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Fungal infections gradually attract global public health attention. Candida, Aspergillus, and Cryptococcus are the most common pathogens of fungal infections.With the widespread use of antifungal drugs, fungal resistance is gradually increasing.Clarifying the current status of antifungal drug resistance and the mechanism is helpful for rational treatment of fungal infections.
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OBJECTIVE To provide reference for the safe use of bevacizumab in cancer patients. METHODS The diagnosis and treatment of a 65-year-old female lung adenocarcinoma patient with diabetic ketoacidosis (DKA) induced by bevacizumab was retrospectively analyzed, and the possible mechanisms and causes were analyzed based on literature review. RESULTS & CONCLUSIONS The diagnosis and treatment process of patients were analyzed, and DKA caused by other drugs and disease factors were excluded. DKA was considered to be caused by the use of bevacizumab according to Naranjo’s ADR evaluation scale; the acidosis of the patient improved rapidly after one hemodialysis treatment. DKA caused by bevacizumab is rare in clinic, clinicians should be aware that bevacizumab may affect pancreatic function and induce DKA, and early detection and treatment should be achieved to improve the prognosis.
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Objective:To investigate the clinical application status and safety of systemic administration of Levofloxacin.Methods:Hospitalized children treated with Levofloxacin from January 1, 2017 to December 31, 2022 in Beijing Children′s Hospital, Capital Medical University and Baoding Hospital of Beijing Children′s Hospital were retrospectively recruited.Clinical data like the gender, age, weight, discharge diagnosis, drug name, dosage, route of administration, course of treatment, pathogenic microorganisms, and adverse events were collected.Results:A total of 220 children were included.The age of Levofloxacin use ranged from 1 month and 14 days to 16 years, 7 months and 27 days.The use of Levofloxacin in children was mainly distributed in school age (70 cases), preschool age (65 cases), and early childhood (44 cases), accounting for 81.37% of all children.Children treated with Levofloxacin were admitted in a total of 13 departments, mainly included the respiratory department (77 cases), hematology department (49 cases), infectious medicine department (47 cases), pediatric intensive care unit (14 cases), and stem cell transplantation department (9 cases), accounting for 89.08% of all children.The use of Levofloxacin was mainly applied to children with pneumonia (150 cases), systemic disseminated tuberculosis (25 cases), sepsis (20 cases) and blood disease complicated with infection (16 cases). The common pathogens were Mycoplasma pneumoniae (102 cases), Klebsiella pneumoniae (14 cases), Stenotrophomonas maltophilia (14 cases), Acinetobacter baumannii complex (10 cases), Staphylococcus aureus(9 cases), and Pseudomonas aeruginosa (9 cases). The use and dosage of Levofloxacin were concerned, including insufficient single dose(25 cases), overdose(9 cases), incorrect dosing frequency (9 cases). There were 3 cases of adverse drug reaction of Levofloxacin, including 2 cases of gastrointestinal reactions (1 case of diarrhea, a single dose of 15 mg/kg, clinically cured; 1 case of nausea, clinically improved), and 1 case of rash (clinically improved). Serious adverse drug reactions were not reported. Conclusions:Levofloxacin is relatively safe for short- and medium-term use in children.It should be administered according to the correct usage and dosage.During the administration period of Levofloxacin, the gastrointestinal system, rash, liver and kidney functions, blood system, blood sugar, heart system, nervous system and other indicators should be closely concerned.
