目的 了解儿童难治性肺炎支原体肺炎(RMPP)患儿23SrRNAV区耐药基因突变情况,比较突变与未突变患儿的临床资料.方法 回顾性分析2021年10月至2022年3月山东大学附属儿童医院呼吸介入科332例确诊RMPP并行可弯曲支气管镜(以下简称支气管镜)检查及肺泡灌洗术患儿的临床资料.提取患儿BALF标本中MP-DNA,采用实时荧光聚合酶链式反应(PCR)法对大环内酯类药物作用靶位MP 23SrRNAV区的2063和2064位点进行突变检测,并根据耐药基因检测结果将其分为耐药组与非耐药组,比较两组患儿的临床资料.结果 332例RMPP患儿中,耐药组患儿270例,非耐药组患儿62例.耐药组患儿发热时间长于非耐药组;实验室指标:外周血血常规(白细胞计数、中性粒细胞百分比)、C-反应蛋白(CRP)、血清乳酸脱氢酶(LDH)、血浆D-二聚体(D-Di)、免疫球蛋白E(IgE)等值均较非耐药组高(P＜0.05);出现中-大量胸腔积液比例高,且易发生坏死性肺炎;耐药组患儿支气管镜下表现以黏膜糜烂、溃疡、坏死,支气管内塑形性栓子,脓性肺泡灌洗液为主.结论 肺炎支原体(MP)耐药突变可能导致并促进RMPP的发生.耐药突变MP感染可能会加重RMPP患儿病情,其影像学及支气管镜下表现更重.支气管镜下表现对早期识别耐药性MP感染具有一定的意义.

Purpose:The repair and treatment of infected bone defects (IBD) is a common challenge faced by orthopedic clinics, medical materials science, and tissue engineering.Methods:Based on the treatment requirements of IBD, we utilized multidisciplinary knowledge from clinical medicine, medical materials science, and tissue engineering to construct a high-efficiency vancomycin sustained-release system with nanodiamond (ND) and prepare a composite scaffold. Its effect on IBD treatment was assessed from materials, cytology, bacteriology, and zoology perspectives.Results:The results demonstrated that the Van-ND-45S5 scaffold exhibited an excellent antibacterial effect, biocompatibility, and osteogenesis in vitro. Moreover, an efficient animal model of IBD was established, and a Van-ND-45S5 scaffold was implanted into the IBD. Radiographic and histological analyses and bone repair-related protein expression, confirmed that the Van-ND-45S5 scaffold had good biocompatibility and osteogenic and anti-infective activities in vivo.Conclusion:Collectively, our findings support that the Van-ND-45S5 scaffold is a promising new material and approach for treating IBD with good antibacterial effects, biocompatibility, and osteogenesis.

Background Along with the wide spread application and technical development of the flexible and rigid bronchoscopy, the airway foreign body removing method cme to the specific technique for different foreign bodies from the single foreign body forceps. Methods Selected 633 children who were diagnosed as airway foreign bodies by the Department of Respiratory Intervention, Children's Hospital affiliated to Shandong University from January 1st, 2018 to December 31st, 2021, and the airway foreign bodies were diagnosed using bronchoscopy. After comprehensive assessment of the foreign body nature in the airway, the foreign bodies were removed by freezing, laser, electrocoagulation, balloon and other techniques, the success rate of the foreign body removed from the airway was observed, the percentage of the foreign body removed using different techniques, the operation time, and the incidence of post-adverse reactions during operation. Results The success rate using flexible bronchoscope alone to remove foreign bodies in the airway was 99.2%. After flexible bronchoscopy, 19 cases of foreign bodies were removed by vacuum suction alone, 513 cases were removed by foreign body forceps alone, 62 cases were combined with cryotherapy, 2 cases were electrocoagulation, 6 cases were mesh baskets, 3 cases were balloons, 5 cases were laser, and various 18 cases of foreign bodies were invloved by technical combination. 5 cases of flexible bronchoscope combined with rigid bronchoscope combined to remove foreign bodies. The operation time was from 5 min to 1 h, with an average of 20 min. There were 17 cases of hypoxemia (2.7%) during operation, 36 cases (5.7%) of bleeding caused by airway mucosa injury after treatment, and 70 cases (11.2%) of laryngeal edema. The total incidence of adverse reactions was 19.6%, there were no deaths due to foreign bodies and treatment. Conclusions According to different properties of airway foreign bodies, it is safe and effective to select appropriate techniques to remove foreign bodies using the flexible bronchoscope, which can increase the removal rate of airway foreign bodies and reduce the occurrence of serious complications.

