Ohga S, Nomura A, Suga N, Hikino S, Kira R, Matsuzaki A, Masuda K, Ueda K
Department of Paediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Eur J Pediatr. 1994 Sep;153(9):687-90. doi: 10.1007/BF02190693.
We describe two Japanese children with idiopathic pulmonary haemosiderosis (IPH), whose refractory haemorrhages were treated with an intravenous lipid emulsion containing dexamethasone (liposteroid). A 22-month-old boy and a 14-month-old girl have been observed with similar symptoms; periodic bouts of anaemia, reticulocytosis, diffuse infiltrates on chest X-ray and the finding of siderophages in sputum or gastric lavage fluid. The MRI of the lung was useful for the diagnosis. Methylprednisolone pulse therapy was successful in treating acute massive bleeding. Subsequent oral prednisolone could not prevent chronic recurrent haemorrhages. However, the intermittent administration of liposteroid (0.05 mg/kg/dose IV) led to a cessation of bleeding; the haemoglobin concentration rose to normal levels. This observation emphasizes the usefulness of liposteroid in the management of refractory IPH.
我们描述了两名患有特发性肺含铁血黄素沉着症（IPH）的日本儿童，他们难治性出血采用含地塞米松的静脉脂质乳剂（脂类固醇）治疗。一名22个月大的男孩和一名14个月大的女孩表现出相似症状；周期性贫血发作、网织红细胞增多、胸部X线弥漫性浸润以及在痰液或洗胃液中发现噬铁细胞。肺部MRI对诊断有帮助。甲泼尼龙冲击疗法成功治疗了急性大量出血。随后口服泼尼松龙无法预防慢性复发性出血。然而，间歇性给予脂类固醇（0.05 mg/kg/剂量静脉注射）导致出血停止；血红蛋白浓度升至正常水平。这一观察结果强调了脂类固醇在难治性IPH治疗中的有用性。
