隨著肺泡毛細血管屏障破壞的進展,高分子量蛋白質、細胞和液體滲漏到肺泡腔。最終,基底內皮細胞和上皮細胞膜遭到破壞,導致肺泡出血。
HAPE 的一個顯著特徵是,隨著海拔下降或簡單地給予氧氣,由於肺血管阻力和肺動脈壓力立即下降,這個過程可以迅速逆轉。
High mean pulmonary artery pressure, over 35 to 40 mmHg, appears to be the initiating event. However, while elevated pulmonary artery pressure is essential for HAPE, it is insufficient. The other necessary factor is uneven vasoconstriction. Specific segmental and subsegmental capillary beds with relatively less vasoconstriction are disproportionately exposed to elevated microvascular pressures (>20 mmHg) from the elevated mean pulmonary artery pressure. This uneven vasoconstriction and regional overperfusion result in failure of the alveolar-capillary barrier and patchy pulmonary edema [6].
As disruption of the alveolar-capillary barrier progresses, high molecular weight proteins, cells, and fluid leak into the alveolar space. Eventually, basement endothelial and epithelial cell membranes are disrupted, leading to alveolar hemorrhage.
A striking feature of HAPE is the rapid reversibility of this process with descent or simply the administration of oxygen, due to the immediate drop in pulmonary vascular resistance and pulmonary artery pressure.
DIAGNOSIS
HAPE is typically diagnosed clinically based on the history and examination findings. The initial symptoms typically begin two to four days after arrival at high altitude, including a subtle nonproductive cough, shortness of breath on exertion, and difficulty walking uphill. Symptoms can develop more precipitously in children. Over one to two days, the cough often becomes productive. Early progression from dyspnea with exertion to dyspnea at rest is a cardinal feature. Prominent examination findings include tachycardia, tachypnea, low-grade fever (up to 38°C), and pulmonary crackles. Oxygen saturation is usually at least 10 points lower than expected for a given altitude. Treatment with supplemental oxygen and rest can lead to rapid improvement. When available, characteristic findings on imaging studies help confirm the diagnosis.
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