Congenital deficiency of tracheal rings is a rare tracheal malformation that can cause central airway obstruction. Herein we reported the clinical data of six patients with symptomatic congenital deficient tracheal rings. There were five cases, with isolated short-segment absent cartilage ring located on the distal trachea (three cases), cervical trachea (one case), and distal trachea combined with bilateral bronchi (one case). Among them, four (4/5) received surgical tracheal resection, three fully recovered, and one died of severe infection. Besides that, one patient, who could not be weaned off the mechanical ventilation, died after rejecting surgery. One case had episodes of recurrent dyspnea and extubation failure due to long-segment tracheomalacia after repair of esophageal atresia and tracheoesophageal fistula. For this patient, deficient cartilage rings were suspected and confirmed at the age of 26 months. Moreover, the clinical characteristics of 12 cases with congenital deficient tracheal cartilage rings reported in previous literature were reviewed. The different characteristics between short- and long-segment deficient cartilage rings were discussed.

RATIONALE:Broncholithiasis is a rare disease and is characterized by calcification in the tracheobronchial tree. This disease has a predilection in the adult and occurrence is rare in children. Considering its infrequency, we report a rare clinical case, with the aim of sharing our experience during the diagnosis and treatment procedures.PATIENT CONCERNS:We report a 9-year- and 6-month-old girl who complained of chronic cough and recurrent wheeze for 2 months.DIAGNOSIS:Bronchialithiasis were found under bronchoscope. Pathologic examination revealed aspergillosis.INTERVENTIONS:The stone and surrounding granulation tissue were removed using a bronchoscope and the patient was treated with antifungal therapy.OUTCOMES:The patient was in good general health without any clinical symptoms during the follow-up period.LESSONS:Pulmonary Aspergillus infection can cause bronchiolithiasis. Stones were removed under bronchoscope, and anti-Aspergillus therapy was effective.

随着肺部计算机断层扫描(CT)及支气管镜术在儿科的普及和发展,中心性气道狭窄越来越多地被诊断.中心性气道狭窄可影响肺通气功能及分泌物排出,患儿常因反复肺炎住院,重者需呼吸机辅助通气,还可能出现撤机困难,常合并营养不良、缺氧性脑损伤、生长发育落后等.以往中心性气道狭窄多采用外科手术治疗.近年来,随着呼吸内镜微创医学的发展,气道内支架置入成为一种有效的、可供选择的治疗方案.该文将对儿科中心性气道狭窄的类型、气道内支架置入的适应证和禁忌证、支架的种类和选择、支架置入术的工作流程等作一阐述.

Joubert syndrome (JBTS) is a clinically and genetically heterogeneous group of ciliopathy with a key diagnostic feature of ‘molar tooth sign’ in brain MRI. So far, over 20 causative genes have been identified, but only one gene ( OFD1 ) results in X-linked Joubert syndrome 10 (JBTS10). Six mutations in the OFD1 gene have been found to cause JBTS10. In this study, we identified a novel OFD1 mutation of c.2843_2844 delAA (p.Lys948ArgfsX) in a 3-month-old boy with a ‘molar tooth sign’ and clinical features of JBTS using targeted exome next-generation sequencing. The de-novo OFD1 mutation in exon 21 leads to a frameshift mutation generating a prematurely truncated protein and is predicted to partly reduce the function of the OFD1 protein. Our study expands the genotype–phenotype spectrum in JBTS and will have applications in prenatal and early diagnosis of the disorder. This is the first report of the OFD1 mutation causing JBTS in a Chinese population.

Previously, the main treatment options for tracheoesophageal fistula included surgery and conservative treatment. Herein, we report a child suffering from severe tracheoesophageal fistula due to button battery ingestion. The child relapsed soon after a repair surgery. Then, he was endotracheally implanted with a fully-covered metallic stent combined with a jejunal tube feeding. He recovered soon and the stent was removed five months later. The fistula was healed with no relapse during a 25-month follow-up. Therefore, endotracheal implantation of fully-covered metallic stent is an alternative treatment for tracheoesophageal fistula due to button battery ingestion, especially in cases with severe respiratory disorders.

