Li Ya-Ting, Guo Yu-Xiong, Cai Liang-Ming, Pan Li, Duan Meng-Qi, Yang Li-Fen, Sun Yue-Yu, Tan Wei-Ping, Chen Zhuang-Gui
Pediatric Intensive Care Unit, Department of Pediatrics, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou 510630, PR China.
Pediatric Intensive Care Unit, Department of Pediatrics, Guangdong General Hospital, Guangdong Academic of Medical Sciences, Guangzhou 510080, PR China.
Am J Emerg Med. 2017 Nov;35(11):1786.e3-1786.e7. doi: 10.1016/j.ajem.2017.07.094. Epub 2017 Jul 31.
Idiopathic pulmonary hemosiderosis (IPH) is an extremely rare cause of massive pulmonary hemorrhage in children. During the acute phase, death due to massive alveolar hemorrhage and subsequent severe respiratory failure. We report two cases of IPH children who developed hypoxemic respiratory failure and massive pulmonary hemorrhage. One case of a 10-year-old boy was treated with methylprednisolone pulse therapy (10mg/kg/d) for the first three days and followed by systemic steroid therapy, he successfully decannulated 10days later and discharged with a favorable quality of life. Another case of a 4year-old female child with Down's syndrome diagnosed as IPH for over one year and treated with oral corticosteroids for maintenance therapy. She sudden suffered severe hypoxemia with rapid falls in the hemoglobin level. We applied methylprednisolone pulse therapy (10mg/kg/d) for three days and other supportive therapies, the girl survived through complicated with oxygen dependence. We suggest that methylprednisolone pulse therapy provides a chance of recovery and survival for patients with IPH at the acute phase, even if accompanied by severe pulmonary hemorrhage.
特发性肺含铁血黄素沉着症(IPH)是儿童大量肺出血的极其罕见的病因。在急性期,可因大量肺泡出血及随后的严重呼吸衰竭而死亡。我们报告两例发生低氧性呼吸衰竭和大量肺出血的IPH患儿。一例为10岁男孩,最初三天接受甲泼尼龙冲击治疗(10mg/kg/d),随后进行全身类固醇治疗,10天后成功拔管,出院时生活质量良好。另一例为4岁患有唐氏综合征的女童,诊断为IPH超过一年,接受口服皮质类固醇维持治疗。她突然出现严重低氧血症,血红蛋白水平迅速下降。我们应用甲泼尼龙冲击治疗(10mg/kg/d)三天及其他支持治疗,该女童虽存活但并发氧依赖。我们认为,甲泼尼龙冲击治疗为急性期IPH患者提供了康复和生存的机会,即使伴有严重肺出血。