摘要
肺动脉高压是一大类以肺动脉阻力进行性升高为特点的心血管疾病,可导致右室肥厚、右心功能不全。其定义为静息时肺动脉平均压〉25mmHg(1mmHg=0.133kPa),运动时〉30mmHg,肺毛细血管楔压(PCWP)≤15mmHg。1954年Dresdale等首次报道了家族性原发性肺动脉高压(FPPH)。2003年在威尼斯召开的WHO第三次肺动脉高压专家工作组会议制定的最新诊断分类将诊断名称更改为家族性肺动脉高压(FPAH),其基本病理特点是肌型小动脉丛样病变,
出处
《中华心血管病杂志》
CAS
CSCD
北大核心
2007年第11期1063-1066,共4页
Chinese Journal of Cardiology
参考文献32
-
1Sztrymf B, Yaici A, Jais X, et al. Genetics of pulmonary arterial hypertension: recent data and practical applications. Rev Mal Respir,2005,22(5 Pt 1):796-805.
-
2Dresdale DT, Michtom RJ, Shultz M. Recent studies in primary pulmonary hypertension, including pharmacodynamic observations on pulmonary vascular disease. Bull N Y Acad Med, 1954,30 (3) :195-207.
-
3Rich S, Dantzer DR, Ayres SM, et al. Primary pulmonary hypertension-a national prospective study. Ann Intern Med, 1987, 107(2) : 216-223.
-
4Nichols WC, Koller DL, Slovis B, et al. Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31- 32. Nat Genet, 1997,15 (3) :277-280.
-
5Lane KB, Machado RD, Pauciulo MW, et al. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet,2000,26(1):81-84.
-
6陆立鹤.骨形态生成蛋白Ⅱ型受体基因与原发性肺动脉高压[J].中国分子心脏病学杂志,2001,1(1):37-40. 被引量:2
-
7Austin Ed,Loyd Je. Genetics and mediators in pulmonary arterial hypertension. Clin Chest Med,2007,28( 1 ):43-57.
-
8Cogan JD, Pauciulo MW, Batchman AP, et al. High frequency of BMPR2 exonic deletions/duplications in familial pylmonary arterial hypertension. Am J Respir Crit Care Med, 2006, 174 (5) : 590- 598.
-
9荆志成,陆立鹤,邹玉宝,尤世杰,韩志岩,张芊,杨跃进,惠汝太,程显声.一例原发性肺动脉高压家族的临床与遗传学特点[J].中华医学杂志,2004,84(3):199-202. 被引量:3
-
10Humbert M, Deng Z, Simonneau G, et al. BMPR2 germline mutations in pulmonary hypertension associated with fenfluramine deft vatives. Eur Respir J,2002,20 (3) : 518 -523.
二级参考文献40
-
1冉丕鑫,钟南山.生长因子、原癌基因与低氧性肺动脉高压[J].基础医学与临床,1996,16(1):1-5. 被引量:24
-
2[1]Nichols WC, Koller DL, Slovis B, et al. Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31-32. Nat Genet 1997; 15:277-280.
-
3[2]Morse JH, Jones AC, Barst RJ, et al. Mapping of familial primary pulmonary hypertension locus(PPH1)to chromosome 2q31-32. Circulation 1997; 95: 2603-2606.
-
4[3]Rubin LJ. Primary pulmonary hypertension. N Engl J Med 2000; 336: 111-117.
-
5[5]Thomson JR, Machado RD, Pauciulo MW, et al.Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR- Ⅱ,a receptor of the TGF- β family. J Med Genet 2000; 37:741-745.
-
6[6]Pietra GC, Edwards WD, Kay JM, et al. A Qualitative and Quantitative Study of Pulmonary Blood Vessels From 58 Patients in the National Heart, Lung,and Blood Institute, Primary Pulmonary Hypertension Registry. J Circulation 1989, 80: 1198-1206.
-
7[7]Tuder RM, Groves B, Badesch DB. et al. Exuberant endothelial cell growth and elements of inflammation are present in plexiform lessions of pulmonary hypertension. Am J Pathol 1994; 144: 275-285.
-
8[8]Cool CD, Stewart JS, Werahera P, et al Threedimensional reconstruction of pulmonary arteries in plexiform pulmonary hypertension using cell-specific marlcer: evidence for a dynamic and heterogeneous process of pulmonary endo-thelial cell growth. Am J Pathol 1999; 155: 411-419.
-
9[9]Gomez-Sanchez MA, de la Saenz C, Gomez-Pajuelo C,et al. Clinical and pathologic manifestations of pulmonary vascular disease in the toxic oil syndtome. J Am Coil Cardiol 1991; 18: 1539-1539.
-
10[10]Weir EK, Reeve HL, Huang JMC, et al. Anorexic agents aminorex,fenfluramine and dexfentluramine inhibit K current in rat pulmonary vascular smooth muscle and causepulmonary vasoconstriction. Circulation 1996; 94: 2216-2220.
共引文献11
-
1汤华.产后原发性肺动脉高压1例[J].临床和实验医学杂志,2006,5(1):83-83.
-
2王金祥,石逸杰,胥振扬,刘宇毅.家族性肺动脉高压二例[J].中国呼吸与危重监护杂志,2006,5(6):463-463.
-
3黄素芳,赵霞霞,姚笠,胡孟英.原发性肺动脉高压一例报告[J].中国优生与遗传杂志,2007,15(9):101-101.
-
4郭相杰,高彩荣,孙俊红,任广睦,梁新华,郑伟.尸检确诊原发性肺动脉高压1例报告[J].山西医科大学学报,2007,38(12):1127-1129.