A retrospective surveillance study on enterovirus D68 was performed in Beijing, China, following the largest and most widespread EV-D68 infection, which occurred in the USA. From January 2011 to July 2015, EV-D68 was identified in 12 individuals with respiratory infections in Beijing, China. The phylogenetic relationships based on the genomic sequence alignment showed that there were two lineages circulating in Beijing from 2011 to 2015. Eight EV-D68 strains belonged to group 1 and four belonged to group 3. All EV-D68 strains from Beijing in 2014 were separately clustered into subgroup II of group 1. Based on these results, we concluded that the Beijing EV-D68 strains had little association with the EV-D68 strains circulating in the 2014 USA outbreak.

1 Shandong Medicinal Biotechnology Centre, Key Laboratory for Modern Medicine and Technology of Shandong Province, Key Laboratory for Virology of Shandong Province, Key Laboratory for Rare & Uncommon Diseases of Shandong Province, Key Laboratory for Biotech-Drugs, Ministry of Health, Shandong Academy of Medical Sciences, No. 18877 Jingshi Road, Jinan 250062, China 2 School of Medicine and Life Sciences, University of Jinan-Shandong Academy of Medical Sciences, Jinan 250200, China 3Department of Laboratory, Qilu Children’s Hospital of Shandong University, Jinan 250022, China 4 School of Dental Medicine, Shandong University, Jinan 250012, China 5 Institute of Basic Medicine, Shandong Academy of Medical Sciences, Jinan 250062, China 6Respiratory Department, Qilu Children’s Hospital of Shandong University, Jinan 250022, China

The viral etiologies of UTRIs and LTRIs in children in Jinan city were investigated between July 2009 and June 2010. Nasal and throat swabs were collected from 397 children with URTIs and bronchoalveolar lavage fluid specimens were collected from 323 children with LRTIs. RT-PCR/PCR was used to examine all samples for IFV, PIV, RSV, RV, hMPV, HBoV, CoV, ADV, RSV, and EV. Viral pathogens were detected in 47.10% of URTI samples and 66.57% samples, and the incidence of viral coinfection was 5.29% and 21.05%, respectively. IFV was the most common virus in URTIs, with a detection rate of 19.40%, followed by PIV (10.83%), RV (10.58%), and EV (6.30%). For LRTIs, PIV and RV were both detected in 27% of samples, followed by RSV (9.91%), HBoV (8.36%), IFV (5.57%), and hMPV (5.57%). RSV and HBoV were more prevalent in the youngest children of no more than six months. Meanwhile, RV, PIV, and RSV were the most frequent viruses combined with bacterial pathogens in LRTIs. In conclusion, the spectrum of respiratory virus infections in URTIs and LRTIs differed in terms of the most common pathogens, seasonal distribution, and coinfection rate.

OBJECTIVES:To assess the feasibility of low-dose prospective ECG-gated dual-source CT (DSCT) in detecting airway anomalies in pediatric patients with cardiovascular anomalies compared with flexible tracheobronchoscopy (FTB). METHODS:33 pediatrics with respiratory symptoms who had been revealed cardiovascular anomalies by transthoracic echocardiography underwent FTB and contrast material-enhanced prospective ECG-triggering CT were enrolled. The study was approved by our institution review board and written informed consent was obtained from all patients' guardian. DSCT examinations were performed to detect cardiovascular abnormalities using weight-adjusted low-dose protocol. Two radiologists independently performed CT image analysis. The FTB reports were reviewed by an experienced pulmonologist. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of DSCT in the detection of airway anomalies were assessed. The tracheobronchial stenoses revealed on FTB were graded. Effective radiation dose was calculated. RESULTS:Thirty cases were diagnosed with tracheobronchial narrowing and/or abnormality in 33 patients by FTB, while 3 patients had normal FTB findings. Twenty-eight cases were diagnosed with airway anomalies by CT, of which 27 were correct positive. 3 patients with normal findings at CT had findings of tracheobronchial narrowing due to tracheobronchomalacia at inspiration at FTB. Sensitivity and specificity of CT were 90.0% (95% CI: 72.3%, 97.4%) and 66.7% (95% CI: 12.5 %, 98.2 %), respectively. PPV and NPV were 96.4% (95% CI: 79.8 %, 99.8%) and 40.0% (95% CI: 7.3%, 83.0%), respectively. Overall accuracy of DSCT in detecting airway anomalies in pediatrics with cardiovascular anomalies was 87.9% (95% CI: 74.5%, 97.6%). In grading of tracheobronchial stenosis, images from CT correlated closely (r = 0.89) with those of FTB. Mean effective dose was 0.60 ± 0.20 mSv. CONCLUSION:In pediatric patients, ECG-triggered CT to evaluate congenital cardiovascular anomalies can also be used to diagnose and characterize fixed airway involvement in relation to the vascular structures.