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Objective:To analyze the clinical characteristics, treatment course and prognosis of children with intermediate-high risk pulmonary embolism.Methods:The clinical data of 48 children with pulmonary embolism treated in Beijing Children′s Hospital, Capital Medical University from January 2017 to December 2021 were analyzed retrospectively.Including 12 intermediate-high risk cases and 36 low-risk cases.The clinical manifestations, laboratory results, treatment and prognosis were compared between groups by the t-test, rank sum test and Chi- square test with the yates continuity correlation or Fisher′ s exact test. Results:There were no significant differences in the sex and age between the intermediate-high risk group and the low-risk group.The proportions of patients with shortness of breath, dyspnea, cyanosis or hypoxemia were higher in the intermediate-high risk group than those of in low-risk group.Twelve children in the low-risk group did not have specific symptoms of pulmonary embolism.There were no significant differences in the D-dimer level, and the distribution of pulmonary embolism between the two groups (all P>0.05). However, the proportion of children with other thromboembolism in the intermediate-high risk group was significantly higher than that of the low-risk group, among which heart thrombosis was the most common (7 cases). There were no significant differences in the underlying diseases and thrombophilia between the two groups (all P>0.05). The treatment of the intermediate-high risk group was more active: 6/12(50.00%) patients in the intermediate-high risk group received reperfusion treatment, including 3 cases of systemic thrombolysis, 1 case of catheter thrombolysis, and 2 cases of thrombectomy.In the low-risk group, only 1 case was treated with systematic thrombolysis.Unfavorable outcomes were reported in 3/48 (6.25%) patients, including 1 death of massive bleeding after catheter-directed thrombolysis in the acute phase, 1 case of recurrent pulmonary embolism after self-decided withdrawal and 1 case of progression of pulmonary embolism that was managed by surgical thrombectomy, all of whom were in the intermediate-high risk group. Conclusions:Shortness of breath, dyspnea, cyanosis or hypoxemia and co-existed venous thromboembolism were more common in intermediate-high risk cases.The treatment regimen of was more aggressive, but the incidence of unfavorable outcomes was higher in intermediate-high risk group; further research is needed to determine the risk factors for intermediate-high risk pulmonary embolism in children.
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China has classified the Corona Virus Disease 2019(COVID-19) as a statutory category B infectious disease and managed it according to Category B since January 8, 2023.In view that Omicron variant is currently the main epidemic strain in China, in order to guide the treatment of severe acute respiratory syndrome coronavirus 2(SARS-CoV-2) infection in children with the times, refer to the Diagnosis and Treatment Protocol for Novel Coronavirus Infection (Trial 10 th Edition), Expert Consensus on Diagnosis, Treatment and Prevention of Novel Coronavirus Infection in Children (Fourth Edition) and the Diagnosis and Treatment Strategy for Pediatric Related Viral Infections.The Expert Consensus on the Diagnosis, Treatment and Prevention of Novel Coronavirus Infection in Children (Fifth Edition) has been formulated and updated accordingly on related etiology, epidemiology, pathogenic mechanism, clinical manifestations, auxiliary examination, diagnosis and treatment, and added key points for the treatment of COVID-19 related encephalopathy, fulminating myocarditis and other serious complications for clinical reference.
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Objective:To assess the clinical effectiveness and safety of Omalizumab for treating pediatric allergic asthma in real world in China.Methods:The clinical data of children aged 6 to 11 years with allergic asthma who received Omalizumab treatment in 17 hospitals in China between July 6, 2018 and September 30, 2020 were retrospectively analyzed.Such information as the demographic characteristics, allergic history, family history, total immunoglobulin E (IgE) levels, specific IgE levels, skin prick test, exhaled nitric oxide (FeNO) levels, eosinophil (EOS) counts, and comorbidities at baseline were collected.Descriptive analysis of the Omalizumab treatment mode was made, and the difference in the first dose, injection frequency and course of treatment between the Omalizumab treatment mode and the mode recommended in the instruction was investigated.Global Evaluation of Treatment Effectiveness (GETE) analysis was made after Omalizumab treatment.The moderate-to-severe asthma exacerbation rate, inhaled corticosteroid (ICS) dose, lung functions were compared before and after Omalizumab treatment.Changes in the Childhood Asthma Control Test (C-ACT) and Pediatric Asthma Quality of Life Questionnaire (PAQLQ) results from baseline to 4, 8, 12, 16, 24, and 52 weeks after Omalizumab treatment were studied.The commodity improvement was assessed.The adverse event (AE) and serious adverse event (SAE) were analyzed for the evaluation of Omalizumab treatment safety.The difference in the annual rate of moderate-to-severe asthma exacerbation and ICS reduction was investigated by using t test.The significance level was set to 0.05.Other parameters were all subject to descriptive analysis.A total of 200 allergic asthma patients were enrolled, including 75.5% ( n=151) males and 24.5% ( n=49) females.The patients aged (8.20±1.81) years. Results:The median total IgE level of the 200 patients was 513.5 (24.4-11 600.0) IU/mL.Their median treatment time with Omalizumab was 112 (1-666) days.Their first dose of Omalizumab was 300 (150-600) mg.Of the 200 cases, 114 cases (57.0%) followed the first Omalizumab dosage recommended in the instruction.After 4-6 months of Omalizumab treatment, 88.5% of the patients enrolled ( n=117) responded to Omalizumab.After 4 weeks of treatment with Omalizumab, asthma was well-controlled, with an increased C-ACT score [from (22.70±3.70) points to (18.90±3.74) points at baseline]. Four-six months after Omalizumab administration, the annual rate of moderate-to-severe asthma exacerbation had a reduction of (2.00±5.68) per patient year( t=4.702 5, P<0.001), the median ICS daily dose was lowered [0 (0-240) μg vs. 160 (50-4 000) μg at baseline] ( P<0.001), the PAQLQ score was improved [(154.90±8.57) points vs. (122.80±27.15) points at baseline], and the forced expiratory volume in one second % predicted (FEV 1%pred) was increased [(92.80±10.50)% vs. (89.70±18.17)% at baseline]. In patients with available evaluations for comorbidities, including allergic rhinitis, atopic dermatitis or eczema, urticaria, allergic conjunctivitis and sinusitis, 92.8%-100.0% showed improved symptoms.A total of 124 AE were reported in 58 (29.0%) of the 200 patients, and the annual incidence was 0(0-15.1) per patient year.In 53 patients who suffered AE, 44 patients (83.0%) and 9 patients (17.0%) reported mild and moderate AE, respectively.No severe AE were observed in patients.The annual incidence of SAE was 0(0-1.9) per patient year.Most common drug-related AE were abdominal pain (2 patients, 1.0%) and fever (2 patients, 1.0%). No patient withdrew Omalizumab due to AE. Conclusions:Omalizumab shows good effectiveness and safety for the treatment of asthma in children.It can reduce the moderate-to-severe asthma exacerbation rate, reduce the ICS dose, improve asthma control levels, and improve lung functions and quality of life of patients.
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Objective:To analyze the clinical characteristics of pulmonary vein stenosis (PVS) in children, and to explore its treatment and prognostic factors.Methods:The clinical data of 19 children with PVS treated in Beijing Children′s Hospital, Capital Medical University from October 2016 to March 2022 were analyzed retrospectively.There were 16 males and 3 females.The median age at diagnosis was (2.81±1.95) years.A descriptive analysis of clinical characteristics of children was made.Results:Of the 19 children, 14 cases (73.7%) had primary PVS and 5 cases (26.3%) had secondary PVS after surgery of anomalous pulmonary venous connection (APVC). Thirteen children (68.4%) had hemoptysis.In the hemoptysis children, 5 cases had life-threatening massive hemoptysis, and 11 cases (57.9%) had a history of recurrent respiratory tract infection or pneumonia.Other manifestations of hemoptysis included failure to thrive (6 cases), cyanosis (5 cases), and dyspnea (3 cases). Complications were pulmonary hypertension (6 cases) and right heart failure (3 cases). There were 16 cases (84.2%) of unilateral PVS and 3 cases of bilateral PVS.Interlobular septal thickening, grid shadow and ground glass opacities were found on CT of all PVS cases.Ten cases underwent surgery, and 2 cases of them received angioplasty, but restenosis occurred in both of them.Eight children underwent pulmonary lobectomy, and their clinical symptoms were all relieved after operation.Nine patients were treated conservatively, and 3 cases of them died of bilateral PVS secondary to APVC.The remaining 6 alive cases still had intermittent clinical symptoms during follow-up.Conclusions:Hemoptysis and recurrent respiratory tract infection are the main clinical manifestations of PVS in children, and life-threatening massive hemoptysis can occur.Lobectomy is an effective treatment for unilateral PVS.The prognosis of secondary PVS after APVC is poorer and its mortality is higher, compared with primary PVS.
