Aronson D C, Heymans H S, Bijlmer R P
Eur J Pediatr. 1984 Feb;141(4):251-3. doi: 10.1007/BF00572773.
A male patient with pycnodysostosis suffered from chronic respiratory insufficiency and pulmonary hypertension. This was caused by concomitant upper airway obstruction, resulting from a low implanted uvula and a long soft palate, in combination with glossoptosis and retrognathia due to the flattened mandibular angles. An inter-current respiratory infection gave rise to an acute deterioration, with right-sided heart failure, severe liver damage and coma. Surgical shortening of the soft palate was performed, after which the blood gas values returned to normal.
一名患有致密性骨发育不全的男性患者患有慢性呼吸功能不全和肺动脉高压。这是由上呼吸道梗阻引起的，上呼吸道梗阻是由于悬雍垂低位植入和软腭过长，再加上下颌角变平导致的舌后坠和下颌后缩。一次并发的呼吸道感染导致病情急性恶化，出现右侧心力衰竭、严重肝损伤和昏迷。对软腭进行了手术缩短，术后血气值恢复正常。