OBJECTIVE:To analyze the effect and safety of bronchoscopic cryosurgery in children with lower airway stenosis caused by granulation and fiber hyperplasia.METHOD:Twenty-two patients had undergone bronchoscopic examination and cryosurgery, 18 patients with atelectasis caused by Mycoplasma pneumoniae pneumonia, 2 patients with endobronchial tuberculosis (EBTB), 1 patient with atelectasis caused by granulation tissue after bronchial foreign body, and 1 case who had severe dyspnea, with tracheal stenosis after long-term endotracheal intubation during surgery for heart disease. All the patients under went bronchoscopic cryosurgery for several times, 1 case with severe tracheal stenosis was operated by electric coagulation before cryosurgery. Before and after the treatment, all the patients were evaluated based on clinical symptoms, tracheal lumen by bronchoscopy, chest CT for atelectasis and pulmonary function for tracheal stenosis.RESULT:The patients were treated with cryosurgery for 1-4 times. Sixteen cases were markedly improved, clinical symptoms disappeared completely, no granulation and fiber hyperplasia were found during bronchoscopy, and all the atelectasis were cured. After the treatment, the bronchial lumen diameter of the patient with tracheal stenosis was increased from 2 mm to about 5 mm, the tidal volume increased from 3.0 ml/kg to 8.8 ml/kg. Five cases were effectively improved, the clinical symptoms also disappeared, during bronchoscopy and bronchial alveolar lavage (BAL), no granulation and fiber hyperplasia were found, but some distal bronchial tubes were atresia or narrowed, the lumen was patent, the atelectasis recovered partially. In 1 case the treatment was ineffective, as the clinical symptoms and granulation disappeared, but because almost all distal bronchi were atresia or narrow, the lumen was not patent during BAL, there was no change on chest CT. The total effective rate was 21/22 (95.5%). No severe complications occurred during and after the procedures. All the patient were followed up for 1 - 12 months, no case had recurrence.CONCLUSION:Treatment with bronchoscopic cryosurgery is a safe and effective methods to tracheal stenosis caused by granulation tissue and fiber hyperplasia in children.

OBJECTIVE:To assess the efficacy and safety of balloon dilatation through flexible bronchoscopy in the management of inflammatory stenosis of grade 4-5 bronchus.METHOD:Thirty patients with inflammatory bronchial stenosis caused by mycoplasmal pneumonia complicated with pulmonary atelectasis were treated with balloon dilatation through fiberoptic bronchoscopy. Before the procedure and after the last operation, therapeutic effect on pulmonary atelectasis were evaluated with CT and all of the patients were followed-up for 1 - 6 months.RESULT:One to three operations were required to achieve satisfactory dilatation. After balloon dilatation, the average airway diameter increased obviously and the farther airways were opened after the therapy with irrigation. In 25 of 30 cases satisfactory immediate effects were obtained, a narrow airway diameter above expansion significantly increased as compared with preoperative diameter. In 5 children treated with balloon dilatation, the stenosis could not be improved significantly. In 3 patients with hyperplasia of granulation tissue, cryotherapy had to be applied. The operations were ineffective in the other two patients whose course of disease exceeded 3 months. After follow-up periods of 1 - 6 months, chest CT manifestation of expanded sites was improved in 28 patients and atelectasis disappeared. No severe complication was found in any patients.CONCLUSION:Bronchoplasty by balloon dilatation through flexible fiberoptic bronchoscopy is a simple, effective and safe method to treat childhood tracheobronchial stenosis after pulmonary infections.