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  • Title: Low lung capacity and hypoxemia in children with thalassemia major.
    Author: Cooper DM, Mansell AL, Weiner MA, Berdon WE, Chetty-Baktaviziam A, Reid L, Mellins RB.
    Journal: Am Rev Respir Dis; 1980 Apr; 121(4):639-46. PubMed ID: 7386977.
    Abstract:
    We evaluated lung function in 17 children with thalassemia major in stable condition receiving blood transfusions at regular intervals and subcutaneous desferoxamine daily. Total lung capacity (TLC) was below 2 SD of normal values for height in 7 of the 17 children and arterialized capillary PO2 was below the normal range in 15. We studied lung mechanics in 4 children with reduced TLC and found static and dynamic compliance below 2 SD of normal values for height in 3, and lung recoil at TLC above normal values and specific upstream conductance (Gus/TLC) above 2 SD of normal values in all 4. Although these alterations in lung function have been described in patients with pulmonary fibrosis, we found no fibrosis in autopsy specimens of lung from 8 other patients with thalassemia. The rate constant of carbon monoxide diffusion (kCO) was above the predicted mean in 14 of 15 children. These findings can be explained by a decrease in the growth of airspace relative to the vascular bed and major airways during childhood.
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