Objective To explore the therapeutic value of bronchoscopy in pediatric status asthmaticus.Methods A total of 16 children with status asthmaticus received standard medical therapies (therapy group) from January 2008 to August 2010 in pediatric intensive care unit (PICU) at First Hospital,Xiamen University.However,l0 of them underwent fiberoptic bronchoscopy and bronchoalveolar lavage (BAL) (bronchoscopy group) along with standard medical therapies from September 2010 to July 2012.The values of oxygenation index (PaO2/FiO2),arterial partial pressure of carbon dioxide (PaCO2),blood oxygen saturation (SaO2),heart rate (HR),respiratory rate (RR) within 1 hour before bronchoscopy procedure,within 6 hour post procedure and within 24 hour post procedure were observed.The extinction time of wheezing sound,PICU length of stay and admission length of stay were observed between therapy and bronchoscopy groups.Results Bronchoscopies revealed a lot of thick mucus plugs and secretions.Large airways were lavaged for clearance of obstructive secretions with normal saline.Within BAL fluid counts of the bronchoscopy group,there were neutrophilia (n =8),eosinophilia (n =4) and neutrophilia/ eosinophilia (n =3).Within BAL fluid cultivation of the bronchoscopy group,4 patients had positive results for Streptococcus pneumoniae (n =2),Pseudomonas aeruginosa (n =1) and Staphylococcus aureus (n =1).In bronchoscopy group,the values of PaO/FiO2,PaCO2,SaO2,HR and RR were 319 ± 19,(40 ±4) mm Hg(1 mm Hg =0.133 kPa),(92.6 ± 1.5) ％,(128 ± 12) rates/min and (35 ± 4) breaths/min within 6 hour post procedure versus 255 ± 24,(54 ± 5) mm Hg,(89.2 ± 2.6) ％,(148 ± 10) rates/min and(50 ± 6)breaths/min within I hour before procedure (P ＜ 0.01).At 24 hour post procedure,the values of PaO2/FiO2,PaCO2,SaO2,HR,RR for bronchoscopy group were 354 ± 21,(40 ± 3) mm Hg,(93.4 ±1.1)％,(125±9)rates/min and(34 ±3)breaths/min versus 317 ±21,(46 ±4)mm Hg,(90.1±2.5) ％,(138 ± 8) rates/min and (43 ± 3) breaths/min respectively for therapy group (P ＜ 0.01).The extinction time of wheezing sound was (67 ± 22) hours for bronchoscopy group vs.(98 ± 23) hours for therapy group(P ＜ 0.01).The mean PICU length of stay was (1.6 ± 0.7) days for bronchoscopy group vs.(2.6 ± 0.7) days for therapy group (P ＜ 0.01).The mean admission length of stay was (5.0 ± 0.7) days for bronchoscopy group vs.(6.6 ± 1.2) days for therapy group(P ＜ 0.01).All patients for bronchoscopy group tolerated the procedure without any complications.Conclusion As a safe adjunctive therapy in pediatric status asthmaticus,flexible bronchoscopy with bronchial lavage may reduce the admission and PICU length of stay and alleviate clinical symptoms.

Objective To study the safety and efficacy of selective head cooling (SHC) with mild systemic hypotherrnia in neonates with HIE. Methods Fifty-four term infants with severe neonatal HIE were randomly assigned to the head cooling group (n=27) and control group (n=27). Forty-one infants in 96 h after admission were eligible for the study(SHC group n=21, control group n=20). In SHC group, the naso-pharyngeal temperature was maintained at (34.0±0.2) ℃ and rectal temperature maintained at 34～35 ℃ for 72 h, then rewarmed spontaneously. In control group, normal rectal temperature was maintained. During the period of the study, the infants of two groups were monitored on nasopharyngeal temperature, heart rate, respiratory rate,transcutaneous arterial oxygen saturation and blood pressure. Primary adverse effects inclu-ding severe arrhythmia, venous thrombosis or hemorrhage and severe hypotension were observed. The efficacy indicators including rate of death and severe disability, exercise and cognition development index were as-sessed. Results Severe arrhythmia, hypotension and renal failure were not found in both groups. Follow-up was conducted until postnatal 18 months and was not available in 6 babies (3 in SHC group and 3 in control group respectively). Death and severe disability occurred in 4 of 18 infants (22.2%)in SHC group and in 9 of 17 infants(52.9% ) in the control group respectively (P<0.05). Conclusion SHC for 72 h with mild systemic hypothermia in neonates with HIE is safe and effective. The therapy could reduce the risk of disabili-ty and handicap significantly.

