Prospective, longitudinal study of plastic bronchitis cast pathology and responsiveness to tissue plasminogen activator.
Prospective, longitudinal study of plastic bronchitis cast pathology and responsiveness to tissue plasminogen activator.
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塑性支气管炎铸造病理学和对组织纤溶酶原激活剂的反应性的前瞻性纵向研究。
DOI：
10.1007/s00246-011-0058-x
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发表时间：
2011-12
影响因子：
1.6
通讯作者：
Stringer, Kathleen A.
中科院分区：
文献类型：
--
作者：
Heath, Lauren;Ling, Shelley;Racz, Jennifer;Mane, Gerta;Schmidt, Lindsay;Myers, Jeffrey L.;Tsai, Wan C.;Caruthers, Regine L.;Hirsch, Jennifer C.;Stringer, Kathleen A.
塑性支气管炎（PB）是一种罕见的疾病，常见于接受分阶段单心室姑息治疗的先天性心脏病（CHD）患者。它的特点是在气道中形成橡胶“铸件”。铅治疗通常包括吸入tPA。然而，tPA对减轻铸造负荷的效果尚不清楚。由于我们缺乏对铸造成分的了解，情况更加复杂。我们从患有冠心病的儿童（n=4）和一名患有特发性PB的成人患者中获得了自发咳痰的PB模型。石蜡保存标本进行病理评价。铸型用磷酸盐缓冲盐水（PBS）或tPA处理。通过铸体重量的变化和纤维蛋白d -二聚体的产生来评估铸体对tPA的反应。与剂量无关，与pbs治疗相比，tPA降低了铸体重量（p=0.001）并增加了d -二聚体水平。组织学染色显示，所有患者的铅模均由纤维蛋白组成，并含有大量淋巴细胞。演员组成并没有随着时间的推移而改变。总的来说，这些数据支持在我们的PB患者中，铸型由纤维蛋白组成，对tPA治疗有反应。这使得吸入tPA成为缓解PB症状的潜在可行选择，同时我们正在努力揭示PB发病机制的复杂性。
Plastic bronchitis (PB) is a rare disease that often occurs in patients with congenital heart disease (CHD) who have undergone staged single ventricle palliation. It is characterized by the formation of rubbery “casts” in the airways. PB treatment frequently includes inhaled tPA. However, the efficacy of tPA to reduce cast burden is unknown. This is further complicated by our lack of knowledge of cast composition. We obtained spontaneously expectorated PB casts from children (n=4) with CHD and one adult patient with idiopathic PB. Pathological assessment was made from paraffin-preserved samples. Casts were treated with phosphate-buffered saline (PBS) or tPA. Cast response to tPA was assessed by changes in cast weight and the production of fibrin D-dimer. Independent of dose, tPA reduced cast weight compared with PBS-treatment (p=0.001) and increased D-dimer levels. Histological staining showed that PB casts from all patients were comprised of fibrin and contained notable numbers of lymphocytes. Cast composition did not change over time. Collectively, these data support that in our PB patients, casts are comprised of fibrin and are responsive to tPA treatment. This makes inhaled tPA a potentially viable option for symptomatic relief of PB while we work to unravel the complexity of PB pathogenesis.
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影响因子：
8
作者：
Costello, JM;Steinhorn, D;Kumar, SP
通讯作者：
Kumar, SP
影响因子：
4.6
作者：
Salman, S.;Shah, A.;Christian, Karla G.
通讯作者：
Christian, Karla G.
DOI：
10.1165/rcmb.2006-0040oc
发表时间：
2006-12-01
影响因子：
6.4
作者：
Chu, Eric K.;Cheng, Jason;Drazen, Jeffrey M.
通讯作者：
Drazen, Jeffrey M.
DOI：
10.1016/j.ijporl.2010.02.005
发表时间：
2010-07-01
影响因子：
1.5
作者：
Preciado, Diego;Verghese, Susan;Choi, Sukgi
通讯作者：
Choi, Sukgi
DOI：
10.1111/j.1432-1033.1984.tb08132.x
发表时间：
1984-01-01
期刊：
EUROPEAN JOURNAL OF BIOCHEMISTRY
影响因子：
--
作者：
SUENSON, E;LUTZEN, O;THORSEN, S
通讯作者：
THORSEN, S