Tepliuk T B, Soboleva S S, Tokarev Iu N, Ol'binskaia L I
Ter Arkh. 1986;58(4):81-3.
Pulmonary arterial pressure was studied using a noninvasive method in 23 patients with hereditary hemorrhagic telangiectasia (HHT) without clinical and x-ray signs of arterial-venous shunting in the lungs. It was established that pulmonary arterial hypertension was typical of HHT patients. A significant decrease in the volume and frequency of hemorrhages in 12 of 17 HHT patients was combined with a statistically significant decrease in pulmonary arterial pressure as a result of HBO therapy. It was assumed that pulmonary microcirculatory disorders played an important role in HHT pathogenesis and were expressed in metabolic derangement of a number of biologically active substances in the lungs resulting in the development of telangiectasia.
采用无创方法对23例遗传性出血性毛细血管扩张症（HHT）患者进行肺动脉压研究，这些患者无肺部动静脉分流的临床及X线征象。结果表明，肺动脉高压是HHT患者的典型表现。17例HHT患者中有12例出血的量和频率显著减少，这与高压氧治疗后肺动脉压在统计学上的显著降低相关。据推测，肺微循环障碍在HHT发病机制中起重要作用，表现为肺内多种生物活性物质的代谢紊乱，进而导致毛细血管扩张的发生。