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Objective @#To examine the association between acute exposure to traffic-related air pollutants (TRAP) NOX and NO2 and outpatient visits of pediatric respiratory diseases. @*Methods @#Data regarding outpatient visits to Department of Respiratory Diseases of Beijing Children's Hospital from 2015 to 2020 were collected, and the concentrations of nitrogen oxides (NOX), nitrogen dioxide (NO2) and other TRAP were collected from the surveillance sites assigned by the Peking University Health Science Center. A time-stratified case-crossover design was employed, and a conditional logistic regression model was created to examine the association between NOX and NO2 acute exposure and outpatient visits of pediatric respiratory diseases. @*Results @#The daily mean outpatient visits of pediatric respiratory diseases were 571 (interquartile range, 554) person-times among children at ages of 0 to 14 years in Beijing Children's Hospital from 2015 to 2020, and the daily mean outpatient visits for upper respiratory tract infections (URI), bronchitis, and pneumonia were 265 (interquartile range, 282), 143 (interquartile range, 178) and 128 (interquartile range, 120) person-times, respectively. The daily mean concentrations of atmospheric NOX and NO2 were 67.8 (interquartile range, 50.7) and 49.3 (interquartile range, 30.7) μg/m3, respectively. Conditional logistic regression analysis showed the largest lagged effect of NOX and NO2 on pediatric respiratory diseases at cumulative lags of 0 to 7 days. An increase in NOX concentrations by an interquartile range resulted in the excess risks of URI, bronchitis and pneumonia by 6.87% (95%CI: 6.37%-7.38%), 7.25% (95%CI: 6.51%-7.99%), and 5.51% (95%CI: 4.69%-6.33%), and an increase in NO2 concentrations by an interquartile range resulted in excess risks of URI, bronchitis and pneumonia by 5.71% (95%CI: 5.12%-6.31%), 5.32% (95%CI: 4.51%-6.14%), and 4.83% (95%CI: 3.91%-5.75%), respectively. NOX and NO2 presented a more remarkable effect on outpatient visits of pediatric respiratory diseases among children at ages of over 5 years. @*Conclusion @#NOx and NO2 acute exposure may increase the outpatient visits of pediatric respiratory diseases.
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Objective:To analyze the clinical manifestations, genetic variations, diagnosis and treatment of children with inherited thrombophilia(IT).Methods:Retrospective study.Children with IT treated in Department of Respiratory Diseases 1 of Beijing Children′s Hospital, Capital Medical University from October 2016 to August 2021 were included in the study and followed up.Results:A total of 5 children met the inclusion criteria, with 3 boys and 2 girls; the age of diagnosis ranged from 7 years to 13 years and 6 months.There were 2 cases of protein C deficiency, 1 case of congenital protein S deficiency, 1 case of activated protein C resistance and 1 case of congenital afibrinogenemia.All 5 cases had pulmonary embolism, 2 cases had deep venous thrombosis of lower limbs, and 1 case had cardiac thrombosis and arterial embolism.The level of protein C was significantly decreased in 1 case, and the level of protein S in 1 case was significantly decreased in the laboratory test of thrombophilia; 2 cases were positive for antiphospholipid antibodies in the acute phase, but negative after 3-6 months of re-examination.Genetic analysis showed 2 cases of PROC gene mutation, 1 case of PROSI gene mutation, 1 case of F5 gene mutation, and 1 case of FGA gene mutation.All children were treated with anticoagulation drugs for long-term, including 4 patients with Warfarin and 1 patient with Rivaroxaban.The follow-up time ranged from 3 months to 5 years.During the follow-up, 1 patient experienced thrombosis recurrence due to infection incentives 1 month after discontinuing anticoagulant drugs on his own. Conclusions:The clinical manifestations of children with IT are the same as those of adults, mainly including venous thromboembolism(VTE); there are limitations in laboratory detection of thrombophilia, and gene analysis is of great significance.Children diagnosed with IT need long-term anticoagulant therapy to reduce the recurrence of VTE.
