High levels of zinc-protoporphyrin identify iron metabolic abnormalities in pulmonary arterial hypertension.
High levels of zinc-protoporphyrin identify iron metabolic abnormalities in pulmonary arterial hypertension.
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DOI：
10.1111/j.1752-8062.2011.00301.x
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发表时间：
2011-08
期刊：
影响因子：
--
通讯作者：
Erzurum SC
中科院分区：
文献类型：
--
作者：
Decker I;Ghosh S;Comhair SA;Farha S;Tang WH;Park M;Wang S;Lichtin AE;Erzurum SC
铁稳态影响与缺氧或血液系统疾病相关的肺动脉高压（PAH）的发展。为了研究特发性PAH（IPAH）的严重程度是否受铁代谢改变的影响，我们评估了IPAH、相关PAH（APAH）和睡眠呼吸暂停诱导的肺动脉高压（PH）患者与健康对照组和哮喘患者的铁代谢标志物，包括锌原卟啉（Zn-pp）、转铁蛋白受体水平以及红细胞数量和形态。尽管铁代谢的测量类似正常，但IPAH和睡眠呼吸暂停患者的Zn-pp水平升高约2倍，表明铁掺入不足形成血红素，并且水平与疾病严重程度的测量密切相关。与高Zn-PP一致，PAH患者红细胞分布宽度（RDW）增加。在包括IPAH和家族性疾病（FPAH）患者的扩展队列中，RDW得到验证，并与严重程度的临床参数相关，包括肺动脉压和6分钟步行距离。这些结果表明，原发性PAH亚临床功能性缺铁的患病率增加，与疾病严重程度定量相关。这表明铁稳态改变影响疾病进展，并证明了密切监测PAH患者铁状态的重要性。
Iron homeostasis influences the development of pulmonary arterial hypertension (PAH) associated with hypoxia or hematologic disorders. To investigate whether severity of idiopathic PAH (IPAH) is impacted by alterations in iron metabolism, we assessed iron metabolic markers, including levels of Zinc-protoporphyrin (Zn-pp), transferrin receptor, and red blood cell numbers and morphology in IPAH, associated PAH (APAH) and sleep apnea induced pulmonary hypertension (PH) patients in comparison to healthy controls and asthmatics. Despite similarly normal measures of iron metabolism, Zn-pp levels in IPAH and sleep apnea patients were elevated ~2-fold, indicating deficient iron incorporation to form heme and levels were closely related to measures of disease severity. Consistent with high Zn-PP, PAH patients had increased red cell distribution width (RDW). In an expanded cohort including patients with IPAH and familial disease (FPAH) the RDW was validated and related to clinical parameters of severity, including pulmonary artery pressures and 6 minute walk distances. These results reveal an increased prevalence of subclinical functional iron deficiency in primary forms of PAH that is quantitatively related to disease severity. This suggests that altered iron homeostasis influences disease progression and demonstrates the importance of closely monitoring iron status in PAH patients.
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影响因子：
--
作者：
Patel, Kushang V.;Ferrucci, Luigi;Ershler, William B.;Longo, Dan L.;Guralnik, Jack M.
通讯作者：
Guralnik, Jack M.
DOI：
10.1164/rccm.200406-710st
发表时间：
2005-04-15
影响因子：
24.7
作者：
American Thoracic Society;European Respiratory Society
通讯作者：
European Respiratory Society
影响因子：
6
作者：
Fijalkowska, Iwona;Xu, Weiling;Tuder, Rubin M.
通讯作者：
Tuder, Rubin M.
影响因子：
15.9
作者：
Hickey, Michele M.;Richardson, Theresa;Simon, M. Celeste
通讯作者：
Simon, M. Celeste
影响因子：
--
作者：
Perlstein, Todd S.;Weuve, Jennifer;Pfeffer, Marc A.;Beckman, Joshua A.
通讯作者：
Beckman, Joshua A.