Pulmonary function analysis of 21 children with idiopathic pulmonary hemosiderosis / 中华实用儿科临床杂志

ABSTRACT

Objective To evaluate the injury of pulmonary function of children with idiopathic pulmonary hemosiderosis(IPH) and the changes after treatment,and to provide some guidance for the diagnosis and treatment of IPH.Methods Twenty-one children with IPH who were admiued at Children's Hospital of Fudan University between June 2012 and May 2016 were selected.The pulmonary function and clinical data of them were analyzed.Results The general pulmonary function of 21 children with IPH before treatment with glucocorticoid was reported that 4 cases (19.05％) were normal and 17 cases (80.95％) were abnormal,including 11 cases (52.38％) with restrictive ventilatory disorder,4 cases (19.05％) with mixed ventilatory disorder,1 case (4.76％) with obstructive ventilatory disorder,and 1 case (4.76％) with small airway dysfunction.Pulmonary function test was performed on 15 cases after 1-2 months of treatment with glucocorticoid.The results showed that maximal vital capacity (VCmax％) vs.the expected value was (77.91 ± 18.86)％ vs.(60.43 ± 23.70)％,forced vital capacity (FVC％) vs.the expected value was (78.96 ±19.24)％ vs.(61.03 ±24.62)％ and forced expiratory volume in one second (FEV1％) vs.the expected value was (86.03 ± 21.69) ％ vs.(65.17 ± 26.89) ％,which were significantly higher than those before treatment,and the differences were statistically significant (t =-4.13,-4.01,-4.54,all P ＜ 0.05).Three cases were followed up for 18 to 40 months by detecting pulmonary function and the results of dynamic monitoring of pulmonary function showed a fluctuation in FVC％ [case 1(69.6-84.2) ％;case 2(56.1-73.7) ％;case 3(40.4-70.2) ％].Conclusion The characteristic pulmonary function changes in children with IPH are restrictive ventilatory disorder.Pulmonary function test play a significant role in diagnosis,treatment and prognosis of IPH.