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06 May 2019, Volume 34 Issue 5 Previous Issue    Next Issue

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Expert consensus on the diagnosis of hypertrophic cardiomyopathy in Chinese children 2019, 34(5): 329-334.  DOI: 10.19538/j.ek2019050601 Abstract ( )

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Expert consensus on immunization in children with special health state（ⅩⅨ）：immunosuppressive agents and immunization Center for Disease Control and Prevention in Shanghai，Center for Disease Control and Prevention in Hangzhou，Center for Disease Control and Prevention in Suzhou，et al 2019, 34(5): 335-336.  DOI: 10.19538/j.ek2019050602 Abstract ( )

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Expert consensus on immunization in children with special health state（ⅩⅩ）：immunization for children receiving intravenous immunoglobulin Center for Disease Control and Prevention in Shanghai，Center for Disease Control and Prevention in Hangzhou，Center for Disease Control and Prevention in Suzhou，et al 2019, 34(5): 336-337.  DOI: 10.19538/j.ek2019050603 Abstract ( )

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Expert consensus on immunization in children with special health state（ⅩⅪ）：inherited metabolic disease and immunization Center for Disease Control and Prevention in Suzhou，Center for Disease Control and Prevention in Shanghai，Center for Disease Control and Prevention in Hangzhou，et al 2019, 34(5): 338-339.  DOI: 10.19538/j.ek2019050604 Abstract ( )

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Precision diagnosis and treatment for hypertrophic cardiomyopathy in children: Current opportunities and challenges LI Zi-pu*，HAN Ling 2019, 34(5): 340-346.  DOI: 10.19538/j.ek2019050605 Abstract ( )   Hypertrophic cardiomyopathy（HCM） is the second commonest cardiac muscle disease affecting children and adolescents and is a leading cause of sudden death in young athletes. HCM is highly heterogeneous in pediatric population，and currently the precision diagnosis of the etiology of the disease faces enormous challenges. In addition，prospective and randomized controlled clinical studies in the diagnosis，treatment and prognosis of pediatric HCM are extremely rare. Evidence-based consensus or guidelines needs to be developed urgently for personalized risk assessment and treatment，as well as standardized family management and genetic counseling.

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Progress in etiology of hypertrophic cardiomyopathy in children ZHANG Xiao-jian，FENG Song，CHEN Xiao-guang，et al 2019, 34(5): 347-352.  DOI: 10.19538/j.ek2019050606 Abstract ( )   Hypertrophic cardiomyopathy（HCM） in children is a kind of common cardiovascular genetic abnormalities disease，mostly single gene autosomal dominant inherited cardiomyopathy，although it does not exclude other genetic patterns. The main features of HCM are symmetrical or asymmetrical ventricular hypertrophy，and histopathological characteristics are myocyte hypertrophy，being disorganized，and fibrosis. Generally，HCM in children is divided into primary and secondary types. Mutation of sarcomere protein gene is the main cause of primary cardiomyopathy，followed by mutation of non-sarcomere protein gene. Mutation of MYH7，MYBPC3，TNNT and other genes are the main causes of sarcomere HCM. Secondary causes include obesity，diabetic mothers and babies，athlete syndrome，abnormal hormone elevation or drug-induced and so on.

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Echocardiographic evaluation of hypertrophic cardiomyopathy in children DING Wen-hong，HAN Ling，LIU Xin-han 2019, 34(5): 352-356.  DOI: 10.19538/j.ek2019050607 Abstract ( )   The transthoracic echocardiography（TTE） plays a critical role in the diagnosis and evaluation of pediatric hypertrophic cardiomyopathy（HCM），and is currently recognized as the first choice in investigation of HCM. Comprehensive TTE is recommended in all children suspected with HCM. To meet the criteria of diagnosis of HCM，the left ventricular wall thickness at any or more segments of left ventricular myocardium should be greater than the predicted mean plus two standard deviations. In the meanwhile，other diseases，such as hypertension，valvular diseases and aortic diseases，which could contribute to increased cardiac load，should be excluded. Besides the measurement of the thickness of the hypertrophic ventricular wall，we should also pay attention to the description of myocardial morphology，myocardial echo intensity，uniformity，and outflow tract obstruction. It is also necessary to assess ventricular size and general systolic and diastolic function，and apply advanced techniques to evaluate myocardial function in order to fully assess the state of the disease or to make differential diagnosis. In addition， echocardiography also has a wide range of application prospects in the treatment of HCM.

