Plasma TGF-β₁ in pediatric cystic fibrosis: potential biomarker of lung disease and response to therapy.
Plasma TGF-β₁ in pediatric cystic fibrosis: potential biomarker of lung disease and response to therapy.
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DOI：
10.1002/ppul.21430
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发表时间：
2011-07
影响因子：
3.1
通讯作者：
Noah, Terry L.
中科院分区：
文献类型：
--
作者：
Harris, William T.;Muhlebach, Marianne S.;Oster, Robert A.;Knowles, Michael R.;Clancy, J. P.;Noah, Terry L.
转化生长因子β-1（TGF-β1）是囊性纤维化（CF）中肺部疾病严重程度的重要遗传修饰因子，但这种疾病相关性背后的机制仍不清楚。研究TGF-β1和CF肺病之间关系的初始步骤包括确定最适合用于TGF-β1蛋白测量的生物标本。在住院的儿童CF患者中，血浆TGF-β1升高与肺部疾病严重程度的临床参数相关。血清和血浆中获得之前和之后静脉抗生素治疗的儿科CF患者住院的肺部恶化。通过ELISA测量的总TGF-β1与肺部疾病的标志物进行比较，包括气道微生物学，肺功能和对治疗的反应。研究了40例CF儿童，其中15例在入院时接受了支气管肺泡灌洗（BAL）。血浆TGF-β1与BAL液TGF-β1呈正相关（r = 0.59，p <0.05）。铜绿假单胞菌阳性受试者入院时血浆TGF-β1升高（p = 0.014），与治疗后肺功能下降呈负相关（p <0.038）。抗生素治疗显著降低血浆TGF-β1（p <0.001）。血清TGF-β1与血浆TGF-β1、BALF TGF-β1及上述临床指标均无相关性。在儿童CF中，血浆（而非血清）TGF-β1升高与假单胞菌感染和肺部疾病相关，并在治疗后降低。这些发现强调了优化生物标本选择对于未来研究TGF-β1在CF肺病中的作用的重要性。
Transforming growth factor beta-1 (TGF-β1) is an important genetic modifier of lung disease severity in cystic fibrosis (CF), yet the mechanism behind this disease association remains unknown. Initial steps in the investigation of the relationship between TGF-β1 and CF lung disease include determining the most appropriate available biospecimen for TGF-β1 protein measurement. In hospitalized pediatric CF patients, plasma TGF-β1 is increased in association with clinical parameters of lung disease severity. Serum and plasma were obtained pre- and post intravenous antibiotic therapy in pediatric CF patients hospitalized for a pulmonary exacerbation. Total TGF-β1, measured via ELISA, was compared with markers of lung disease, including airway microbiology, lung function and response to therapy. Forty CF children were studied, 15 of whom underwent bronchoalveolar lavage (BAL) at the time of admission. Plasma TGF-β1 positively correlated with BAL fluid TGF-β1 (r = .59, p < .05). Admission plasma TGF-β1 was increased in subjects positive for Pseudomonas aeruginosa (p = .014) and was inversely associated with diminished lung function (p < .038) after therapy. Treatment with antibiotics significantly decreased plasma TGF-β1 (p < .001). Serum TGF-β1 was not associated with plasma TGF-β1, BALF TGF-β1 or these clinical parameters of lung disease. In pediatric CF, plasma (but not serum) TGF-β1 is increased in association with Pseudomonas infection and lung disease, and is reduced in response to therapy. These findings emphasize the importance of optimizing biospecimen selection for future studies investigating the role of TGF-β1 in CF lung disease.
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影响因子：
10
作者：
Hilliard, Tom N.;Regamey, Nicolas;Davies, Jane C.
通讯作者：
Davies, Jane C.
影响因子：
3.1
作者：
Muhlebach, MS;Miller, MB;Leigh, MW
通讯作者：
Leigh, MW
影响因子：
8.9
作者：
El-Gamel, A;Awad, MR;Hutchinson, IV
通讯作者：
Hutchinson, IV
DOI：
10.1513/pats.200703-040br
发表时间：
2007-08-01
期刊：
Proceedings of the American Thoracic Society
影响因子：
--
作者：
Mayer-Hamblett, Nicole;Ramsey, Bonnie W;Kronmal, Richard A
通讯作者：
Kronmal, Richard A
影响因子：
3.1
作者：
Corvol, Harriet;Boelle, Pierre-Yves;Clement, Annick
通讯作者：
Clement, Annick