弥漫性肺泡出血4例

Diffuse alveolar hemorrhage in 4 girls

摘要:

本文报道4例以反复咳嗽、贫血为表现的患儿，均为女性，起病年龄均为4岁以内，3例有气促，均无急性咯血表现；肺泡灌洗液含铁血黄素试验均阳性；影像学呈毛玻璃样3例，间质改变1例。其中3例按照特发性肺含铁血黄素沉着症 （IPH）治疗无效。4例患者行选择性支气管动脉造影术，均提示存在支气管动脉-肺循环瘘，确诊为支气管动脉-肺循环瘘 （BPS），均经导管行栓塞治疗。术后随访半年，均未因咳嗽、贫血再入院。BPS是一类病因不明的支气管动脉与肺动脉或肺静脉之间异常分流的疾病，儿童少见，对于初诊考虑IPH但激素治疗效果不佳的弥漫性肺泡出血患者需警惕该病。

Abstract:

This article reports 4 girls with clinical manifestations of recurrent cough and anemia. The age of onset was less than 4 years, and three of them had shortness of breath. None of them had acute hemoptysis. All the girls had positive results of hemosiderin test for bronchoalveolar lavage fluid. As for imaging examination, 3 patients had ground-glass opacity, and 1 had interstitial change. Three girls were given the treatment for idiopathic pulmonary hemosiderosis and had no response. Selective bronchial arteriography was performed for the 4 girls and found bronchial artery to pulmonary circulation shunt (BPS). After they were diagnosed with BPS, they were given transcatheter embolization. The girls were followed up for half a year after surgery, and none of them was readmitted due to "cough and anemia". BPS manifests as abnormal shunt between the bronchial artery and the pulmonary artery/vein and has unknown causes. It is rare in children and should be considered for children who were thought to have idiopathic pulmonary hemosiderosis and had poor response to corticosteroid therapy.