Restrictive pulmonary dysfunction and its predictors in young patients with β‐thalassaemia major

Bourli, Evangelia; Dimitriadou, Meropi; Economou, Marina; Vlachaki, Efthimia; Christoforidis, Athanasios; Maratou, E; Stanopoulos, Ioannis; Argyropoulou, Paraskevi; Aivazis, Victor

doi:10.1002/ppul.22506

Cited by 20 publications

(37 citation statements)

References 28 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…This is similar to other published studies done by Rahim et al 4 and Bourli et al 6 where restrictive abnormalities were most frequently reported. Studies done by Kanj et al 4 and Amin et al 7 showed both Obstructive and Restrictive pattern of Respiratory dysfunction.…”

Section: Discussionsupporting

confidence: 82%

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

et al. 2015

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 82%

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

et al. 2015

View full text Add to dashboard Cite

show abstract

“…However, only two reports have described this fibrosis through recognition of areas of air-trapping on imaging techniques (high-resolution computed tomography) and showed the presence of iron-laden macrophages in bronchoalveolar lavage suggestive of lymphocyte alveolitis and interstitial fibrosis (Filosa et al, 2000;Piatti et al, 2006). Various studies have unsuccessfully searched for an association between pulmonary alterations and iron accumulation (estimated through serum ferritin levels or liver iron overload evaluated through T2* MRI): a relationship with serum ferritin levels has been described in a few cases (Kanj et al, 2000), but this association was not confirmed by other authors (Filosa et al, 2001;Abu-Ekteish et al, 2007;Bourli et al, 2012). Various studies have unsuccessfully searched for an association between pulmonary alterations and iron accumulation (estimated through serum ferritin levels or liver iron overload evaluated through T2* MRI): a relationship with serum ferritin levels has been described in a few cases (Kanj et al, 2000), but this association was not confirmed by other authors (Filosa et al, 2001;Abu-Ekteish et al, 2007;Bourli et al, 2012).…”

Section: Discussionmentioning

confidence: 99%

Pulmonary dysfunction in thalassaemia major: is there any relationship with body iron stores?

et al. 2016

View full text Add to dashboard Cite

Although pulmonary function abnormalities in thalassaemia major (TM) were described in 1980, the pathogenetic mechanism is not clear and data are contradictory, probably because of study heterogeneity and the multifactorial nature of the pathogenesis. We retrospectively analysed 73 adult TM patients to evaluate the prevalence of pulmonary dysfunction in adult TM and investigate relationships with iron load. All patients underwent body plethysmography and carbon monoxide diffusion (DLCO) was assessed in 63, in addition to blood tests, echocardiogram and T2* myocardial and liver magnetic resonance imaging. Restrictive lung disease was present in 26 (35·6%) patients. Serum ferritin levels were higher in patients with restrictive pattern (1526 μg/l vs. 975 μg/l, P = 0·05). Restrictive lung disease did not correlate with cardiac or liver iron overload. However, considering only patients with serum ferritin >2500 μg/l, those with restrictive pattern also had heart (T2* 14·28 ± 9·99 ms vs. 31·59 ± 7·43 ms) and liver iron overload (LIC 16·02 ± 8·44 mg vs. 5·02 ± 2·69 mg Fe/g dry weight) compared to those without restrictive pattern. Twenty-five patients (39·7%) had decreased DLCO. No correlation was observed with iron parameters. In our data restrictive pattern was predominant; we observed a relationship with serum ferritin levels suggesting that iron, particularly its chronic effect, could play a role in the pathogenesis of pulmonary disease.

show abstract

“…Serum ferritin düzeylerinin restriktif patern saptanan grupta, normal SFT olan gruba göre daha yüksek düzeyde olduğu gösterilmiştir 82 . Srittippayawan ve arkadaşları tarafından yapılan bir çalışmada 9-15 yaş arası, ayda bir kez transfüze edilen, şelasyon tedavisi almayan 21 talasemi majör tanılı hasta değerlendirilmiştir.…”

Section: Solunum Sistemi Bulgularıunclassified

“…Fung ve arkadaşlarının bir çalışmasında ise, hepatosplenomegalinin restriktif solunum fonksiyon bozukluğuyla ilişkili olmadığı gösterilmiştir 77 . Başka bir çalışmada ise, splenektomi yapılan ve yapılmayan hastalar ile restriktif SFT bozukluğu arasında anlamlı ilişki saptanmamıştır 82 .…”

Section: Solunum Sistemi Bulgularıunclassified

Talasemilerde Solunum Sistemi Bozuklukları

Kazgan¹,

Küpeli²

2017

Arşiv Kaynak Tarama Dergisi

View full text Add to dashboard Cite

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.

show abstract

Restrictive pulmonary dysfunction and its predictors in young patients with β‐thalassaemia major

Abstract: Our study shows that pulmonary impairment is shown in a great proportion even among asymptomatic young thalassaemic patients, thus, regular screening of pulmonary function should be adopted in the routine clinical follow up of these patients.

Cited by 20 publications

References 28 publications

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

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

Pulmonary dysfunction in thalassaemia major: is there any relationship with body iron stores?

Talasemilerde Solunum Sistemi Bozuklukları

Contact Info

Product

Resources

About