title: Low lung capacity and hypoxemia in children with thalassemia major.
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.
mesh_terms: D000293:Adolescent; D001784:Blood Gas Analysis; D002648:Child; D004947:Esophagus / Q000503:physiopathology; D006801:Humans; D000860:Hypoxia / Q000503:physiopathology*; D008168:Lung / Q000473:pathology / Q000503:physiopathology*; D008176:Lung Volume Measurements; D008448:Maximal Expiratory Flow Rate; D010100:Oxygen / Q000097:blood; D010313:Partial Pressure; D011653:Pulmonary Diffusing Capacity; D013789:Thalassemia / Q000473:pathology / Q000503:physiopathology*; D014109:Total Lung Capacity
chemical_list: D010100:Oxygen