Correlation Analysis Of The Ciliary Ultrastructure And Clinical Features Of The 53 Children Undergoing Bronchoscopic Ciliary Biopsy

Objective:To analyze the clinical characteristics and ciliary damage of children with bronchoscopic ciliary biopsy,as well as the differences between PCD and SCD in the literature review,so as to provide some reference basis for clinical diagnosis,therapy and prognosis evaluation.Methods:Retrospectively analyse the clinical data of the children who carried bronchoscopic ciliary biopsy in Children's Hospital affiliated to Chongqing Medical University during 2015-2018 considering the respiratory epithelium cilia damage,as well as those of the bronchoscopic ciliary biopsy children in Chinese database since 1990 with the SPSS 22.0software.Patients are divided into normal and abnormal group according to the cilium ultrastructure,and into acute phase and convalescence group according to whether the infection is under control or not while carrying the biopsy,and also into primary and secondary group based on the cause of the cilia damage.Results:A total of 53 cases were included in our hospital,including 26males and 27 females,with a male to female ratio of 1:1.04.The median age was 79 months.TEM results indicated 15cases of normal ciliary ultrastructure and 38 cases(71.7%)of abnormal cilium ultrastructure,which were mainly manifested by abnormal AMC(24,44.4%),other ciliary ultrastructuralabnormalitiesincludecompositecilium/swollen cilium/cilium membrane loss(10,18.5%),microvilli(9,16.7%),abnormal BB development(6,11.1%),and the absence of DA(ODA and/or IDA)(5,9.3%).Compared with the normal group,the children in the abnormal group had more wheezing and moist rales(p=0.021,p=0.043),as well as higher WBC count while carrying biopsy(p=0.009).However,in the normal group,the course of disease was longer(p=0.026),and the incidence of unilateral respiratory tone reduction and atelectasis was higher(p=0.018,p=0.046,respectively).in other clinical characteristics,there was no significant difference between the two groups(all p>0.05).Respiratory secretions pathogens were mainly composed of streptococcus pneumoniae(20,46.5%)and haemophilus influenzae(10,23.2%).There was no significant difference between two groups in the detection rate of various pathogens(all p>0.05).After literature search and screening,a total of 166 cases from 10different regions were included in this study,with a male to female ratio of1:1.A total of 173 times of bronchoscopic ciliary biopsies were performed,among which 114 cases were found with ciliary ultrastructural abnormalities(72.6%).Consistent with the results of this study,the abnormality of cilium ultrastructure in the multi-center analysis was also dominated by the abnormality of AMC(59,37.8%).There were 49 cases with acute infection and 79 cases in convalescence while biopsy.The proportion of normal ciliary ultrastructure in convalescent group was significantly higher than that in acute phase group(?~2=6.957,P=0.008).PCD was mainly composed of the absence of DA,while SCD mainly consist of composite cilia/swollen cilia/cilia membrane loss,and the differences are significant(p=0.013,p=0.015,respectively).There were 8 cases diagnosed with PCD while 10cases diagnosed with SCD.Compared with the SCD group,the PCD group had longer hospitalization time(p=0.040),longer cough time(p=0.009),more fever(p=0.041),and higher incidence of bronchiectasis,neonatal pneumonia,sinusitis,and visceral inversion,all with statistically significant differences(p=0.036,p=0.007,p=0.025,p=0.002,respectively).There were no statistically significant differences in lobar resection rate,detection rate of various etiology and other clinical characteristics between the primary group and the secondary group(all p>0.05).Conclusion:1The ciliary ultrastructural abnormalities are of various types.In one ciliary biopsy,two or more types of abnormal structures can be found at the same time.Not considering the ciliary damage is primary of secondary,the disarrangement,the damage of the number and structure of the AMC account for the majority.The children with abnormal ciliary ultrastructure were more severe and had poor prognosis.Therefore,it's essential to strengthen the management of respiratory tract and to avoid repeated infection;2The PCD was mainly manifested by the loss of DA.In the primary group,the course of disease as well as the duration of cough and hospitalization was longer,and the incidence of bronchiectasis was higher in these children.Meanwhile,the clinical features of sinusitis,visceral inversion,and the history of neonatal pneumonia has some specificity for the PCD diagnose.When the ultrastructure of the cilia is normal,but the clinical manifestations are relatively heavy,further genetic examination is needed to confirm the diagnosis;3SCD is mainly manifested by composite cilia/swollen cilia/cilium membrane loss,and the destruction of the ultrastructure of the cilia is reversible.SCD can be gradually repaired,the time required for cilia repairment was at least 2 weeks,but the specific time was unknown,Repeated respiratory infection can difacilitate the repairment of cilia,so children with abnormal cilia ultrastructure should strengthen the respiratory management and prevent re-infection for at least 2-3 weeks after biopsy.4There was no significant difference between the normal and abnormal ciliary ultrastructure groups,neither between the PCD and SCD groups.The pathogen was dominated by streptococcus pneumoniae and haemophilus influenzae.This could guide the empirical therapy before we get the respiratory secretion culture results,such as streptococcus pneumoniae and haemophilus influenzae sensitive antibiotics.