الفهرس 
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Abstract
The aim of our study was to evaluate pulmonary functions in children with transfusion dependent thalassemia.
This was a prospective observational study conducted at the hematology clinic of Minia University children hospital, Faculty of Medicine, Minia University.
Children included in our study were subdivided into 2 groups: group 1 (thalassemic children) included 50 children they were 31 (62 %) males and 19 (38 %) females. Their age ranged from 9 to 18 years (mean age was13.2 ± 2.7 years) and group 2 (control) included 25 children, they were 17 (68 %) males and 8 (32 %) females. Their age ranged from 9 to 18 years (mean age was13.4 ± 2.7 years).
All thalassemic children included in our study were subjected to careful history taking, full clinical examination, laboratory studies included (pre-transfusion hemoglobin, serum ferritin, liver function tests, viral markers, renal function tests), pre-transfusion oxygen saturation, chest x-ray, echocardiography and pulmonary function tests “spirometry, lung volumes and DLCO”
Thalassemic children when compared to control group, had significantly lower pulmonary function tests parameters except FEV1/FVC % and PEFR %. Seventy % of our thalassemic children had isolated or combined pulmonary function abnormalities. Diffusion impairment reported in 18 (36 %) of the thalassemic children, while combined diffusion and restriction impairment was presented in 17 (34 %) of the thalassemic children.
Thalassemic children with combined diffusion and restrictive impairment had statistical significant differences than thalassemic children with normal pulmonary functions regarding older age, longer duration of blood transfusion, higher mean serum ferritin level with higher frequency of serum ferritin above the dangerous threshold (>2500ng/ml).
Thalassemic children with serum ferritin above dangerous threshold were significantly older and had significantly lower values of FVC%, FEV1%, PEFR, TLC% and DLCO% than children below the dangerous threshold.
The diffusion pulmonary impairment had only significant correlation with serum ferritin level. The restrictive pulmonary impairment correlated with age, mean duration of blood transfusion, serum ferritin level, and duration of chelation.
The only predictor of diffusion pulmonary impairment was to have a serum ferritin level above the dangerous threshold. On the other hand, restrictive pulmonary impairment could be predicted by being older age, having longer duration of blood transfusion, having shorter duration of chelation and having a serum ferritin level above the dangerous threshold, while the strongest predicting factor for the restrictive ventilation impairment was having a serum ferritin level above the dangerous threshold.
from our study we can concluded that:
Asymptomatic children with transfusion dependent thalassemia exhibit impaired diffusing capacity and/or restrictive pulmonary dysfunction in a significant proportion. The diffusion impairment was the predominant pulmonary function abnormality in children with transfusion dependent thalassemia. The severity of pulmonary dysfunction in thalassemic children was statistically significantly associated with higher serum ferritin values. The strongest predicting factor for the restrictive ventilation impairment was having a serum ferritin level above the threshold of 2500 ng/ml.