地中海贫血对呼吸功能的限制性影响:一个中心的经验。

Northern clinics of Istanbul Pub Date : 2023-09-14 eCollection Date: 2023-01-01 DOI:10.14744/nci.2023.65768
Gizem Zengin Ersoy, Ercan Nain, Mehtap Ertekin, Ozlem Terzi, Ayse Senay Sasihuseyinoglu, Gurcan Dikme
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摘要

目的:重型地中海贫血(TM)患者的呼吸功能与螯合作用差是一个经常研究的问题。我们的研究旨在评估慢性输血项目中TM患者的肺功能,并将其与年龄、铁蛋白水平和输血前血红蛋白值相关联。方法:记录97名没有任何潜在心脏或慢性呼吸系统疾病的患者(55名男孩和42名女孩)的身高、体重、肺功能测试(PFT)结果、输血前血红蛋白水平和铁蛋白水平。PFT由用力肺活量(FVC)和1秒用力呼气量(FEV1)、FEV1/FVC与呼气峰流量(PEF)的比率以及呼气中流量的25%至75%之间的用力呼气中流量(MEF25-75)组成。结果:58例(60%)患者出现FVC低,26例(27.6%)患者出现FEV1低,62例(64.5%)患者出现PEF低,8例(8.3%)患者出现MEF25-75低。年龄、身高和铁蛋白值显著影响MEF25-75(p25-75(r=-0.221)),输血前血红蛋白与FVC呈弱正相关(r=0.222)。结论:年龄和身高是影响FEV1、MEF25-75%和PEF的主要危险因素。在我们的研究中,血清铁蛋白仅对MEF25-75有影响。TM患者的呼吸功能受到限制性影响。
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Restrictive effects of thalassemia on respiratory functions: One center experience.

Objective: Respiratory functions in thalassemia major (TM) patients concerning poor chelation are a frequently researched issue. Our study aims to evaluate the lung functions of our patients with TM in the chronic transfusion program and to correlate them with their age, ferritin levels, and pre-transfusion hemoglobin values.

Methods: Height, weight, pulmonary function test (PFT) results, pre-transfusion hemoglobin levels, and ferritin levels of 97 patients (55 boys and 42 girls) without any underlying cardiac or chronic respiratory disease were recorded. PFT is consisted of forced vital capacity (FVC) and forced expiratory volume in one second (FEV1), the ratio of FEV1/FVC to peak expiratory flow (PEF), and forced mid-exhaled flow between 25% and 75% of mid-expiratory flow (MEF25-75). Data were analyzed with IBM SPSS V25.

Results: Low FVC was observed in 58 patients (60%), and low FEV1 was observed in 26 patients (27.6%). Low PEF was observed in 62 patients (64.5%), and low MEF25-75 was observed in 8 (8.3%). PFT was affected in 75 patients (78.1%). The pattern of involvement was restrictive. Age, height, and ferritin values significantly affected the MEF25-75 (p<0.05). Age and pre-transfusion hemoglobin values had a significant effect on the FVC test (p<0.05). There was a weak negative correlation between ferritin values and MEF25-75 (r=-0.221) and a weak positive correlation between pre-transfusion hemoglobin and FVC (r=0.222).

Conclusion: Age and height are the main risk factors affecting FEV1, MEF25-75, and PEF. Serum ferritin has only an effect on MEF25-75 in our study. The respiratory functions of TM patients were affected in a restrictive pattern.

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