Pediatric Cardiology and Pulmonology Unit, Hospital Arnaud de Villeneuve, Montpellier, France.
Pediatr Pulmonol. 2010 Jan;45(1):99-102. doi: 10.1002/ppul.21128.
We report the case of a 5-year-old child with dyskeratosis congenita who presented cyanosis and dyspnea at exertion. He had severe hypoxemia with elevated alveolar-arterial oxygen gradient in the setting of liver disease. Technetium-99m-labeled macroaggregated albumin scan showed abnormally high uptake in the brain, confirming hepatopulmonary syndrome.
我们报告了一例先天性角化不良的 5 岁儿童，其在活动时出现发绀和呼吸困难。他患有肝病，存在严重的低氧血症和肺泡-动脉氧梯度升高。锝-99m 标记的巨聚合白蛋白扫描显示大脑异常摄取，证实存在肝肺综合征。
