Pulmonary dysfunction in children with beta thalassemia major in relation with iron overload - a cross sectional hospital based study

Abstract

Background: Previously many patients with thalassemia major died from severe anemia during first decade, but with modern transfusion therapy many now survive beyond childhood. Because of such therapy, however excessive iron deposition occurs in lungs and causing respiratory dysfunction. Respiratory impairment occurs because of defective chelation and repeated transfusions. Objectives: To a) study pattern of respiratory impairment using spirometry, b) estimate iron overload by measuring serum ferritin levels, c) correlate a&b. Methodology:Thalassemia children >7year, on regular blood transfusion were included in the study after getting institutional ethical clearance .Standardised pulmonary function test was done using spirometry. Iron overload was assessed using serum ferritin levels. Severity of pulmonary dysfunction was correlated with serum ferritin levels. Results:Total of 42 children were included 62% were males and 38% were females (with median age 12yrs). By spirometry 95% had restrictive pattern of respiratory dysfunction. Mean ferritin value was 4152. Out of them10 (23.8%) mild, 25(59%) moderate and 5(12%) severe dysfunction based on FEV1 and FVC. The mean ferritin values in severe respiratory dysfunction is 6275 which is significantly higher when compared to moderate (4249) and mild (3066) pattern of respiratory dysfunction. None of the children had evidence of CCF. Significant correlation (p value=0.003) was found between severity of pulmonary dysfunction with ferritin values and also with weight of the child (p value=0.007). No other significant correlation found between severity pattern and transfusion index, age or height. Conclusion:Restrictive pattern is most common pulmonary dysfunction seen in chronic iron overloaded thalassemia major children. Regular blood transfusions with adequate chelation decrease incidence of pulmonary dysfunction. Screening of all thalassemia children using spirometry is need of the hour.

Pulmonary functions in Egyptian children with transfusion‐dependent β‐thalassemia

“…Several studies confirmed the correlation between serum ferritin or the body iron burden and pulmonary dysfunctions (Kanj et al, 2000;Carnelli et al, 2003). However, this contradicted with other studies (Li et al, 2002;Boddu et al, 2015). The discrepancy of the results among the studies could be due to the multifactorial causes of pulmonary dysfunctions in thalassemic patients.…”

“…Thirty-five (70%) thalassemic children had isolated or combined PFT abnormalities. Many studies addressed the pulmonary functions in children with -thalassemia major, reporting percentages of pulmonary dysfunction ranging between 36·1 and 95% (Arora et al, 2001;Abu-Ekteish et al, 2007;Parakh et al, 2007;Rahim et al, 2008;Eidani et al, 2009;Alyasin et al, 2011;Boddu et al, 2015;Ozyoruk & Misirlioglu, 2015). This wide range of percentages may be attributed to the measurement of lung volumes and diffusion capacities, beside spirometry, in some studies and the use of spirometry alone in others.…”

“…However, it is the most common and least invasive one used for monitoring body iron stores and chelation therapy in thalassemic patients (Hamdy et al, 2007). Restrictive pulmonary impairment had a significant negative correlation with the duration of chelation as deterioration of lung functions occurs with inadequate chelation (Boddu et al, 2015). Therefore, difficulties facing chelation therapy should be addressed to maintain normal PFT, such as the availability of chelator drugs, their tolerability, transfusional iron burden and the patient's compliance.…”

“…A restrictive pattern has been reported as the most frequent abnormality in the pulmonary function test in thalassemia patients in the literature, especially in adults (Abu-Ekteish et al, 2007). However, airway obstruction, diffusion impairment or small-airway disease were also reported (Alyasin et al, 2011;Bourli et al, 2012;Boddu et al, 2015).…”

Kadar Feritin Serum Terhadap Fungsi Paru pada Pasien Talasemia β Mayor

“…Konsekuensi perubahan ini berupa penurunan atau hilangnya mRNA untuk satu atau lebih rantai goblin atau tidak efektifnya pembentukan mRNA. 13 Gangguan fungsi paru pada pasien talasemia β mayor telah diketahui dalam tiga dekade terakhir meskipun sampai saat ini mekanisme patogenesisnya belum sepenuhnya diketahui. Berbagai variasi gangguan fungsi paru ditemukan pada pasien talasemia β mayor berupa gangguan fungsi paru restriktif, obstruktif, dan atau campuran.…”