Lung function tests in splenectomized beta-thalassemia/Hb E patients.
Pulmonary function tests were performed on 30 patients with beta-thalassemia/Hb E. Only one patient had normal pulmonary function. Arterial hypoxemia at rest was present in 22 of 30 patients (73.3%). Abnormal VC, FEV1, FEV1/FVC, and end tidal FEV1 were found in 29 (96.7%), 17 (56.7%), 6 (20.0%), and 17 (56.7%) patients, respectively. Single-breath carbon monoxide diffusing capacity was abnormal in one out of 11 patients (9.1%). These results suggest the presence of abnormal ventilatory function included restrictive, obstructive, and combined defects. The hypoxemia in thalassemia was probably due to ventilation/perfusion mismatch and a diffusion defect.
Department of Medicine, Siriraj Hospital, Bangkok, Thailand.
P
P
Youngchaiyud
T
T
Suthamsmai
S
S
Fucharoen
V
V
Udompanich
R
R
Pushpakom
P
P
Wasi
D000328:Adult; D000368:Aged; D005260:Female; D006446:Hemoglobin E / Q000032:analysis*; D006455:Hemoglobins, Abnormal / Q000032:analysis*; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests; D013156:Splenectomy; D013789:Thalassemia / Q000097:blood* / Q000503:physiopathology / Q000601:surgery
D006455:Hemoglobins, Abnormal; D006446:Hemoglobin E