埃及重型地中海贫血患儿肺功能及血清转化生长因子-β1

H. A. Mohamed, A. Mansour, N. Abousamra, Gehan A Elswaah, E. Ahmed
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引用次数: 0

摘要

背景:重度地中海贫血(TM)患者对肺部的影响已经确立;然而,尚未观察到一种特殊的疾病模式。转化生长因子-β1 (TGF-β)超家族成员在气道阻塞性疾病发病机制中的优先作用的建议来自以往的遗传学研究。目的探讨TM患儿血清TGF-β1水平及其与肺功能的关系。患者和方法共纳入40例β-TM患者,与40例年龄匹配、性别匹配的正常儿童作为对照组。对病例进行临床评估,包括人体测量、呼吸检查、肺活量测定和胸片检查,而对病例和对照组的血清TGF-β1进行评估,使用TGF-β1捕获酶联免疫吸附试验。结果肺功能障碍类型不同:阻塞性12例(30%),限制性7例(17.5%),混合性9例(22.5%),肺功能正常12例(30%)。血清TGF-β1与1 s内用力呼气量均呈显著负相关(r: - 0.317, P)。结论血清TGF-β1等炎症标志物可能在地中海贫血患者肺功能的影响中发挥作用,无论是阻塞性还是限制性。有一个潜在的作用,过量的铁蛋白沉积在气道,导致肺功能检查阻塞性肺型。
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Pulmonary affection and serum transforming growth factor-β1 among Egyptian children with thalassemia major
Background Pulmonary affection in patients with thalassemia major (TM) is well established; however, a special pattern of disease has not yet been observed. Suggestions in preference of the role for transforming growth factor-β1 (TGF-β) superfamily members in the pathogenesis of airway obstructive diseases has arisen from previous genetic studies. Objectives We aimed to assess TGF-β1 serum level in the blood and its relation to pulmonary affection in children with TM. Patients and methods A total of 40 patients with β-TM were included, who were compared with 40 age-matched and sex-matched normal children as a control group. Cases were subjected to clinical assessment for anthropometric measurements and respiratory examination, spirometry, and chest radiograph, whereas serum TGF-β1 was assessed in cases and control using a TGF-β1 capture enzyme-linked immunosorbent assay. Results Different patterns of pulmonary dysfunction in spirometry were found: 12 (30%) patients had obstructive pattern, seven (17.5%) patients had restrictive pattern, nine (22.5%) patients had mixed pattern, whereas 12 (30%) patients had normal pulmonary function. Moreover, a significant negative correlation between serum TGF-β1 and both forced expiratory volume in 1 s (r: −0.317, P Conclusion Inflammatory markers such as serum TGF-β1 may play a role in pulmonary affection in patients with thalassemia, either obstructive or restrictive pattern. There is a potential role of excess ferritin deposited in the airways, resulting in obstructive pulmonary pattern in pulmonary function tests.
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