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...

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Bibliographic Details
Published inBirth defects original article series Vol. 23; no. 5A; p. 361
Main Authors Youngchaiyud, P, Suthamsmai, T, Fucharoen, S, Udompanich, V, Pushpakom, R, Wasi, P
Format Journal Article
LanguageEnglish
Published United States 1987
Subjects
Adult
Aged
Female
Hemoglobin E - analysis
Hemoglobins, Abnormal - analysis
Humans
Lung - physiopathology
Male
Middle Aged
Respiratory Function Tests
Splenectomy
Thalassemia - blood
Thalassemia - physiopathology
Thalassemia - surgery
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Summary: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.
ISSN:0547-6844