Abnormal vascular tone in infants and children with lung hypoplasia: Findings from cardiac catheterization and the response to chronic therapy.
Abnormal vascular tone in infants and children with lung hypoplasia: Findings from cardiac catheterization and the response to chronic therapy.
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肺发育不全的婴儿和儿童血管张力异常：心导管插入术的发现和对慢性治疗的反应。
DOI：
10.1097/01.pcc.0000244401.53189.cb
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发表时间：
2006
期刊：
影响因子：
--
通讯作者：
Fineman,JeffreyR
中科院分区：
文献类型：
--
作者：
Keller,RobertaL;Moore,Phillip;Teitel,David;Hawgood,Samuel;McQuitty,John;Fineman,JeffreyR
目的：我们描述了四例因严重先天性膈疝（CDH）或先天性囊性腺瘤样畸形（CCAM）导致的患有慢性肺病和肺发育不全的婴儿和儿童慢性肺动脉高压的病例。我们报告各种条件下心导管插入术的数据：基线呼吸支持和室内空气、高氧和吸入一氧化氮挑战。我们进一步报告了单独使用西地那非或西地那非与吸入一氧化氮联合治疗的慢性肺血管扩张剂治疗后的心导管检查措施（三名患者）。设计：病例系列。地点：三级学术中心。患者：患有 CDH (n= 3) 或 CCAM (n= 1) 且有慢性肺动脉高压证据的 0-11 岁婴儿和儿童 超声心动图和肺心病（n = 3）。干预措施：导管插入术和肺血管扩张剂治疗。测量和主要结果：评估肺血管阻力、肺动脉压以及这些测量的变化。肺血管阻力 20% 的变化被认为是具有临床意义的反应。 4 名患者进行了 10 次导管插入术。所有患者在初次插管时均出现肺血管阻力和肺动脉压升高，并在吸入一氧化氮时出现显着的血管舒张。结论：CDH 和 CCAM 肺发育不全后的慢性肺病与婴儿和儿童肺血管张力异常有关，有慢性肺动脉高压的证据。长期肺血管扩张剂治疗可以改善婴儿和儿童的肺血管功能并促进肺部生长，这些婴儿和儿童在肺部快速生长的潜力期间接受治疗。
Objective:We describe four cases of chronic pulmonary hypertension in infants and children with chronic lung disease and pulmonary hypoplasia due to severe congenital diaphragmatic hernia (CDH) or congenital cystic adenomatoid malformation (CCAM). We report data from cardiac catheterization under various conditions: baseline respiratory support and room air, hyperoxic and inhaled nitric oxide challenge. We further report cardiac catheterization measures after chronic pulmonary vasodilator therapy with sildenafil alone or a combination of sildenafil and inhaled nitric oxide (three patients).Design:Case series.Setting:Tertiary academic center.Patients:Infants and children ages 0–11 yrs with CDH (n= 3) or CCAM (n= 1) with evidence of chronic pulmonary hypertension by echocardiogram and cor pulmonale (n= 3).Interventions:Catheterization and pulmonary vasodilator therapy.Measurements and Main Results:Pulmonary vascular resistance, pulmonary arterial pressure, and changes in these measures were assessed. A 20% change in pulmonary vascular resistance was considered a clinically significant response. Ten catheterizations were performed in four patients. All patients had elevated pulmonary vascular resistance and pulmonary arterial pressures at initial catheterizations and significant vasodilation during inhaled nitric oxide.Conclusions:Chronic lung disease following pulmonary hypoplasia from CDH and CCAM is associated with abnormal pulmonary vascular tone in infants and children with evidence of chronic pulmonary hypertension. Chronic pulmonary vasodilator therapy may improve pulmonary vascular function and enhance lung growth in infants and children who are treated during their period of potential for rapid lung growth.
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DOI：
--
发表时间：
1983
期刊：
影响因子：
--
作者：
N. L. Allinger;H. L. Flanagan
通讯作者：
H. L. Flanagan
影响因子：
4.1
作者：
ASKELOF, P;GUTHENBERG, C;MANNERVIK, B
通讯作者：
MANNERVIK, B
研究谷胱甘肽 S-转移酶同工酶 A2 和 C2 对芳烃和氮杂芳烃氧化物的动力学和立体化学识别。
DOI：
10.1016/0006-291x(84)90773-3
发表时间：
1984
影响因子：
3.1
作者：
Boehlert,CC;Armstrong,RN
通讯作者：
Armstrong,RN
谷胱甘肽 S-转移酶同工酶 C 对芳烃和氮杂芳烃氧化物的立体选择性。
影响因子：
2.9
作者：
Cobb,D;Boehlert,C;Lewis,D;Armstrong,RN
通讯作者：
Armstrong,RN
底物和酶促反应产物与谷胱甘肽 S-转移酶 A 的结合。
DOI：
10.1016/s0021-9258(18)50287-8
发表时间：
1979
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
I. Jakobson;M. Warholm;B. Mannervik
通讯作者：
B. Mannervik