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Monkeypox is a zoonotic disease.Previous studies have shown that children are vulnerable to monkeypox and are also at high risk for severe disease or complications.In order to improve pediatricians′ understanding of monkeypox and achieve early detection, early diagnosis, early treatment and early disposal, the committee composed of more than 40 experts in the related fields of infectious diseases, pediatrics, infection control and public health formulate this expert consensus, on the basis of the latest clinical management and infection prevention and control for monkeypox released by the World Health Organization (WHO), the guidelines for diagnosis and treatment of monkeypox (version 2022) issued by National Health Commission of the People′s Republic of China and other relevant documents.During the development of this consensus, multidisciplinary experts have repeatedly demonstrated the etiology, epidemiology, transmission, clinical manifestations, laboratory examinations, diagnosis and differential diagnosis, treatment, discharge criteria, prevention, case management process and key points of prevention and control about monkeypox.
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Objective:To investigate the clinical features of Kawasaki disease complicated with multiple pulmonary nodules and to summarize the experience of diagnosis and treatment.Methods:Clinical data of 2 cases of Kawasaki disease complicated with multiple pulmonary nodules admitted to the Department of Respiratory, Beijing Children′s Hospital in 2019 were retrospectively analyzed, and relevant literature was reviewed.Results:The 2 cases were both 3-month-old infants, with fever as the main manifestation.Both were consistent with the diagnosis of Kawasaki disease, and the respiratory symptoms were mild or not obvious.Radiological examination revealed multiple pulmonary nodules, and the results of etiology, immunology and tumor-related exams were all negative.In the follow up stage after treatment, pulmonary nodules gradually disappeared.Among 10 cases of Kawasaki disease with pulmonary nodules (8 cases from previous studies and 2 cases from this report), 90% were 3-6-month-old babies, 80% were diagnosed with incomplete Kawasaki disease, and all patients presented more than 2 nodules in the lungs, with irregular distribution and different sizes.Besides, 80% of the 10 cases had coronary artery involvement of varying degrees.One patient died in the acute disease phase, while pulmonary nodules of the remaining patients disappeared in 12 days to 1 year.Conclusions:Kawasaki disease may be complicated with multiple pulmonary nodules, especially in young infants.Most of the patients present with incomplete Kawasaki disease and are prone to coronary artery lesions, which may be related to pulmonary vascular involvement caused by Kawasaki disease.The prognosis is mostly good, and pulmonary nodules can disappear naturally with the treatment and course of Kawasaki disease.
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Since December 2019, severe acute respiratory syndrome coronavirus 2(SARS-CoV-2) infections have raged globally for more than 2 years.China has always adopted scientific and effective prevention and control measures to achieved some success.However, with the continuous variation of SARS-CoV-2 cases and imported cases from abroad, the prevention and control work has become more difficult and complex.With the variation of the mutant strain, the number of cases in children changed, and some new special symptoms and complications were found, which proposed a new topic for the prevention and treatment of SARS-CoV-2 infection in children in China.Based on the third edition, the present consensus according to the characteristics of the new strain, expounded the etiology, pathology, pathogenesis, and according to the clinical characteristics and experience of children′s cases, and puts forward recommendations on the diagnostic criteria, laboratory examination, treatment, prevention and control of children′s cases for providing reference for further guidance of effective prevention and treatment of SARS-CoV-2 infection in children in China.
