Restrictive pulmonary dysfunction and its predictors in young patients with β-thalassaemia major.
BACKGROUND
Pulmonary dysfunction represents one of the most undervalued and less recognized complications in patients with β-thalassaemia.


OBJECTIVE
The aim of this study was to assess the pattern of pulmonary dysfunction and consequently to investigate possible associated factors that might contribute to lung impairment in young patients with β-thalassaemia major.


METHODS
Fifty-two children and young adults (mean age: 21.33 ± 6.24 years) with β-thalassaemia major on conventional treatment (transfusions and iron chelation therapy) were included in the study. A complete computerized pulmonary function testing (PFT) system for recording pulmonary diffusion capacity and simultaneous determination of alveolar volume and pulmonary volumes was equipped.


RESULTS
Results showed that 20 patients (38.46%) had restrictive pulmonary pattern that was preferentially observed in older and shorter patients. Serum ferritin levels were higher in the restrictive group (2,096 ± 1,831 ng/dl) compared to patients with normal pulmonary function (1,354 ± 942 ng/dl) (P = 0.066). Diffusional impairment characterized by significantly lower DLCO*% values, was observed in the restrictive group (P = 0.004), implicating the 62.5% of the population studied. Paired linear correlations showed that age was negatively correlated to DLCO*% (r = -0.548, P < 0.001) and SaO(2) % (r = -0.789, P < 0.001) and with most of the pulmonary functional parameters that determine a restrictive. Multivariate regression analysis identified age as the major predictor for restrictive pulmonopathy followed by serum ferritin levels.


CONCLUSIONS
Our study shows that pulmonary impairment is shown in a great proportion even among asymptomatic young thalassaemic patients, thus, regular screening of pulmonary function should be adopted in the routine clinical follow up of these patients.
First Paediatric Department, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Evangelia
E
Bourli
Meropi
M
Dimitriadou
Marina
M
Economou
Efthimia
E
Vlachaki
Athanasios
A
Christoforidis
Eleni
E
Maratou
Ioannis
I
Stanopoulos
Paraskevi
P
Argyropoulou
Victor
V
Aivazis
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D008171:Lung Diseases / Q000209:etiology / Q000503:physiopathology*; D008297:Male; D012129:Respiratory Function Tests; D012307:Risk Factors; D017086:beta-Thalassemia / Q000150:complications / Q000503:physiopathology*
D005293:Ferritins