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CMR and CT examination of hypertrophic cardiomyopathy in children TAN Ying，LYU Tie-wei 2019, 34(5): 357-362.  DOI: 10.19538/j.ek2019050608 Abstract ( )   Hypertrophic cardiomyopathy（HCM） is an inherited cardiomyopathy characterized by left ventricular hypertrophy without ventricular enlargement，which is one of the main causes of athletic sudden death in adolescents. Early detection of symptoms and timely diagnosis and treatment are of great value in preventing athletic sudden death and improving the prognosis. The diagnosis of HCM is based on medical history，physical examination and echocardiography，as well as family history and genetic testing in recent years. In recent years，cardiac magnetic resonance（CMR） and CT examination have become more and more important in the diagnosis of HCM. The aim of the article is to explore the characteristics of CMR and CT in the diagnosis of HCM.

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Research progress in genetics of hypertrophic cardiomyopathy in children ZHANG Yan-min 2019, 34(5): 362-367.  DOI: 10.19538/j.ek2019050609 Abstract ( )   Hypertrophic cardiomyopathy（HCM） is one of the leading causes of sudden cardiac death（SCD） in children and young adults. The incidence of HCM in adults is 1/500，which is mainly coding sarcomere-associated protein gene mutations. The most common are MYH7 and MYBPC3. The incidence of HCM in children is unclear，and the etiology is more complicated. The clinical manifestations are highly heterogeneous. There are many kinds of non-sarcomere mutations，including metabolic storage diseases，RASopathies，neurodegenerative diseases and mitochondrial diseases. Up to now，more than 40 genes are associated with pediatric HCM. Multiple modes of inheritance account for HCM，among which autosomal dominant inheritance is the most common mode. Others include autosomal recessive，X-linked，and mitochondrial inheritance.

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Clinical classification，risk factors assessment and drug treatment of hypertrophic cardiomyopathy in children NA Jia， YUAN Yue 2019, 34(5): 367-370.  DOI: 10.19538/j.ek2019050610 Abstract ( )   Hypertrophic cardiomyopathy（HCM） is mainly hereditary heart disease caused by gene mutation encoding cardiac sarcomere protein. HCM is the main cause of sudden cardiac death in athletes and adolescents. The clinical manifestations of HCM in children are complex and varied，including being asymptomatic，exercise intolerance，syncope，and sudden death，etc. Labor dyspnea and chest pain are the most common symptoms in older children. The diagnosis of HCM in children is mainly based on imaging examination，which can be divided clinically into three types：obstructive，non-obstructive and occult obstructive. Risk factors were assessed according to the patient’s age，clinical symptoms，imaging findings and family history to guide further treatment，management and prognosis. Drug therapy mainly includes beta blockers，non-dihydropyridine calcium antagonists and other anti-arrhythmic drugs，as well as anticoagulants，diuretics and other applications. For those who are not satisfied with the effect of drug treatment，implantable cardioverter defibrillator（ICD） or surgical treatment can be used. In recent years，with the development of gene detection，the diagnosis of HCM in children is more refined and the treatment is accurate.

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Surgical treatment for hypertrophic cardiomyopathy in children YAN Jun，DUAN Ya-bing 2019, 34(5): 371-374.  DOI: 10.19538/j.ek2019050611 Abstract ( )   Hypertrophic cardiomyopathy（HCM） is the second commonest form of heart muscle disease affecting children and adolescents and is a leading cause of sudden death in young athletes. The aetiology of HCM is heterogeneous in the paediatric population，and includes inborn errors of metabolism，neuromuscular disorders and malformation syndromes. However，most cases of apparently idiopathic HCM in childhood are caused by mutations in cardiac sarcomere protein genes. Hypertrophic obstructive cardiomyopathy（HOCM） is not uncommon in children who don’t respond to drug treatment；for such children，surgical treatment is the only solution. Previous routine surgical methods include modified Konno operation，modified extended Morrow operation and interventional alcohol ablation or radiofrequency ablation for pediatric patients. At present， the reports and large-scale clinical experience are rare.