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Objective:To investigate epidemiological characteristics and macrolide-resistance of hospitalized children with Mycoplasma pneumoniae (MP) infections in Beijing from 2016 to 2019, so as to provide basis for the prevention and treatment of pediatric Mycoplasma pneumoniae pneumonia (MPP).Methods:The clinical data were analyzed retrospectively from 8 691 children hospitalized with community acquired pneumonia in Beijing Children′s Hospital between January 2016 and September 2019.MP RNA was detected by simultaneous amplification and testing (SAT), and macrolide resistance of MP was examined by MP and macrolide-resistant isolate diagnostic kit (PCR with fluorescence probes). Chi- square test was used for categorical analysis. Results:Among 8 691 cases detected by SAT, the overall detection rate of MP was 28.10% (2 442/8 691 cases). The detection rates of MP from 2016 to 2019 were 26.23%, 31.36%, 27.84 % and 26.57%, respectively.The detection rate of MP in 2017 was significantly higher than that in other years ( χ2=16.11, P<0.05). The detection rate of MP in females was 29.65%(1 107/3 733 cases), which was evidently higher than that in males 26.93%(1 335/4 958 cases) ( χ2=7.85, P<0.05). The positive rates of MP in summer[32.21% (726/2 254 cases)] and autumn[39.76%(852/2 143 cases)] were significantly higher than those in spring[17.00% (327/1 924 cases)] and winter[22.66%(537/2 370 cases)] ( χ2=315.15, P<0.001). The percentages of MP were 35.06%(732/2 088 cases) in preschoolers and 37.71%(1 160/3 076 cases) in school-age children, which were significantly higher than 11.20%(232/2 072 cases) in infants and 22.01% (318/1 445 cases) in toddlers ( χ2=509.89, P<0.001). Macrolide resistance detection was conducted in 1 524 patients by fluorescent PCR.Among them, 1 386 patients were positive for drug resistance, and the positive rate was 90.94%.The prevalence of macrolide-resistant MP from 2016 to 2019 were 88.19%, 90.93%, 90.56% and 92.90%, respectively.Macrolide-resistant rates were not related with gender, age and season. Conclusions:MP can be detected in all seasons, but most prevalently in summer and autumn.Girls are more prone to MP infections than boys.The detection rate of MP increases with age, and the positive rate is higher in preschoolers and school-age children.During the 4-year study period, the drug resistant rate of MP remain high.
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Group A Streptococcus (GAS) is a very important pathogen, especially for children.On a global scale, GAS is an important cause of morbidity and mortality.But the burden of disease caused by GAS is still unknown in China and also has not obtained enough attention.For this purpose, the expert consensus is comprehensively described in diagnosis, treatment and prevention of GAS diseases in children, covering related aspects of pneumology, infectiology, immunology, microbiology, cardiology, nephrology, critical care medicine and preventive medicine.Accordingly, the consensus document was intended to improve management strategies of GAS disease in Chinese children.
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Cystic fibrosis (CF) is a monogenetic disease involving multiple organs.Malnutrition is common in children with CF, and it is closely related to the prognosis and survival of children.Malnutrition is caused by various factors and individualized plans should be made for children with CF at different stages.Besides, it is essentially important to monitor and assess the nutritional status of CF patients in a long term.In 2020, the 8 th edition of the clinical guidelines for the care of children with CF in Royal Brompton Hospital was updated, in which the nutritional management of children with CF was recommended and guided.This part of the guidelines was interpreted in the present paper, including nutritional management and evaluation, tips for pancreatic enzyme replacement therapy, types of nutrition supplement and how to solve the feeding difficulties of children with CF.The aim of this paper is to guide early clinical nutritional management of CF patients and improve their prognosis.
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Cystic fibrosis (CF) is a life-threatening autosomal recessive hereditary disease, involving multiple organs and systems such as respiratory and digestive systems.Malnutrition caused by various factors is common in CF children.CF children with pancreatic dysfunction suffer from malabsorption and/or growth disorder at birth or 1 year old.Nutritional status is associated with lung function and prognosis of CF patients, so nutritional management is extremely important to CF children.However, there is inadequate nutritional management for CF children in clinical practice in China, and Chinese CF children has shorter survival time than CF children in European and American countries.Thus, in this study, the importance of nutritional management, nutritional management methods, nutritional assessment and monitoring of CF patients were introduced.The aim is to enhance clinical attention to the nutritional status of CF children and clinical understanding of nutritional management.Early identification and intervention can improve prognosis and prolong the life of pediatric CF patients.
