FOWLER N. O., SMITH O., GREENFIELD J. C. Arterial blood oxygenation in sickle cell anemia. Am J Med Sci. 1957 Oct;234(4):449–458. doi: 10.1097/00000441-195710000-00010. [DOI] [PubMed] [Google Scholar]
  9. Femi-Pearse D., Gazioglu K. M., Yu P. N. Pulmonary function studies in sickle cell disease. J Appl Physiol. 1970 May;28(5):574–577. doi: 10.1152/jappl.1970.28.5.574. [DOI] [PubMed] [Google Scholar]
  10. GOLDMAN H. I., BECKLAKE M. R. Respiratory function tests; normal values at median altitudes and the prediction of normal results. Am Rev Tuberc. 1959 Apr;79(4):457–467. doi: 10.1164/artpd.1959.79.4.457. [DOI] [PubMed] [Google Scholar]
  11. KORY R. C., CALLAHAN R., BOREN H. G., SYNER J. C. The Veterans Administration-Army cooperative study of pulmonary function. I. Clinical spirometry in normal men. Am J Med. 1961 Feb;30:243–258. doi: 10.1016/0002-9343(61)90096-1. [DOI] [PubMed] [Google Scholar]
  12. MOSER K. M., LUCHSINGER P. C., KATZ S. Pulmonary and cardiac function in sickle cell lung disease: preliminary report. Dis Chest. 1960 Jun;37:637–648. doi: 10.1378/chest.37.6.637. [DOI] [PubMed] [Google Scholar]
  13. McCarthy D. S., Spencer R., Greene R., Milic-Emili J. Measurement of "closing volume" as a simple and sensitive test for early detection of small airway disease. Am J Med. 1972 Jun;52(6):747–753. doi: 10.1016/0002-9343(72)90080-0. [DOI] [PubMed] [Google Scholar]
  14. McGRATH M. W., THOMSON M. L. The effect of age, body size and lung volume change on alveolar-capillary permeability and diffusing capacity in man. J Physiol. 1959 Jun 11;146(3):572–582. doi: 10.1113/jphysiol.1959.sp006212. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Miller G. J., Serjeant G. R. An assessment of lung volumes and gas transfer in sickle-cell anaemia. Thorax. 1971 May;26(3):309–315. doi: 10.1136/thx.26.3.309. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Wall M. A., Platt O. S., Strieder D. J. Lung function in children with sickle cell anemia. Am Rev Respir Dis. 1979 Jul;120(1):210–214. doi: 10.1164/arrd.1979.120.1.210. [DOI] [PubMed] [Google Scholar]
  17. Wasserman K., Whipp B. J. Excercise physiology in health and disease. Am Rev Respir Dis. 1975 Aug;112(2):219–249. doi: 10.1164/arrd.1975.112.2.219. [DOI] [PubMed] [Google Scholar]
  18. Young R. C., Jr, Wright P., 3rd, Banks D. D. Lung function abnormalities occurring in sickle cell hemoglobinopathies: a preliminary report. J Natl Med Assoc. 1976 May;68(3):201-5, 181. [PMC free article] [PubMed] [Google Scholar]

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