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Manifestations and management of electrocardiogram abnormalities in children with hypertrophic cardiomyopathy SUN Ling，SHEN Jie 2019, 34(5): 374-379.  DOI: 10.19538/j.ek2019050612 Abstract ( )   Hypertrophic cardiomyopathy（HCM） is a common cause of sudden deaths in children and adolescents，mostly due to ventricular arrhythmia. The abnormal ECG activity of HCM is related to the histological characteristics of myocardial hypertrophy. Although ECG lacks specificity，some changes still have certain sensitivity in HCM，or give hints to potential causes. These can provide valuable basis for the early diagnosis，as well as the monitoring and management of high-risk patients，and help estimate the time of ICD treatment.

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Hypertrophic cardiomyopathy associated with malformation syndrome in children WANG Ben-zhen，LI Zi-pu 2019, 34(5): 379-384.  DOI: 10.19538/j.ek2019050613 Abstract ( )   Hypertrophic cardiomyopathy（HCM） is defined by the presence of increased left ventricular（LV） wall thickness that is not solely explained by abnormal loading conditions. It includes familial HCM caused by mutations in the genes encoding the cardiac muscle branch contraction-associated protein，as well as hereditary HCM caused by mutations in genes encoding non-muscle-contracting system-associated proteins. Malformation syndromes are symptom complex characterized by multi-system and multi-organ abnormality. HCM can be the only or major manifestation of its cardiovascular system lesions. The article will briefly introduce HCM associated with malformation syndrome in children.

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Hypertrophic cardiomyopathy associated with inborn errors of metabolism in children YAN Hui，CHEN Yong-hong 2019, 34(5): 385-388.  DOI: 10.19538/j.ek2019050614 Abstract ( )   Inborn errors of metabolism in children is an important cause of hypertrophic cardiomyopathy. Characteristic manifestations of the diseases are helpful for rapid diagnosis. Most of the diseases are autosomal recessive inheritance，a few of them are autosomal dominant inheritance，X-linked inheritance，while some mitochondrial diseases are maternal inheritance. Conventional cardiac examinations such as electrocardiogram and echocardiography can provide diagnostic clues for the underlying causes of some diseases. With the rapid progress of new treatment methods such as enzyme replacement therapy，strengthening cardiac assessment，appropriate treatment for underlying diseases and multi-disciplinaries collaboration will provide more opportunities of survival for the patients.

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Hypertrophic cardiomyopathy associated with mitochondrial disease in children BAI Wei，QI Jian-guang 2019, 34(5): 388-392.  DOI: 10.19538/j.ek2019050615 Abstract ( )   Mitochondrial disease is a heterogeneous group of hereditary diseases caused by the defects in the mitochondrial respiratory chain and abnormal cellular energy metabolism. Heart is one of the most common organs involved，and hypertrophic cardiomyopathy is the most common and important type of cardiac involvement in mitochondrial disease. Hypertrophic cardiomyopathy in the patients with mitochondrial disease with childhood onset is more common than those with adulthood onset. Mortality in children with cardiac involvement caused by mitochondrial disease is significantly higher than that in children without cardiac involvement，so the early diagnosis and treatment is very important. But the early diagnosis is still difficult due to the complexity of clinical manifestations of mitochondrial disease. There is no specific treatment for mitochondrial disease and its associated hypertrophic cardiomyopathy，so supportive therapy is still the main treatment.

