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Pulmonary functions in Egyptian children with transfusion-dependent β-thalassemia
A A Abd El Hakeem1, S M O Mousa1, M T AbdelFattah2
1Pediatric Department, Faculty of Medicine, Minia University, El-Minya, Egypt.
Insights
Beta thalassemia in children leads to lung diffusion impairment and restrictive abnormalities, even when asymptomatic. High iron overload, indicated by serum ferritin levels, significantly worsens these pulmonary issues.
Area of Science:
- Hematology
- Pulmonology
- Pediatrics
Background:
- Beta-thalassemia is characterized by ineffective erythropoiesis, hemolysis, and iron overload, affecting organs like the lungs.
- Iron deposition in vital organs is a significant complication of beta-thalassemia.
Purpose of the Study:
- To assess pulmonary function in asymptomatic children with beta-thalassemia on regular transfusions.
- To investigate the relationship between pulmonary dysfunction and iron overload in these children.
Main Methods:
- Pulmonary function tests (spirometry, lung volumes, diffusion capacity) were performed on 50 beta-thalassemic children and 50 controls.
- Serum ferritin levels, hemoglobin, chest X-rays, and echocardiography were analyzed for beta-thalassemic participants.
Main Results:
- 70% of beta-thalassemic children exhibited diffusion impairment, with 34% also showing restrictive abnormalities.
- Elevated serum ferritin (>2500 ng/mL) correlated with reduced lung function (FVC, FEV1, PEFR, TLC, DLCO).
- High serum ferritin was a key predictor of diffusion and restrictive lung dysfunction.
Conclusions:
- Asymptomatic children with beta-thalassemia frequently have pulmonary diffusion and restrictive impairments.
- These lung dysfunctions are significantly associated with the degree of iron overload, as measured by serum ferritin.
Background:
In β-thalassemia, there are varying degrees of ineffective haematopoiesis, intermittent haemolysis and iron overload. Excess iron is deposited in organs such as the heart, the liver, the endocrine glands and the lungs.
Objectives:
To evaluate the pulmonary functions in asymptomatic beta thalassemic children on regular transfusion therapy and their relation to iron overload.
Methods:
The study included 50 transfusion-dependent β-thalassemic children and 50 apparently healthy children as control. All children had undergone pulmonary function tests (spirometry, lung volumes and diffusion capacities). In addition, test to determine the mean serum ferritin of the last 2 years and pre-transfusion haemoglobin and chest radiograph and echocardiography were performed for the thalassemic children only.
Results:
A total of 70% of the thalassemic children had diffusion impairment, whereas 34% of them had associated restrictive abnormality. Thalassemic children with serum ferritin >2500 ng mL-1 had significantly lower values of forced vital capacity (FVC), forced expiratory volume at one second (FEV1), peak expiratory flow (PEFR), total lung capacity (TLC) and diffusing capacity of carbon monoxide (DLCO) (P < 0·05). Only diffusion impairment had a significant positive correlation with serum ferritin level. Restrictive impairment had significant positive correlations with age, duration of blood transfusion and serum ferritin level and a significant negative correlation with duration of chelation (P < 0·05). Having a serum ferritin >2500 ng mL-1 was the only predicting factor for diffusion impairment and the strongest predicting factor for restrictive dysfunction.
Conclusion:
Despite being asymptomatic, the majority of thalassemic children in this study suffered from diffusion impairment either alone or in combination with restrictive dysfunction. These pulmonary dysfunctions correlated significantly with body iron stores measured by serum ferritin.
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