Pulmonary function tests in beta thalassemia

Arora, M.; Chandra, Jagdish; Suri, Jyotsna; Narayan, Shashi; Dutta, A. K.

doi:10.1007/bf02723198

Cited by 26 publications

(32 citation statements)

References 10 publications

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“…But this treatment has some complications, such as progressive iron deposition in vital organs, which impairs their functions [3]. There are some debates about abnormalities of respiratory function and causes of its dysfunction [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22].…”

mentioning

confidence: 99%

PULMONARY FUNCTION TEST IN TRANSFUSION-DEPENDENT Β-Thalassemia PATIENTS

Azarkeivan

Mehrvar

Pour³

et al. 2008

Pediatric Hematology and Oncology

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Beta-thalassaemia is the most common hemoglobinopathies in our region with treatment of regular blood transfusion. Iron overload and hemosiderosis can cause organ involvement. Recent studies have focused on pulmonary involvement and pathophysiology of lung damage. The goal of this study was to investigate the pulmonary abnormalities in thalassemic patients in relation with sign and symptoms and iron overload. The authors studied pulmonary function test (PFT) at the Adult Thalassemia Clinic in Tehran. The history of blood transfusion, iron chelation, respiratory problems, and drug usage was taken. Physical examination, PFT, arterial blood gas (ABG), and chest X-ray (CXR) were done. In total, 139 patients were studied. The mean age was 21.1 years and mean duration of transfusion was 18 years. It was found that 133 patients (95.7%) did not have respiratory problems and only 6 (4.3%) had some respiratory complaints. In CXR, 100 patients (89.3%) had normal lung pattern and others (10.7%) had variable degrees of abnormal lung pattern. In ABG, mean of Po(2) was 73.5% and mean of O(2) saturation was 90.6%. In PFT, 101 patients (72.7%) had restrictive pattern, 35 (25.1%) had normal pattern, and 3 (2.2%) had combined pattern. According to vital capacity, the patients were placed in five categories: 54 patients (38.8%) normal, 37 (26.6%) mild, 35 (25.3%) moderate, 10 (7.2%) severe, and 3 (2.1%) extremely severe pulmonary deficit. There was no statistical significance between PFT results with all variables studied, except duration of blood transfusion, which may be considered a indirect effect of iron load (p = .05, r = .361). According to these results, restrictive pattern was the most common finding (72.7%) in PFT, while 95.7% of patients had no respiratory complaint, and in the chest X-ray group, 89.3% had normal pattern. The authors conclude that the lung may be considered a site for organ damage, and alteration of pulmonary function may be expected in transfusion-dependent patients in spite of no pulmonary symptoms or normal CXR. In recent years, because of new iron chelating drugs, doctors can expect thalassemic patients to have a long life-time and need to increase their quality of life. One way to do this is to evaluate the respiratory system by PFT to prevent the squeal of pulmonary disease.

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confidence: 99%

PULMONARY FUNCTION TEST IN TRANSFUSION-DEPENDENT Β-Thalassemia PATIENTS

Azarkeivan

Mehrvar

Pour³

et al. 2008

Pediatric Hematology and Oncology

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“…Akciğerlerdeki demir depolanması teorik olarak serum ferritin düzeyi ve karaciğerdeki demir depoları ile bağlantılıdır 76 . Serum ferritin düzeyi şelasyon tedavisinin takibinde en sık kullanılan parametre olmasına rağmen bazı yayınlarda total demir yükünü tam olarak yansıtmadığı bildirilmiştir 80 .…”

Section: Solunum Sistemi Bulgularıunclassified

“…Guidotti ve arkadaşları tarafından yapılan çalışmada ise, β -TM'li hastalarda %35.6 oranında restriktif bozukluk saptanmış ve serum ferritin düzeyleri ile restriktif patern arasında anlamlı ilişki olduğu bildirilmiştir 97 . Arora ve arkadaşlarının yaptığı bir çalışmada ise karaciğer demir yükünün non-invazif bir göstergesi olduğu düşünülen serum ferritin değerinin solunum fonksiyon testi anormalliği üzerine zayıf etkisi olduğu gösterilmiştir (p=0.07) 76 .…”

Section: Solunum Sistemi Bulgularıunclassified

“…Bu çalışmada transfüzyon programında olan 8 yaş üstü 30 hastaya SFT yapılmış; 26 hastada restriktif patern ve restriktif patern ile splenektomi arasında negatif korelasyon saptanmıştır (p=0.36). Aynı çalışmada hastaların yaşı (p=0.93), transfüzyon sayısı (p=0.84) ve hemoglobin değerleri (p=0.45) ile restriktif patern arasında anlamlı ilişki olmadığı görülmüştür (p>0.05) 76 . Fung ve arkadaşlarının bir çalışmasında ise, hepatosplenomegalinin restriktif solunum fonksiyon bozukluğuyla ilişkili olmadığı gösterilmiştir 77 .…”

Section: Solunum Sistemi Bulgularıunclassified

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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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“…Previous studies have reported pulmonary dysfunction in patients with β-thalassemia (Thal); mostly with restrictive impairment (RI) in patients being transfusion-dependent [1][2][3][4][5][6][7][8][9][10][11] and also in non-transfusion dependent patients. [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).…”

Section: Introductionmentioning

confidence: 99%

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

Pornsuriyasak

Vongvivat

Likittanasombat

et al. 2013

Thalassemia Reports

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The effect of splenectomy on pulmonary function test (PFT) and pulmonary hypertension (PH) in thalassemia remains unclear. We aimed to investigate PFT and their association with PH in patients with hemoglobin E/β-thalassemia stratified by their splenic status. Thirteen splenectomized patients (SP) and 12 non-splenectomized patients (NSP) were compared regarding to the PFT abnormalities and PH (mean pulmonary artery pressure from right-heart catheterization ≥25 mmHg or estimated systolic pulmonary artery pressure from echocardiography ≥40 mmHg). Eleven (84%) SP and 9 (75%) NSP had restrictive impairment (RI). Of these, more patients having severe RI in SP than in NSP (8 vs 2, P=0.035). FVC and PaO 2 were lower in SP than in NSP (66±15% vs 77±12%, P=0.043, and 79.38±1.6 mmHg vs 98.83±6.2 mmHg, P<0.001, respectively). Residual volume was higher in SP than in NSP (78±17% vs 64±15%, P=0.036). Seven (54%) SP who developed PH had a longer time interval between splenectomy and the onset of PH than those who did not (17±4.9 years vs 9.8±6.1 years, P=0.04). In conclusion, greater severity of extrapulmonary restrictive impairment and hypoxemia were more common in SP. These patients developed PH as a late complication unrelated to hypoxemia and PFT parameters.

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Pulmonary function tests in beta thalassemia

Cited by 26 publications

References 10 publications

PULMONARY FUNCTION TEST IN TRANSFUSION-DEPENDENT Β-Thalassemia PATIENTS

PULMONARY FUNCTION TEST IN TRANSFUSION-DEPENDENT Β-Thalassemia PATIENTS

Talasemilerde Solunum Sistemi Bozuklukları

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

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