Sakamoto Rieko, Matsumoto Shiro, Mitsubuchi Hiroshi, Nakamura Kimitoshi
Department of Pediatrics, Faculty of Life Science, Kumamoto University, Kumamoto Japan.
Respir Med Case Rep. 2018 Mar 20;24:22-24. doi: 10.1016/j.rmcr.2018.03.011. eCollection 2018.
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease in children, with unknown etiology. The classical clinical triad is hemoptysis, hypochromic anemia and diffuse parenchymal infiltrations on chest X-ray. Liposteroid dexamethasone palmitate, which was developed in Japan, has shown good efficacy for IPH. We present the case of a patient with IPH, who suffered from a life-threatening respiratory dysfunction, and was rescued by a trial administration of liposteroid with methylprednisolone (mPSL). A 6-year-old girl was admitted to our hospital for repeated dyspnea and blood-stained sputum. She was diagnosed with IPH at the age of three-months by iron staining of gastric fluid and sputum studies. Her cumulative dose of steroids (equivalent to prednisolone (PSL)) was 1062 mg/kg. However, she could not achieve remission. We decided to initiate liposteroid therapy. We administered an infusion of liposteroid 0.8 mg/kg intravenously, for three consecutive days as a therapy for acute bleeding. After administration of liposteroid, she developed high fever with CRP elevation. We suspected that the inflammation was caused by palmitate, which is present as a lipo base in liposteroid. Hence, we added 2 mg/kg mPSL per day for 1 week. As a maintenance treatment, a single infusion of liposteroid was administered followed by mPSL administration for 6 days in every week. Her respiratory condition slowly improved. Tracheostomy was performed for airway management. She was shifted out of the ICU on the 34th day. Steroid is a key therapy for hemosiderosis. When IPH is diagnosed, oral prednisone therapy is initiated. Although this is effective, there are limitations due to significant adverse effects. Maintaining drug therapy is very important for IPH patients to keep the disease under control. Liposteroid has the same mechanism of action as dexamethasone. It has a Lipo-base, palmitate, which could induce pro-inflammatory cytokine activation. We used mPSL to inhibit the inflammation following liposteroid administration. This was effective. A combination of liposteroid and mPSL administration was useful method of treatment for the patient.
特发性肺含铁血黄素沉着症（IPH）是一种病因不明的儿童罕见疾病。典型的临床三联征为咯血、低色素性贫血以及胸部X线显示的弥漫性实质浸润。在日本研发的脂溶性类固醇地塞米松棕榈酸酯已显示出对IPH有良好疗效。我们报告一例IPH患者，该患者出现危及生命的呼吸功能障碍，通过试用脂溶性类固醇联合甲泼尼龙（mPSL）得以救治。一名6岁女孩因反复呼吸困难和痰中带血入住我院。她在3个月大时通过胃液铁染色和痰液检查被诊断为IPH。她的类固醇累积剂量（相当于泼尼松龙（PSL））为1062mg/kg。然而，她未能实现缓解。我们决定开始脂溶性类固醇治疗。我们静脉输注0.8mg/kg脂溶性类固醇，连续3天作为急性出血的治疗方法。给予脂溶性类固醇后，她出现高热且C反应蛋白升高。我们怀疑炎症是由脂溶性类固醇中作为脂质基础存在的棕榈酸酯引起的。因此，我们每天添加2mg/kg mPSL，持续1周。作为维持治疗，每周给予一次脂溶性类固醇单次输注，随后mPSL给药6天。她的呼吸状况逐渐改善。为进行气道管理实施了气管切开术。她在第34天转出重症监护病房。类固醇是治疗含铁血黄素沉着症 的关键疗法。诊断出IPH时，开始口服泼尼松治疗。虽然这有效，但由于显著的不良反应存在局限性。维持药物治疗对IPH患者控制病情非常重要。脂溶性类固醇与地塞米松具有相同的作用机制。它有一个脂质基础棕榈酸酯，可诱导促炎细胞因子激活。我们使用mPSL抑制脂溶性类固醇给药后的炎症。这是有效的。脂溶性类固醇和mPSL联合给药是该患者有用的治疗方法。
