Steinhorn R H, Cox P N, Fineman J R, Finer N N, Rosenberg E M, Silver M M, Tyebkhan J, Zwass M S, Morin F C
Department of Pediatrics, Children's Hospital, Buffalo, NY 14222, USA.
J Pediatr. 1997 Mar;130(3):417-22. doi: 10.1016/s0022-3476(97)70203-8.
A complex vascular abnormality in the lungs, termed alveolar capillary dysplasia (ACD) and misalignment of the lung vessels, has been recently recognized in some infants with persistent pulmonary hypertension. These infants die despite maximal medical support including extracorporeal membrane oxygenation (ECMO). Inhaled nitric oxide has been reported to improve oxygenation in neonates with persistent pulmonary hypertension of the newborn, and may allow some infants to avoid the need for ECMO. We identified five infants who had received inhaled nitric oxide to treat refractory hypoxemia caused by persistent pulmonary hypertension of the newborn, and who subsequently died and had autopsy confirmation of ACD. Each infant received care at a different medical center. In each patient, inhaled NO increased the arterial partial pressure of oxygen dramatically. Despite initial clinical improvement, the response to NO was not sustained in any patient. As responsiveness was lost, each infant with ACD required inhaled NO concentrations of 80 ppm or higher to sustain oxygenation. Each infant died, four after extensive periods of ECMO support. This experience demonstrates that a short-term improvement after inhalation of nitric oxide does not lead to long-term survival in ACD. Further, in three infants the diagnosis of ACD was established by lung biopsy before death. Increasing awareness of this clinical entity may allow for the avoidance of costly, invasive procedures such as ECMO until more specific therapies become available.
肺部一种复杂的血管异常，称为肺泡毛细血管发育不良（ACD）及肺血管排列紊乱，最近在一些患有持续性肺动脉高压的婴儿中被认识到。尽管给予包括体外膜肺氧合（ECMO）在内的最大程度的医疗支持，这些婴儿仍死亡。据报道，吸入一氧化氮可改善新生儿持续性肺动脉高压患儿的氧合，且可能使一些婴儿无需接受ECMO治疗。我们确定了5名接受吸入一氧化氮治疗新生儿持续性肺动脉高压所致难治性低氧血症的婴儿，这些婴儿随后死亡且尸检证实患有ACD。每名婴儿在不同的医疗中心接受治疗。在每名患者中，吸入一氧化氮均显著提高了动脉血氧分压。尽管最初临床症状有所改善，但在任何患者中对一氧化氮的反应均未持续。随着反应性丧失，每名患有ACD的婴儿都需要吸入浓度为80 ppm或更高的一氧化氮来维持氧合。每名婴儿均死亡，其中4名在接受长时间ECMO支持后死亡。这一经验表明，吸入一氧化氮后的短期改善并不能使ACD患儿长期存活。此外，在3名婴儿中，在死亡前通过肺活检确诊为ACD。提高对这一临床实体的认识可能有助于避免在更具体的治疗方法出现之前进行诸如ECMO等昂贵的侵入性操作。
