H. A. Mohamed, A. Mansour, N. Abousamra, Gehan A Elswaah, E. Ahmed
{"title":"埃及重型地中海贫血患儿肺功能及血清转化生长因子-β1","authors":"H. A. Mohamed, A. Mansour, N. Abousamra, Gehan A Elswaah, E. Ahmed","doi":"10.4103/ajop.ajop_4_21","DOIUrl":null,"url":null,"abstract":"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.","PeriodicalId":7866,"journal":{"name":"Alexandria Journal of Pediatrics","volume":"154 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Pulmonary affection and serum transforming growth factor-β1 among Egyptian children with thalassemia major\",\"authors\":\"H. A. Mohamed, A. Mansour, N. Abousamra, Gehan A Elswaah, E. Ahmed\",\"doi\":\"10.4103/ajop.ajop_4_21\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"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.\",\"PeriodicalId\":7866,\"journal\":{\"name\":\"Alexandria Journal of Pediatrics\",\"volume\":\"154 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2021-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Alexandria Journal of Pediatrics\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/ajop.ajop_4_21\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Alexandria Journal of Pediatrics","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/ajop.ajop_4_21","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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.