Respiratory function in patients with thalassaemia and iron overload

Dimopoulou, Ioanna; Kremastinos, D.; Maris, Thomas G.; Mavrogeni, Sophie; Tao, Ge

doi:10.1183/09031936.99.13360299

Cited by 31 publications

(42 citation statements)

References 15 publications

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“…Among complications, lung involvement in β-thalassemia has been emphasized only in recent years [1, 2]. Lung disease is rather frequent, having been reported in up to 80% of patients affected [3, 4]. …”

Section: Introductionmentioning

confidence: 99%

Lung Function in β-Thalassemia Patients: A Longitudinal Study

Piatti¹,

Allegra²,

Fasano³

et al. 2006

Acta Haematol

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Patients with β-thalassemia often present with a restrictive pattern at pulmonary function tests (PFTs) due to several pathogenetic factors. However, the long-term evolution is unknown. We performed a longitudinal study of pulmonary function in asymptomatic, non-smoking patients with β-thalassemia major and intermedia. We looked for temporal changes in lung function and characteristics that would predict the development of PFT abnormalities. In 1996, 18 patients with major β-thalassemia (9 males and 9 females; age range: 18–35 years) and 11 patients with intermediate β-thalassemia (5 males and 6 females; age range: 25–51 years) underwent clinical assessment and PFT, including body plethysmography and gas transfer study (carbon monoxide diffusion capacity, DL_CO). Patients were reassessed in 2003. An echocardiographic evaluation was also obtained to exclude pulmonary hypertension. In 55.5% of major and 45.4% of intermediate β-thalassemia patients, a restrictive pattern was found in 1996; in 2003 only 38.8 and 27.2% of patients, respectively, exhibited total lung capacities below the predicted values. DL_CO was unchanged in both groups of patients, being reduced in 5 thalassemia major patients and within the normal range in intermediate patients. We conclude that asymptomatic patients with β-thalassemia have a high prevalence of PFT abnormalities, but without significant increases over time. An improvement may be observed when good control of the iron balance is reached with optimal chelation therapy.

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Section: Introductionmentioning

confidence: 99%

Lung Function in β-Thalassemia Patients: A Longitudinal Study

Piatti¹,

Allegra²,

Fasano³

et al. 2006

Acta Haematol

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“…Ancak histopatolojik incelemelerde, akciğerlerde histolojik olarak saptanan demir birikimi ile solunum disfonksiyonu arasında neden-sonuç ilişkisi gösterilememiştir 75,85 .…”

Section: Solunum Sistemi Bulgularıunclassified

Talasemilerde Solunum Sistemi Bozuklukları

Kazgan¹,

Küpeli²

2017

Arşiv Kaynak Tarama Dergisi

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Thalassemia major is a worldwide healthcare problem requiring high cost medical treatment. Patients usually die of treatment associated complications in the second decade. However, improvements in treatment and meticulous follow-up strategies for treatment associated complications have led to an increase in expected life span of the patients. Regular transfusions cause progressive iron deposition in critical organs such as heart, liver and lungs. Iron deposition in the lungs results in respiratory dysfunction and impairment in quality of life of patients with thallassemia. Although iron deposition in respiratory system has been a known reality for many years in thallassemia, the effect of this deposition on respiratory functions has been an ongoing debate. Key words: Thalassemia major, transfusion, respiratory function tests, respiratory dysfunction. ÖZETTalasemi majör, tüm dünyada önemli bir sağlık sorunu olup, yüksek maliyetli tedavi gerektiren bir hastalıktır. Hastaların çoğu ikinci dekatta tedaviye bağlı komplikasyonlar nedeniyle kaybedilmektedir. Son yıllarda kullanıma giren yeni tedavi rejimleri ve tedaviye bağlı komplikasyonların dikkatli bir şekilde izlenmesiyle hastaların beklenen yaşam süreleri artmaktadır. Düzenli kan transfüzyonları nedeniyle kalp, karaciğer ve akciğer gibi hayati organlarda ilerleyici demir birikimi görülmektedir. Akciğer de demir birikiminin yaşandığı organlardan birisidir. Akciğerlerdeki demir birikimi akciğer dinamiklerinde değişikliklere yol açmakta ve bu durum hastaların yaşam kalitesini olumsuz etkilemektedir. Bu hastalarda akciğerlerde demir birikiminin olduğu uzun süreden beri bilinen bir gerçek ise de, bu birikimin akciğerler üzerine nasıl etkide bulunduğu konusu hala tartışma konusudur. Anahtar kelimeler: Talasemi majör, transfüzyon, solunum fonksiyon testi, respiratuar disfonksiyon.

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“…low predicted total lung capacity (TLC)) was noted to be a common abnormality in TM patients. Although it was postulated that this might be due to iron deposition in the respiratory system, no correlation was found between reduced TLC and hepatic iron loading [19]. They have also described impaired oxygen kinetics and limited functional capacity of peripheral muscles in TM patients during cardiopulmonary exercise testing [20,21].…”

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confidence: 99%