Recent insights into the pathogenesis and therapeutics of pulmonary hypertension

被引：25

作者：

Strange, JW ^{[1
]}

Wharton, J ^{[1
]}

Phillips, PG ^{[1
]}

Wilkins, MR ^{[1
]}

机构：

[1] Hammersmith Hosp, Imperial Coll, Clin Pharmacol Sect, London W12 0NN, England

来源：

CLINICAL SCIENCE | 2002年 / 102卷 / 03期

关键词：

BMP type II receptor; genetic predisposition; hypoxia; pulmonary hypertension; therapeutics; transforming growth; factor beta receptor;

D O I：

10.1042/CS20010212

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

The normal adult pulmonary circulation is a low-pressure, high-capacity circuit. Pulmonary vascular resistance is regulated by alveolar oxygen tension, potassium channels and a variety of locally produced and circulating vasoactive factors. Perturbations of these systems may contribute to the pathogenesis of pulmonary hypertension. Recently, mutations in BMPR2 and ALK-1, genes that encode members of the transforming growth factor-beta (TGF-beta) receptor superfamily, have been found in patients with primary pulmonary hypertension. These observations provide a novel insight into the pathogenesis of primary pulmonary hypertension, and emphasize the importance of the integrity of the TGF-beta receptor family in the maintenance of normal pulmonary vascular structure and function. This review discusses the latest developments in the field of pulmonary vascular biology and the prospects for improving the treatment of pulmonary hypertension.

引用

页码：253 / 268

页数：16

共 156 条

[1] Appetite-suppressant drugs and the risk of primary pulmonary hypertension [J].

Abenhaim, L ;

Moride, Y ;

Brenot, F ;

Rich, S ;

Benichou, J ;

Kurz, X ;

Higenbottam, T ;

Oakley, C ;

Wouters, E ;

Aubier, M ;

Simonneau, G ;

Begaud, B .

NEW ENGLAND JOURNAL OF MEDICINE, 1996, 335 (09) :609-616

[2]

Abrams D, 2000, Heart, V84, pE4, DOI 10.1136/heart.84.2.e4

[3] PULMONARY-HYPERTENSION PREDICTS MORTALITY AND MORBIDITY IN PATIENTS WITH DILATED CARDIOMYOPATHY [J].

ABRAMSON, SV ;

BURKE, JF ;

KELLY, JJ ;

KITCHEN, JG ;

DOUGHERTY, MJ ;

YIH, DF ;

MCGEEHIN, FC ;

SHUCK, JW ;

PHIAMBOLIS, TP .

ANNALS OF INTERNAL MEDICINE, 1992, 116 (11) :888-895

[4] NIFEDIPINE REDUCES PULMONARY PRESSURE AND VASCULAR TONE DURING SHORT-TERM BUT NOT LONG-TERM TREATMENT OF PULMONARY-HYPERTENSION IN PATIENTS WITH CHRONIC OBSTRUCTIVE PULMONARY-DISEASE [J].

AGOSTONI, P ;

DORIA, E ;

GALLI, C ;

TAMBORINI, G ;

GUAZZI, MD .

AMERICAN REVIEW OF RESPIRATORY DISEASE, 1989, 139 (01) :120-125

[5] Vascular remodeling and ET-1 expression in rat strains with different responses to chronic hypoxia [J].

Aguirre, JI ;

Morrell, NW ;

Long, L ;

Clift, P ;

Upton, PD ;

Polak, JM ;

Wilkins, MR .

AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY, 2000, 278 (05) :L981-L987

[6] Primary pulmonary hypertension - A vascular biology and translational research "work in progress" [J].

Archer, S ;

Rich, S .

CIRCULATION, 2000, 102 (22) :2781-2791

[7] A placebo-controlled trial of prostacyclin in acute respiratory failure in COPD [J].

Archer, SL ;

Mike, D ;

Crow, J ;

Long, W ;

Weir, EK .

CHEST, 1996, 109 (03) :750-755

[8] Molecular identification of the role of voltage-gated K+ channels, Kv1.5 and Kv2.1, in hypoxic pulmonary vasoconstriction and control of resting membrane potential in rat pulmonary artery myocytes [J].

Archer, SL ;

Souil, E ;

Dinh-Xuan, AT ;

Schremmer, B ;

Mercier, JC ;

El Yaagoubi, A ;

Nguyen-Huu, L ;

Reeve, HL ;

Hampl, V .

JOURNAL OF CLINICAL INVESTIGATION, 1998, 101 (11) :2319-2330

[9] Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease -: A randomized, controlled trial [J].

Badesch, DB ;

Tapson, VF ;

McGoon, MD ;

Brundage, BH ;

Rubin, LJ ;

Wigley, FM ;

Rich, S ;

Barst, RJ ;

Barrett, PS ;

Kral, KM ;

Jöbsis, MM ;

Loyd, JE ;

Murali, S ;

Frost, A ;

Girgis, R ;

Bourge, RC ;

Ralph, DD ;

Elliott, CG ;

Hill, NS ;

Langleben, D ;

Schilz, RJ ;

McLaughlin, VV ;

Robbins, IM ;

Groves, BM ;

Shapiro, S ;

Medsger, TA ;

Gaine, SP ;

Horn, E ;

Decker, JC ;

Knobil, K .

ANNALS OF INTERNAL MEDICINE, 2000, 132 (06) :425-+

[10] RELATION OF RIGHT VENTRICULAR EJECTION FRACTION TO EXERCISE CAPACITY IN CHRONIC LEFT-VENTRICULAR FAILURE [J].

BAKER, BJ ;

WILEN, MM ;

BOYD, CM ;

DINH, H ;

FRANCIOSA, JA .

AMERICAN JOURNAL OF CARDIOLOGY, 1984, 54 (06) :596-599

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