Long-term prognosis of allergic bronchopulmonary aspergillosis in children with cystic fibrosis
10.3760/cma.j.cn101070-20201217-01907
- VernacularTitle:囊性纤维化并变应性支气管肺曲霉病患儿的远期预后
- Author:
Xiaolei XU
1
;
Hao WANG
;
Ju YIN
;
Mingxue MU
;
Jun LIU
;
Qiang QIN
;
Xiaomin DUAN
;
Yun PENG
;
Chanjuan HAO
;
Baoping XU
;
Kunling SHEN
Author Information
1. 国家儿童医学中心，首都医科大学附属北京儿童医院呼吸科，国家呼吸系统疾病临床医学研究中心，北京 100045
- Keywords:
Cystic fibrosis;
Allergic bronchopulmonary aspergillosis;
Treatment;
Prognosis
- From:
Chinese Journal of Applied Clinical Pediatrics
2021;36(12):949-953
- CountryChina
- Language:Chinese
-
Abstract:
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.
