Respiratory function in patients with thalassaemia major: relation with iron overload

Abstract

Aims: (1) To determine the pattern of respiratory impairment in children with thalassaemia major (TM); (2) to assess the relation between the degree of respiratory impairment and total body iron content. Methods: Twenty nine TM patients were recruited. All underwent physical examination, standardised pulmonary function tests (spirometry, lung volume, and single breath diffusion capacity for carbon monoxide), and magnetic resonance imaging measurements of the liver. Serum ferritin was measured. The signal intensity ratio of liver to that of paraspinal muscle (T1 weighted sequence) and serum ferritin were used as surrogate index of body iron content. Results: Sixteen boys and 13 girls (median age 14.2 years) were studied. None had clinical evidence of congestive heart failure. Sixteen had normal lung function. Impairment of diffusion capacity (median DL co 83.5% predicted) was the most common abnormality, being observed in 34% of patients. Pure restrictive and obstructive ventilatory impairment was found in one and two patients respectively. Five patients had a combination of ventilation and diffusion defects. There was no correlation between the degree of impairment of each respiratory abnormality and body iron content. Conclusion: Diffusion impairment was the commonest abnormality found in our cohort of paediatric TM patients. Our data did not support the notion that respiratory function impairment was correlated with body iron content.

Talasemilerde Solunum Sistemi Bozuklukları

“…Hastaların %55'inin SFT'si normal olarak bulunurken geriye kalan %34'ünde ise difüzyon bozukluğu saptanmıştır 80 . Birçok çalışmada da β -TM tanılı hastalarda temel solunum sistemi bozukluğunun obstrüktif veya restriktif değil difüzyon bozukluğu olduğu öne sürülmüştür 72,80,87 . Bu durumun düşük hemoglobin konsantrasyonunun yanı sıra, ilerleyen zamanlarda alveoler-kapiller membran kalınlığının artışı ile difüzyon kapasitesinde azalma neticesinde görüldüğünü öne sürmüşlerdir.…”

“…Serum ferritin düzeyi şelasyon tedavisinin takibinde en sık kullanılan parametre olmasına rağmen bazı yayınlarda total demir yükünü tam olarak yansıtmadığı bildirilmiştir 80 . Bununla birlikte Borgna-Pignatti ve arkadaşları tarafından serum ferritin düzeylerinin ≥3000 ng/dL olduğu düzeylerde yüksek olasılıkla akciğer hasarı geliştiği ; ≥2500 ng/dL düzeylerinde ölüm riskinin 4 kat arttığı bildirilmiştir 94 .…”

Pulmonary Function Abnormalities in Non-Splenectomized and Splenectomized Adult Hemoglobin E/β-Thalassemia Patients and Their Correlation With Pulmonary Hypertension

“…[12][13][14] Other pulmonary abnormalities consist of small airway obstruction, 12,15 impairment of diffusion capacity, 16,17 arterial hypoxemia, 16,18 and pulmonary hypertension (PH). [19][20][21] Blood transfusion and iron chelation therapy for thalassemia disease in Thailand are usually not as intensive as in Western countries due to limited resources.…”

Comparison of pulmonary functions of thalassemic and of healthy children

“…Some studies find a significant inverse correlation between total lung capacity and iron burden 1-3 as well as between total lung capacity and age. 1,2 Others find that neither age 4 nor iron load 4,5,8 correlated with pulmonary function. To our knowledge, no data on this issue is available in Indonesia.…”

“…There was no correlation between percentage from predicted forced vital capacity and serum ferritin levels, whether in male (r=0.191; P=0.967) or female (r =-0.076, P=0.695) thalassemic subjects. Keywords: pulmonary function, thalassemia, spirometry, serum ferritin level, restrictive lung dysfunction T halassemia refers to a heterogeneous group of heritable hypochromic anemias of various degrees of severity; [1][2][3][4][5][6][7][8][9] it is considered to be the most prevalent genetic disorder in the world. 3 Transfusions of 15-20 ml/kg of packed cells are usually required every 4-5 weeks.…”