-
5王毅,解卫平.肺动脉高压发病机制的研究进展[J].临床肺科杂志,2010,15(11):1621-1623. 被引量:26
-
6樊辉娟,高芬.低氧性肺动脉高压相关基因的研究进展[J].国际呼吸杂志,2011,31(5):388-392.
-
7廖驰林.常见先天性心脏病合并肺动脉高压的治疗现状与对策[J].中国社区医师(医学专业),2012,14(15):205-206. 被引量:2
-
8刘辉,刘锦铭,郭建,杨文兰,史小芳,赵勤华,王岚.摄氧效率与肺动脉高压患者运动耐力的相关性[J].中华医学杂志,2015,95(6):420-424. 被引量:2
-
9段永建,易群.肺动脉高压病因及治疗研究进展[J].西部医学,2016,28(8):1178-1180. 被引量:13
-
10张静静,王岗,李满祥.内皮-间充质转化在肺动脉高压发病机制中的研究进展[J].中国病理生理杂志,2018,34(9):1724-1728. 被引量:4
同被引文献21
-
1Austin ED,Loyd JE. Genetics and mediators in pulmonary hypertension [ J ]. Glin Chest Med, 2007,28 ( 1 ) :43 - 57.
-
2Roberts KE, McElroy JJ, Wong WP, et al. BMPR2 mutations in pulmona- ry arterial hypertension with congenital heart disease [ J ]. Eur Res J, 2004,24 ( 3 ) : 371 - 374.
-
3Johri S, Dunnington GH, Vnencak - Jones CL. A novel BMPR2 mutation associated with pulmonary arterial hypertension in an octogenarian [ J ]. Lung,2010,188(4) :349 -352.
-
4Aldred MA, Comhair SA, Varella - Garcia M, et al. Somatic chromosome abnormalities in the lungs of patients with pulmonary arterial hyperten- sion[J] .Am J Respir Crit Care Meal,2010,182(9) :1153 - 1160.
-
5Chida A, Shintani M, Nakayama T, et al. Missense mutations of the BM PR1 B (ALK6)gene in childhood idiopathic pulmonary arterial hyperten- sion[J]. Circ J,2012 [ Epub ahead of print].
-
6Girerd B, Montani D, Eyries M, et al. Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hy- pertension [ J ]. Respir Res,2010,11:73.
-
7Pfarr N, Szamalek - Hoegel J, Fischer C, et al. Hemodynamic and clini- cal onset in patients with hereditary pulmonary arterial hypertension and BMPR2 mutations[ J]. Respir Res ,2011,12:99.
-
8Girerd B, Montani D, Coulet F ,et al. Clinical outcomes of pulmonary arterial hypertension in patients carrying an ACVRL1 ( ALK1 ) mutation [J].Am J Respir Crit Care Med,2010 ,181( 8 ) :851 -861.
-
9De Gottardi A, Seijo S, Milo M, et al. Bone morphogenetic protein receptor 2 in patients with idiopathic portal hypertension [ J ]. J Cell Mol Med,2011 [ Epub ahead of print].
-
10Morrell NV~. Role of bone morphogenetie protein receptors in the develop- ment of pulmonary arterial hypertension [ J ]. Adv Exp Med Biol, 2010, 661:251 -264.
引证文献3
-
1魏美丽,韩波,王来城,孙瑾.先天性心脏病并肺动脉高压患儿骨形成蛋白Ⅱ型受体基因新突变[J].实用儿科临床杂志,2012,27(13):994-996. 被引量:2
-
2杨子云,蒋婷婷,葛春光,贾晓东,刘清发,王琪,彭兰博,兰菲,齐军.基于蛋白质互作网络的COVID⁃19对肺动脉高压的影响机制[J].生物信息学,2022,20(3):195-202.
-
3宋金芮,张静文,李博,邓明君,曹云山.骨形态发生蛋白受体2 c.282突变致肺动脉高压1家系[J].中华心血管病杂志,2023,51(7):769-772.
-
1荆志成,程显声,邹玉宝,尤世杰,裴卫东,韩志岩,杨跃进,惠汝太.中国汉族人群家族性原发性肺动脉高压二家系及文献复习[J].中华结核和呼吸杂志,2002,25(11):684-685. 被引量:2
-
2何富强,唐智,吴宗照.前列腺素E_1治疗慢性肝炎和重型肝炎疗效探讨[J].华南国防医学杂志,1995,9(1):59-60. 被引量:1
-
3陈伟丹,李守军.西地那非治疗先天性心脏病相关肺动脉高压[J].中国心血管杂志,2010,15(2):151-153. 被引量:3
-
4荆志成,徐希奇,邓可武.甲状腺疾病与特发性肺动脉高压[J].中华医学信息导报,2004,19(17):15-15.
-
5王金祥,石逸杰,胥振扬,刘宇毅.家族性肺动脉高压二例[J].中国呼吸与危重监护杂志,2006,5(6):463-463.
-
6李巧章.急性冠状动脉综合征107例临床分析[J].岭南急诊医学杂志,2012,17(5):341-342.
-
7郭志刚,侯玉清,马立勤,屠燕.急性冠状动脉综合征非ST段抬高患者心血管危险因素及其影像学特征[J].中国临床康复,2005,9(35):76-78.
-
8王宝恩.慢性肝炎的诊断、分级与分期[J].中华内科杂志,1995,34(4):223-225. 被引量:8
-
9荆志成,徐希奇.攻克肺动脉高压的长征之路:虽艰难但前途光明[J].中华内科杂志,2013,52(8):628-630. 被引量:7
-
10康健,于娜.肺动脉高压药物治疗的新进展[J].中华结核和呼吸杂志,2007,30(9):654-656. 被引量:7