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Enteritis ; diagnosis ; Eosinophilia ; diagnosis ; Gastritis ; diagnosis ; Humans ; Pancreatitis ; diagnosis

Objective:To investigate the effects of lipopolysaccharide (LPS)on the acute lung injury (ALI)and expressions of aquaporin 1 (AQP1)and aquaporin 5 (AQP5)in lung tissue of the rats. Methods:Forty-eight SPF grade male Wistar rats were randomly divided into control group and LPS group (n=24).The rats in LPS group were intravenously injected with LPS to induce ALI models,and the rats in control group were injected with saline. The rats were sacrificed at 2,6,12 and 24 h,and the samples were collected after the successful modeling.The pathological changes of lung tissue were observed with HE staining;the lung wet/dry weight (W/D)ratio and lung permeability index were detected;ELISA was used to detect the levels of TNF-αand MIP-1α.The expression levels of AQP1 and AQP5 protein and mRNA were measured by Western blotting,immunohistochemistry and Real-Time PCR methods. Results:Compared with control group, the TNF-α and MIP-1α levels in LPS group were significantly elevated at 2,6 and 12 h (P<0.05),and at 24 h they were gradually reduced to the normal level. The HE staining results showed the alveolar and interstitial edema at 2 h after LPS injection,obviously in 12 h. The lung W/D ratios and pulmonary permeability indexes at different time points in LPS group were significantly higher than those in control group (P<0.05),and they reached the peak at 12 h.The expression levels of AQP1 and AQP5 mRNA and protein in lung tissue of the rats at different time points in LPS group were significantly lower than those in control group (P<0.01 ). Conclusion:LPS can induce ALI in the rats and down-regulate the expressions of AQP1 and AQP5;LPS is involved in the formation of pulmonary edema.

Objective To explore the clinical-pathological characteristics, treatment and prognosis of eosinophilic gastro-enteritis in children. Methods The data of clinical manifestation, laboratory examination, endoscopy, biopsy, management and prognosis from 2 pediatric patients with eosinophilic gastroenteritis were retrospectively analyzed. Related articles were reviewed. Results The age of two patients was 13 and 14 years old. Case 1 was male and had an onset with acute pancreatitis. Case 2 was female with a history of food allergy and had an onset with unexplained ascites. Both patients presented with signiifcantly increased peripheral eosinophil count (45.9%-64.8%) and serum IgE (246-393 IU/ml). Bone marrow cytology showed increased proportion of eosinophils in both patients. Gastroscopy was performed in case 1 while gastroscopy and colonoscopy endoscopy were performed in Case 2 and the results indicated eosinophilic inifltration in duodenum. Duodenal mucosa biopsy showed eosinophilic inifltration in case 1 while gastrointestinal pathology biopsy showed multiple mucosal eosinophil inifltration in case 2. After the therapy of food avoidance, steroid and anti-allergic drugs, both patients had complete remission 1 week later. Meanwhile the peripheral eosinophil count was decreased to normal. Case 1 was followed up for 24 months, and case 2 was followed up for 2 months. Both patients showed no evidence of relapse. Conclusions The clinical manifestation and endoscopic examination of eosinophilic gastroenteritis in children are protean and lack of speciifcity. Unexplained gastrointestinal symptoms accompanied with eosinophilia may suggest the possibility of eosinophilic gastroenteritis. Eosinophilic inifltration in ascites and gastrointestinal mucosa strongly indicate the di-agnosis of eosinophilic gastroenteritis. After exclusion of other possible diseases, the deifnite diagnosis can be made.

