论文部分内容阅读
目的分析儿童特发性肺含铁血黄素沉着症(IPH)临床特点、治疗及预后,为临床防治该病提供依据。方法回顾性分析2005年1月-2016年6月在西京医院首次诊断并完成1年以上随访的36例IPH患儿的临床资料,根据单用泼尼松口服治疗时间进行分组,比较各组复发情况;根据病程中是否有反复发作进行分组,分析反复发作的危险因素。结果36例患儿中,男20例、女16例,发病中位年龄4.3岁,确诊中位年龄6岁,主要临床表现为咳嗽、咯血、气促、贫血、发热、乏力等。急性发作期糖皮质激素治疗能控制症状,单用泼尼松口服治疗≤1年组、~2年组、~3年组及>3年组中,不同时间的复发率差异无统计学意义(P>O.05)。误诊时间(OR=0.084,95%CI:0.01~0.93)及初诊咯血史(OR=0.02,95%CI:0~0.461)是IPH反复发作的独立危险因素。结论 IPH临床表现多样,易误诊,糖皮质激素可较好的控制急性期病情,延长糖皮质激素治疗时间对IPH复发无影响。应尽早诊断,及时正规治疗,有助于缓解症状,延缓肺纤维化进程,改善预后。
Objective To analyze the clinical features, treatment and prognosis of idiopathic pulmonary hemosiderosis (IPH) in children and provide the basis for clinical prevention and treatment of this disease. Methods The clinical data of 36 patients with IPH who were diagnosed for the first time and were followed up for more than one year at Xijing Hospital from January 2005 to June 2016 were retrospectively analyzed. The patients were divided into groups according to the oral administration time of prednisone alone. The recurrence rate of each group was compared According to the course of the disease whether there are recurrent episodes were grouped to analyze the risk factors for recurrent episodes. Results Among the 36 children, 20 were males and 16 were females. The median age was 4.3 years. The median age at diagnosis was 6 years. The main clinical manifestations were cough, hemoptysis, anemia, fever, fatigue and so on. Acute exacerbation of glucocorticoid treatment can control the symptoms, with prednisone alone ≤ 1 year group, ~ 2 years group, ~ 3 years and> 3 years group, the recurrence rate at different time was no significant difference P> O.05). Misdiagnosis time (OR = 0.084, 95% CI: 0.01-0.93) and history of newly diagnosed hemoptysis (OR = 0.02,95% CI: 0-0.461) were independent risk factors for recurrent IPH. Conclusions The clinical manifestations of IPH are diverse and easily misdiagnosed. Glucocorticoid can better control the acute phase disease and prolonging the treatment time of glucocorticoid has no effect on the recurrence of IPH. Should be diagnosed as soon as possible, regular formal treatment, help to relieve symptoms, delay pulmonary fibrosis, improve prognosis.