以呼吸窘迫為表現的特發性肺血鐵質沉積症：一病例報告

特發性肺血鐵質沉積症（Idiopathic pulmonary hemosiderosis）在小兒科非常罕見，很容易被忽略。我們報告一個女童在兩歲五個月大時第一次發病的經過及其後將近二年間的診斷、治療及追蹤的結果。本病例的肺部病狀僅有呼吸窘迫而沒有咳血或肺出血。除了嚴重缺鐵性貧血外，臨床表現類似急性呼吸窘迫症候群。支氣管肺泡沖洗液中可見到大量含血鐵質的巨嗜細胞，經一系列徹底的檢查排除其他疾病可能性後，才確立特發性肺血鐵質沉積症的診斷。在遇到缺鐵性貧血伴有呼吸窘迫的病人時，若胸部X光有瀰漫性肺部浸潤，即使沒有咳血的症狀，肺泡出血也必須要列入鑑別診斷。除了心臟學及血清免疫學檢查外，支氣管肺泡沖洗液的細胞學檢查或更進一步的肺生檢是必要的。

關鍵字

肺血鐵質沉積症；肺泡出血；缺鐵性貧血；呼吸窘迫

並列摘要

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease in children. We report a girl who had the first episode of respiratory distress at age of 2 years and 5 months. Her clinical presentations were similar to acute respiratory syndrome accompanied with severe iron-deficiency anemia. Iron stain of bronchoalveolar lavage fluid showed many hemosiderin-laden macrophages. Diagnosis of IPH was established by the exclusion of known causes for pulmonary hemorrhage. Facing an anemic patient with recurrent episodes of respiratory distress and diffuse infiltrates on chest radiograph, the possibility of alveolar hemorrhage should be considered, even if the patient has no hemoptysis. In addition to cardiological and immunological studies, bronchoalveolar lavage fluid cytology and further lung biopsy are necessary for definite diagnosis.

並列關鍵字

pulmonary hemosiderosis ； alveolar hemorrhage ； iron deficiency anemia ； respiratory distress

國際替代計量

以呼吸窘迫為表現的特發性肺血鐵質沉積症：一病例報告

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