Objective:To explore the feasibility and safety of long-term home mechanical ventilation(HMV) in children with chronic respiratory failure.Methods:Clinical data of 11 children with chronic respiratory failure, who underwent HMV with the care of the First Affiliated Hospital of Xiamen University from January 2013 to December 2019, were retrospectively reviewed. The clinical manifestation, growth and development, quality of life, adverse events and prognosis of HMV children were analyzed.Results:There were 8 boys and 3 girls with the onset age of 26 days to 13 years old; and the age at starting HMV was 3 months to 13 years old. Eight children were diagnosed as neuromuscular diseases, and 3 children were diagnosed as respiratory diseases. The duration of institutional mechanical ventilation was 2 weeks to 8 months. Six patients underwent invasive HMV via a tracheostomy, and 5 received non-invasive ventilation via nasal and face masks. Bi-level positive airway pressure ventilation mode was applied in all the patients. The duration of HMV was 3 months to 27 months. During follow-up, no HMV related adverse events were observed. Both the quality of life and nutritional status were improved in all cases. One patient lost follow-up 9 months later and 1 patient died of severe adenovirus pneumonia during hospitalization for examination, the remaining 9 cases survived. Liberation from HMV was obtained in 4 patients. The frequency of readmission was 1 to 2 times.Conclusion:It is suggested that long-term HMV is safe and feasible for children with chronic respiratory failure.

Objective: To review children′s primary ciliary dyskinesia (PCD) in the pathogenesis, clinical manifestation, diagnosis and treatment. Method: To summarize and analyze the clinical data of a patient who was admitted to the first affiliated hospital of Xiamen University with primary ciliary dyskinesia in April 2014 while referring to related literature. Result: An 11 years old boy, weighting about 22 kg, had a course of more than 10 years with repeated cough, stuffy and runny nose shortly after the birth. Examinations after admission to hospital showed that he presented with visible clubbing, bilateral paranasal sinus area tenderness, pharynx posterior wall with visible yellow pussy stuff drip and bilateral lung had scattered wet rales. Auxiliary examination revealed bilateral maxillary sinus, ethmoid sinus inflammation and bronchitis with left lower lung bronchiectasis. Fiberoptic bronchoscopy discovered congestion and a lot of sputum; ciliary biopsy pathology displayed that cilia were sparse and partial cilia 9+ 2 microtubules structural abnormalities. Full sequence of exon gene sequencing revealed two mutations located at chromosome 16 chr16: 71061369 (non-coding regions) and chr16: 70993591 (coding). Two novel mutations m. 3362A>G(E20) and c. 6101G>A(E39) in exon 16 of the HYDIN gene were identified. With the" ciliary motility disorder, gene" as keywords , the CNKI, Wanfang digital knowledge service platform and PubMed were searched for relevant articles from the establishment to July 2016. The studies retrieved included 9 cases and these cases were summarized. Comprehensive analysis showed that HYDIN gene mutations related PCD patients had the typical PCD performance such as repeatedly wet cough, sinusitis, bronchiectasis, and otitis media. The majority of patients have a history of acute respiratory distress syndrome in infancy and no visceral dislocation was not found. Most of the patients had no obvious structural abnormalities in cilia electron microscopic examination. Conclusion The PCD patients with HYDIN genes mutations have clinical manifestations such as sinusitis, otitis media, bronchiectasis but without transposition of viscera. Cilia structure can be normal under the electron microscopic examination in some of patients.

Objective: To investigate general condition of children′s rheumatic disease associated medical resources in Fujian Province. Methods: This questionnaire-based survey was conducted in 19 hospitals in Fujian province from December 2, 2018 to May 1, 2019. The questionnaire was designed to survey the general condition of the medical resources and the hospitalization of patients with rheumatic diseases from January 1, 2014 to December 1, 2018. Results: In the 19 hospitals, there were 15 general hospitals and 4 children′s hospitals, and only 5 hospitals had children′s rheumatic specialist clinic. There were only 53-62 beds for rheumatic disease patients in the 19 hospitals, accounting for 1.7%-2.0% of the total inpatient beds (3 137). There are 29 pediatric rheumatologists in total, accounting for 2.6% (29/1 120) of the total pediatricians. In the past five years, 613 patients with rheumatic diseases, accounting for 0.1% (613/625 214) of total hospitalized patients, were treated in these hospitals. Among them, 201 had juvenile idiopathic arthritis, 295 had systemic lupus erythematosus, 39 had dermatomyositis, 7 had scleroderma, and 57 had inflammatory bowel disease, 9 had Sjogren′s syndrome, 5 had Behcet′s disease, and none had overlap syndrome or mixed connective tissue disease. Conclusion The medical resources of children rheumatic diseases in Fujian province are insufficient which need to be developed.

With the improvement of pediatric treatment level, the number of children with prolonged mechanical ventilation has increased rapidly.The transition of children from PICU to home mechanical ventilation is a very complex and arduous process that requires multi-faceted support and management.Long-term mechanically ventilated children and their families bear a huge social and psychological burden, and they need psychological support and humanistic care from the society.