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The clinical data of a cystic fibrosis (CF) child with allergic bronchopulmonary aspergillosis (ABPA) and hemoptysis in the Department of Respiratory Disease Ⅰ, Beijing Children′s Hospital, Capital Medical University in May 2021 were retrospectively analyzed.Meanwhile, relevant literature was reviewed to analyze the clinical characteristics, diagnosis and treatment of CF patients with ABPA.This patient was a 15 years and 4 months old boy and complained of recurrent cough with sputum.The test showed increased blood eosinophils, total serum IgE higher than 500 IU/mL, positive aspergillus fumigatus specific IgE and IgG antibodies.Chest CT revealed central bronchiectasis and high-density mucus thrombus, and the patient was initially diagnosed with ABPA.Further examinations suggested the sweat chloride concentration was 89 mmol/L, and the genetic results showed a compound heterozygous mutation of CFTR (c.2909G>A from his father, c.3310G>T from his mother). Then, he was diagnosed with CF complicated with ABPA and treated with glucocorticoid and antifungal therapy.The disease was repeated after drug withdrawal.Due to hemoptysis, the right upper lobe lobectomy was performed.Unfortunately, ABPA occurred again 2 years later.The child is being followed up at present.CF is a rare monogenetic disease with poor prognosis.It is difficult to treat CF patients with ABPA and the disease repeats easily.Early identification and treatment will improve the prognosis.
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Cystic fibrosis (CF) is an autosomal recessive monogenetic disease caused by homozygous and double-heterozygous mutations of the cystic fibrosis transmembrane conductance regulator ( CFTR)gene.The CFTR gene encodes a chloride ion channel protein, which is distributed in the airway, pancreatic duct epithelium and other body parts.CFTR dysfunction will give rise to a series of clinical manifestations.Remarkable achievements have been made in specific molecular regulatory therapy targeting CFTR dysfunction over the past 10 years.CF has thus become a model disease, which provides new approaches for the research on genetics, molecular and cellular pathogenesis, and drug discovery of other rare genetic diseases.In this article, the latest research progress on the pathogenesis and molecular regulatory therapy of CF in recent years was reviewed to improve clinical understanding of the disease mechanism and molecular regulatory therapy.
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Hypoxemia is a common complication of pneumonia, asthma, and bronchopulmonary dysplasia in children.Rapid identification of hypoxemia is of great significance for the disposal and management of critical children.Pulse oximetry is recognized by the World Health Organization as the best way to monitor hypoxemia in children, and it can monitor pulse oxygen saturation noninvasively and continuously.Based on the related literature at home and abroad, combined with the clinical needs of pediatrics, the " Expert consensus on clinical application of pulse oximetry in children" is formulated to improve the understanding of pediatricians and nurses on the application in pediatric clinical practice, principle, operation techniques, and limitations of pulse oximetry.
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Objective:To investigate the knowledge, attitudes, and practices (KAP) of pulse oximetry among pediatric healthcare providers in China and analyze the factor influencing the KAP.Methods:A self-developed questionnaire was used for an online research on the KAP of 11 849 pediatric healthcare providers from 31 provinces, autonomous regions, and municipalities of China from March 11 to 14, 2022.The factors influencing the KAP of pulse oximetry among pediatric healthcare providers were examined by Logistic regression. Results:The scores of KAP, of pulse oximetry were 5.57±0.96, 11.24±1.25 and 11.19±4.54, respectively.The corresponding scoring rates were 69.61%, 74.95%, and 55.99%, respectively. Logistic regression results showed that the gender and working years of pediatric healthcare providers, the region they were located, and whether their medical institution was equipped with pulse oximeters were the main factors affecting the knowledge score (all P<0.05). Main factors influencing the attitude score of pediatric healthcare providers included their knowledge score, gender, educational background, working years, region, medical institution level, and whether the medical institution was equipped with pulse oximeters (all P<0.05). For the practice score, the main influencing factors were the knowledge score, gender, age, and whether the medi-cal institution was equipped with pulse oximeters (all P<0.05). Conclusions:Chinese pediatric healthcare providers need to further improve their knowledge about and attitudes towards pulse oximetry.Pulse oximeters are evidently under-used.It is urgent to formulate policies or guidelines, strengthen education and training, improve knowledge and attitudes, equip more institutions with pulse oximeters, and popularize their application in medical institutions.