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A multicenter study of 1823 cases of hospitalized childhood cardiomyopathy from 2006 to 2016 Cooperation Group of Precision Diagnosis and Treatment of Cardiomyopathy in Children，the Subspecialty Group of Cardiology，the Society of Pediatrics，Chinese Medical Association 2019, 34(5): 393-399.  DOI: 10.19538/j.ek2019050616 Abstract ( )   Objective　To analyze the clinical data of hospitalized children with cardiomyopathy in 16 hospitals in China in nearly 10 years，and to provide evidence for its precision diagnosis and treatment. Methods　The clinical data of hospitalized children treated in 16 pediatric departments in China from July 2006 to June 2016 were analyzed retrospectively，which included the gender，age，cardiac function on admission，concomitant disease on admission，electrocardiogram，echocardiograph，laboratory examinations and genetic tests；Their conditions after discharge were followed up by making telephone calls. Results　A total of 1823 hospitalized children with cardiomyopathy were admitted，accounting for 0.1% of pediatric hospitalized children during the same period. Dilated cardiomyopathy（DCM） was the most common kind of cardiomyopathy（33.57%），followed by endocardial fibroelastosis（27.81%） and left ventricular noncompaction（LVNC）（11.03%）.The age at onset was diverse. The largest percentage was 0-6 month-old children（27.3%），followed by 1-3 year-olds（22.7%）. A total of 43.2% of the patients’cardiac function was NYHA class Ⅲ or above and Ross grade 7 or above，and 4.3% had cardiogenic shock；the incidence of thromboembolism during hospitalization was 0.6%，mainly in patients with DCM and LVNC. Arrhythmia was presented in 36.3% of patients，and the most common types included premature beats，tachycardia，conduction block，and pre-excitation syndrome. Echocardiography revealed 18.3% of the patients had pericardial effusion. The incidence of valvular regurgitation was 77.6%，and mitral regurgitation was more common. The overall mortality rate was 2.80%. Conclusion　The hospitalized children with cardiomyopathy have increased year by year. The common types of cardiomyopathy are DCM，EFE，and LVNC. The age of onset is under age 3 in most children with cardiomyopathy，and arrhythmia is the most common complication. Only 3.24% of the patients completed genetic test. It is necessary to further improve the diagnosis and treatment of childhood cardiomyopathy.

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Analysis of safety and efficacy of propofol used as anesthesia and deep sedation during  flexible bronchoscopy  in  children DONG Wei-ran*，AN Shu-hua，LI Quan-heng，et al 2019, 34(5): 400-404.  DOI: 10.19538/j.ek2019050617 Abstract ( )   Objective　To investigate the efficacy and  safety of propofol used as anesthesia and deep sedation during flexible bronchoscopy in children. Methods　The clinical data of 206 children with atelectasis who underwent flexible bronchoscopic alveolar lavage in the Endoscopy Room of the Respiratory Department of Hebei Children’s Hospital from January 2016 to January 2017 were retrospectively analyzed. Children for  ASAⅠ/Ⅱ level were divided into two groups according to the sedation method :there were 106 cases in the propofol group（2 mg/kg） and 100 cases in the midazolam group（0.1 mg/kg）. To compare the onset time of anesthetict，heart rate（HR），respiratory rate（RR），mean arterial pressure（MAP），percutaneous oxygen saturation（SPO2） before and after anesthesia induction（T0，T1），during endoscopy placement（T2）and after awakening（T3） at 4 time points，operative duration，the waked duration of postoperation，the rate of  adverse reactions（hiccups，respiratory depression）in the operation and Ramsay score between two groups. All data were analyzed by SPSS 20.0 statistical software. Results　（1）There were statistically significant differences at the onset time of anestheticts，operative duration and the rate of side-effects between the two groups（P＜0.05）. There was no statistical differences in awake time（P＞0.05）.（2）There was no significant difference in MAP or SPO2 at time points of T0，T1，T2，T3（P＞0.05），whereas the difference in HR and RR at time points of  T1，T2，T3 between the two groups was statistically significant（P＜0.05）. （3）The sedative effect of propofol group was significantly better than that of midazolam group in Ramsay sedative scoring. Conclusion　Propofol used for anesthesia and deep sedation works fast，safely and effectively in flexible bronchoscopy for children；the time to gain consciousness is short，the operation time is short and there is fewer side effects，which is worth promoting.

