Tobai Hiromi, Yano Jun, Sato Norio, Amanuma Fumitaka, Takahashi Mikio, Endo Mikiya, Ishimura Masataka, Ohga Shouichi, Maruyama Hidekazu
Department of Pediatrics, Iwate Prefectural Iwai Hospital, Ichinoseki, Japan.
Department of Pediatrics, Graduate School of Medical Sciences, Kyusyu University, Fukuoka, Japan.
Case Rep Pediatr. 2020 Apr 25;2020:5292947. doi: 10.1155/2020/5292947. eCollection 2020.
Idiopathic pulmonary hemosiderosis (IPH) is a rare and life-threatening disorder. Early diagnosis and appropriate management are essential for their better prognosis and patients' quality of life (QOL). It is considered that Down syndrome patients with IPH have a worse prognosis compared to other IPH cases. A 2-year-old girl with Down syndrome received the diagnosis of IPH after two episodes of massive pulmonary hemorrhage requiring assist ventilation, who suffered from recurrent IPH during tapering period of oral corticosteroid, started liposteroid therapy. We report here a case of successful control of recurrent IPH and improved QOL enormously with tapering dose of corticosteroid after starting liposteroid therapy.
特发性肺含铁血黄素沉着症(IPH)是一种罕见且危及生命的疾病。早期诊断和恰当治疗对于改善其预后及患者生活质量(QOL)至关重要。一般认为,患有IPH的唐氏综合征患者相较于其他IPH病例预后更差。一名2岁唐氏综合征女童在经历两次需要辅助通气的大量肺出血后被诊断为IPH,在口服糖皮质激素减量期间出现IPH复发,遂开始脂质体类固醇治疗。我们在此报告一例在开始脂质体类固醇治疗后,通过逐渐减量糖皮质激素成功控制IPH复发并极大改善生活质量的病例。