囊性纤维化并变应性支气管肺曲霉病患儿的远期预后

Long-term prognosis of allergic bronchopulmonary aspergillosis in children with cystic fibrosis

目的分析囊性纤维化(CF)并变应性支气管肺曲霉病(ABPA)患儿的远期预后及影响因素。方法采用观察性研究,纳入2014年8月至2018年6月首都医科大学附属北京儿童医院呼吸科收治的CF合并ABPA,并进行2年以上随访的患儿。结果符合纳入标准患儿3例。其中男2例,女1例;确诊年龄分别为14岁、8岁、9岁。随访时间2~6年。所有患儿确诊后给予全身糖皮质激素联合抗真菌治疗,例1泼尼松起始剂量为0.75 mg/(kg·d),疗程超过5年,肺功能无明显减退,但存在咯痰、胸痛等临床表现,影像学检查显示病变进展,表现为糖皮质激素依赖;例2泼尼松起始剂量为2 mg/(kg·d),疗程9个月,血清总免疫球蛋白E(IgE)水平维持在正常水平,但因咯血,肺内病变包绕肺动脉,行肺叶切除术;例3泼尼松起始剂量为0.6 mg/(kg·d),疗程18个月,确诊后2年因持续性低氧血症、肺功能降低,行肺移植术。结论全身糖皮质激素联合抗真菌治疗是CF并ABPA患儿的主要治疗方法,但其疗效存在个体差异。血清总IgE水平与肺功能、胸部影像学改变不一定一致。本病总体预后不良,影响预后因素复杂,难以单一因素评估预后。

Objective To analyze the long-term prognosis and prognostic factors of allergic bronchopulmonary aspergillosis(ABPA)in children suffering from cystic fibrosis(CF).Methods An observational study was performed.All children who were admitted to the Department of Respiratory,Beijing Children′s Hospital,Capital Medical University from August 2014 to June 2018,with more than 2 years of followed up for the diagnosis of CF accompanied by ABPA were involved.Results Three children met the inclusion criteria,with 2 boys and 1 girl,and their diagnostic age were 14,8 and 9 years old,respectively.The follow-up duration ranged from 2 to 6 years.All the 3 cases were treated with systemic corticosteroids and antifungal agent.In case 1,the initial dose of prednisone was 0.75 mg/(kg·d),and the course of treatment was more than 5 years.The corticosteroid-dependent patient suffered from expectoration and chest pain,and radiographic findings indicated exacerbation,while his lung function was normal.Treating with initial dose of prednisone 2 mg/(kg·d)for 9 months,case 2 had normal serum immunoglobulin E(IgE)concentration,but his pulmonary artery was infiltrated by lesions,thus leasing to lobectomy.In case 3,the initial dose of prednisone was 0.6 mg/(kg·d),and the course of treatment was 18 months.And she developed persistent hypoxemia,and decreased pulmonary function,so lung transplantation was necessary 2 years after diagnosis.Conclusions Systemic glucocorticoid combined with antifungal therapy is the main treatment for CF with ABPA,but there are individual differences in the efficacy.The level of serum total IgE is not always consistent with lung function and chest images.The overall prognosis is poor,and it is infeasible to evaluate the prognosis by single factor.