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Application analysis of temporary pacing for children with bradyarrhythmia LIU Xiang，LYU Tie-wei 2019, 34(5): 405-409.  DOI: 10.19538/j.ek2019050618 Abstract ( )   Objective　To explore the effects and results of temporary cardiac pacing in children with bradyarrhythmia. Methods　The clinical data of 55 children with bradyarrhythmia who received temporary cardiac pacing between June 2007 and May 2018 in Children’s Hospital of Chongqing Medical University were analyzed retrospectively. The clinical characteristics and curative effects were summarized and statistically analyzed. Results　A total of 55 cases of bradyarrhythmia included 29 cases of severe atrioventricular block（AVB），sinus bradycardia or cardiac arrest caused by myocarditis，5 cases of Ⅲ°AVB or sick sinus syndrome caused by cardiomyopathy，6 cases of Ⅲ°AVB caused by congenital heart disease after surgery，5 cases of congenital Ⅲ°AVB，and 10 cases of perioperative temporary pacing Ⅲ°AVB. Among them，39 cases were compared in Adams-Stokes syndrome（P＝0.003），34 cases were compared in ejection fraction and fractional shortening（P＝0.000，P＝0.001），and 26 patients were compared in left ventricular end diastolic diameter（P＝0.001） before and after temporary pacing，and there were statistically differences. After treatment，18 cases were clinically cured，36 cases were improved，and 1 case died，among which 45% arrhythmia were improved or recovered，and 10 cases of perioperative children all passed through the anesthesia period. Kruskal-Wallis H(K） test showed that the clinical and arrhythmia prognosis of different basic cardiovascular diseases were not completely the same（P＝0.001，P＝0.000）； Mann-Whitney U test indicated a better prognosis in the myocarditis group（P＝0.001，P＝0.000）.  Conclusion　Temporary cardiac pacing can effectively relieve the clinical symptoms of severe bradyarrhythmia caused by various etiologies，promote the recovery of acute conduction system injury，and reduce Ⅲ°AVB perioperative risk. Temporary cardiac pacing is a safe and effective prevention and first-aid technique.

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Risk factors of purulent meningitis complicated with subdural effusion in infants and young children WANG Xian-zhu，LI Jiu-jun 2019, 34(5): 410-413.  DOI: 10.19538/j.ek2019050619 Abstract ( )   Objective　To investigate the risk factors of purulent meningitis complicated with subdural effusion in infants and young children. Methods　The clinical data of the infants and young children who were diagnosed with purulent meningitis in PICU of Shengjing Hospital of China Medical University from January 2014 to December 2017 were analyzed retrospectively. All of them were divided into 2 groups according to whether there was complication of subdural effusion. The statistical data were analyzed by SPSS 20.0 software. Results　There were significant differences in hemoglobin，C reactive protein and protein in cerebrospinal fluid between control group and subdural effusion group（P＜0.05）. Logistic regression analysis showed that hemoglobin（OR＝0.940，95%CI：0.899—0.998），C reactive protein（OR＝1.015，95%CI：1.004—1.028） and protein in cerebrospinal fluid（OR＝2.490，95%CI：1.151—6.315） were independent risk factors for purulent meningitis complicated with subdural effusion（P＜0.05）. Conclusion　Infants and young children diagnosed with purulent meningitis are with lower hemoglobin. Higher C reactive protein and higher protein in cerebrospinal fluid are likely to be complicated with subdural effusion.

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Clinical features and risk factors analysis in children with acute leukemia complicated with multidrug-resistant bloodstream infection WU Yi-li，YU Li-hua，LIN Dan-na，et al 2019, 34(5): 414-418.  DOI: 10.19538/j.ek2019050620 Abstract ( )   Objective　To investigate the clinical features and risk factors of multidrug-resistant bloodstream infection in children with acute leukemia. Methods　The clinical data of 121 blood culture-positive patients with acute leukemia admitted from January 1，2013 to September 30，2018 to Department of Pediatrics，Zhujiang Hospital of Southern Medical University were analyzed retrospectively. Results　Of the 121 patients with acute leukemia infected with bacterial bloodstream，55 were in the multidrug-resistant（MDR） group and 66 in the non-multidrug-resistant（non-MDR） group. There were 31 gram-positive bacteria in the MDR group. The top three strains were coagulase-negative Staphylococci，Staphylococcus aureus and Streptococcus mutans. Escherichia coli was the main strain of gram-negative bacteria. Logistic analysis suggested that MDR bloodstream infection was more likely to occur in the patiens with AML（P＝0.038；OR 2.505；95%CI 1.036—6.058） and at induction chemotherapy stage（P＝0.038；OR 2.226；95%CI 1.045—4.774）. Other high-risk factors included neutropenic dysplasia ＞7 d before fever（P＝0.003；OR 3.36；95%CI 1.520—7.428），hemoglobin ＜70 g/L（P＝0.122；OR 1.897；95%CI 0.842—4.274），and platelet＜20 g/L（P＝0.005；OR 2.995；95%CI 1.388—6.464）. The fever duration and antibiotic course in the MDR group were longer than those in the non-MDR group，and the procalcitoni and C-reactive protein were higher in the MDR group.  The empirical treatment of the MDR group was less effective，and the transfer rate for ICU and mortality rate were higher. Conclusion　AML，induction chemotherapy，neutrophil deficiency time before fever ＞7 days ，hemoglobin＜70 g/L and platelet＜20×109/L are risk factors for MDR bloodstream infection. The inflammation response is severe MDR bloodstream infections，which may result in longer anti-infective treatments and a worse prognosis.

