Pulmonary function tests in beta thalassemia |
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Authors: | Meeta Arora Jagdish Chandra J C Suri S Narayan A K Dutta |
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Institution: | (1) Department of Pulmonary and Critical Care Medicine, Safdarjang Hospital, India;(2) Department of Pathology, Lady Hardinge Medical College and Kalawati Saran Children’s Hospital, New Delhi;(3) Department of Pediatrics, Lady Hardinge Medical College and Kalawati Saran Children’s Hospital, New Delhi;(4) 10, Lecturer’s Flats, Lady Hardinge Medical College Campus, Bangla Sahib Road, 110 001 New Delhi |
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Abstract: | To study pulmonary function tests (PFT) in multiple transfusion recipient thalassemics, PFTs were done for 30 thalassemics
and 20 matched controls. Confirmed cases of thalassemia on regular transfusion therapy were the subject of study. Apart from
history and physical examination of the thalassemics, serum ferritin estimation and spirometry were done. Parameters studied
included lung volumes—functional residual capacity (FRC), forced vital capacity (FVC), residual volume (RV) and total lung
capacity (TLC); and flow rates —forced expiratory volume in one second (FEV,), forced expiratory volume in 1 second/forced
vital capacity (FEV,/FVC), peak expiratory flow 25–75 (PEF 25–75%) and peak expiratory flow rate (PEFR). Single breath carbon
monoxide diffusing capacity (DLco) and arterial blood gas (ABG) were also analysed. The mean height and weight of thalassemics
were below that of age matched controls. A restrictive abnormality in PFT was found in 86.6% cases. These patients were found
to have a decrease in all the lung volumes namely FVC, FRC, RV and TLC with a proportional decrease in the flow rates, FEV1,
PEF 25–75% and PEF with a normal (> 0.75) FEV1/FVC ratio. DLco was decreased in all the patients with restrictive lung disease
and fall in DLco showed a good correlation (r = 0.7, P <. 001) with the severity of restrictive disease suggesting that some
intrapulmonary pathology is likely to be responsible for the restrictive pattern. None of the cases had an obstructive or
mixed pattern of pulmonary dysfunction. No correlation was found between severity of restrictive disease and the serum ferritin
levels. A negative correlation with degree of hepatosplenomegaly was found. No correlation was found between severity of the
defect and age, number of blood transfusions received and hemoglobin at the time of doing the test. To conclude, restrictive
lung disease is the predominant abnormality in multi-transfused thalassemics, which is probably due to pulmonary parenchymal
pathology. The abnormality of PFTs is not directly related to iron overload. |
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Keywords: | Pulmonary function tests Thalassemia Lung volumes |
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