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Research on prognostic influence of medical intervention for nasal disease in children’s acute laryngitis YANG Yao-wei，PANG Chong，LU Ying-xia，et al 2019, 34(5): 419-421.  DOI: 10.19538/j.ek2019050621 Abstract ( )

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Renal static imaging combined with DWI in the diagnosis of upper urinary tract infection in children ZHAO Yun，GUO Yan-yan，LI Peng，et al 2019, 34(5): 422-424.  DOI: 10.19538/j.ek2019050622 Abstract ( )

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Research progress of pulmonary regurgitation after percutaneous balloon pulmonary valvuloplasty BING Zhen，PAN Si-lin 2019, 34(5): 425-428.  DOI: 10.19538/j.ek2019050623 Abstract ( )

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Treatment progress of idiopathic pulmonary hemosiderosis in children SUN Jia-liang，LI Yan-chun，CHENG Huan-ji 2019, 34(5): 429-433.  DOI: 10.19538/j.ek2019050624 Abstract ( )

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Research progress on the long non-coding RNAs in cardiac development and congenital heart disease XIE Mei-jun，JIANG Hong，JIANG Hong-kun 2019, 34(5): 434-437.  DOI: 10.19538/j.ek2019050625 Abstract ( )

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Maturity-onset diabetes of the young type 6 with a novel NEUROD1 mutation：A case of report ZHAO Ning-ning，DONG Guan-ping，WU Wei，et al 2019, 34(5): 438-441.  DOI: 10.19538/j.ek2019050626 Abstract ( )

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Application of simulation-based medicine education in pediatric resident training WU Jing-yan，YIN Rong，ZHOU Wen-hao 2019, 34(5): 442-445.  DOI: 10.19538/j.ek2019050627 Abstract ( )   Simulation-based medicine education（SBME） refers to the clinical teaching method of using medical simulation equipment or simulator design to simulate clinical scenes，replacing the actual content of real medical scenes in an interactive way and the actual patient. Simulation is particularly important in pediatric resident training，and a large amount of evidence-based evidence confirms that simulation training used in resident training ensures patients’ safety and improves patients’outcomes. The paper mainly expounds the application principle，theoretical basis，main content and the method of establishing the simulation center in the training of pediatric resident in simulated medical education，intending to promote the further development of simulation training in the training of domestic pediatric resident.

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Practice of clinical thinking and clinical judgement in the standardized resident training CAI Xiao-hong，CAI Hui-lin，YU Chen-yi，et al 2019, 34(5): 446-448.  DOI: 10.19538/j.ek2019050628 Abstract ( )   The standardized resident training is an important step for training high-level professionals in clinical medicine and it is an important part of medical education after graduation. The training of residents’ clinical thinking and clinical judgement should be emphasized in all resident training programs. Some resident programs in China still focus on the teaching of important knowledge that are originated from the medical colleges.  Therefore，it is urgent for faculty to adopt new teaching methods aiming at the training of clinical thinking and clinical judgement. The paper introduces a mode for teaching clinical thinking and clinical judgement in the resident training programs. The goal is to improve the level of resident physicians in clinical diagnosis and treatment in China.