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06 May 2023, Volume 38 Issue 5 Previous Issue   

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Expert consensus on application of silvelestat sodium in Pediatric Intensive Care Unit Society of Pediatric Critical Care of Chinese Medical Doctor Association 2023, 38(5): 321-326.  DOI: 10.19538/j.ek2023050601 Abstract ( )   Sivelestat sodium is a specific neutrophil elastase inhibitor，which is a new drug for the treatment of acute lung injury（ALI） and acute respiratory distress syndrome（ARDS）. However，there is no specific recommendation for its application in children in pediatric intensive care unit. In order to promote the rational application of sivelestat sodium in children with critical illness，the Society of Pediatric Critical Care of the Chinese Medical Doctor Association organized experts to analyze，summarize and discuss relevant domestic and foreign literature，and formulated this consensus to provide reference for the use of sivelestat sodium in children with critical illness.

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Chinese expert consensus on diagnosis and treatment of systemic juvenile idiopathic arthritis （2023 edition） Academic Group of Pediatric Rheumatology and Immunology, Society of Pediatrics, Chinese Medical Doctor Association, Children's Immunization and Health Alliance （China）, Editorial Committee of Chinese Journal of Practical Pediatrics 2023, 38(5): 327-334.  DOI: 10.19538/j.ek2023050602 Abstract ( )   The consensus has beenreached based on the clinical experience of children's rheumatology and immunology professionals in China and in combination with relevant literature， and the diagnosis，treatment，standard treatment and long-term management of the disease are elaborated，providing a reference for children's rheumatology and immunology professionals to standardize the diagnosis and treatment of SJIA.

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Interpretation of Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia in 2021 CAI Yu-xin , CHENG Shi-tong, WANG Lu-ya 2023, 38(5): 335-340.  DOI: 10.19538/j.ek2023050603 Abstract ( )   Familial hypercholesterolemia（FH）is a serious inherited metabolic disease. Due to the various types of genetic mutations，the low-density lipoprotein cholesterol （LDL-C） is abnormally elevated，causing premature atherosclerosis and xanthoma in multiple body skin. Clinically，there are two types of FH： homozygous FH （HoFH） and heterozygous FH （HeFH）. HoFH is rare and serious，while HeFH is comparatively common. Early diagnosis and early treatment are essentially beneficial to improving patients' prognosis and long-term quality of life. In recent years，a number of guidelines for the management of adults with FH have been issued globally，but there are few reports on guidelines for children and adolescents. Recently，Associations of Preventive Pediatrics of Serbia，Mighty Medic，and the International Lipid Expert Panel have jointly published Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia and proposed a comprehensive approach to cardiovascular risk assessment and clinical management of HeFH in children.This article provides a detailed interpretation of the consensus in order to provide a reference for the cardiovascular risk assessment and clinical management of children and adolescents with HeFH in China.

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Molecular diagnosis in neonatal encephalopathy ZHOU Wen-Hao, XIAO Tian-tian 2023, 38(5): 341-344.  DOI: 10.19538/j.ek2023050604 Abstract ( )   Neonatal encephalopathy is an important clinical syndrome with large heterogeneity in etiology and prognosis，and evidence-based treatment is limited. With the develo-pment of molecular diagnostic techniques，the diagnosis and treatment of neonatal encephalopathy has entered the molecular era. Understanding the application of molecular diagnosis in neonatal encephalopathy is helpful to the diagnosis and treatment in clinical practice.

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Clinical application of multimodal magnetic resonance imaging  in  neonatal  encephalopathy HOU A-na, FU Jian-hua 2023, 38(5): 344-349.  DOI: 10.19538/j.ek2023050605 Abstract ( )   There are many kinds of encephalopathy in neonatal period，such as hypoxic ischemic encephalopathy，bilirubin encephalopathy，hypoglycemic encephalopathy，encephalopathy of prematurity，sepsis-associated enceph-alopathy，hereditary metabolic encephalopathy，mitochondrial encephalopathy and so on. Magnetic resonance imaging（MRI） can be used to diagnose and evaluate the disease，which is an ideal imaging method. Conventional MRI is most widely used，however，it is often difficult to achieve the purpose of early diagnosis and accurate identification，as well as cerebral function evaluation. Currently，a variety of MRI techniques have been developed，including diffusion weighted imaging，diffusion tensor imaging，magnetic sensitivity weighted imaging，proton magnetic resonance spectroscopy imaging，fluid attenuated inversion recovery and so on，which are collectively known as multimodal MRI technology. These techniques have the advantages of high sensitivity，strong specificity，high signal contrast，reflecting the metabolic level of brain tissue and so on，which can make up for the shortcomings of conventional MRI techniques. It has a broad application prospect for early diagnosis and differentiation of neonatal encephalopathy，evaluation of brain function and prognosis judgment.

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Bedside brain function imaging of neonatal encephalop-athy and brain development  HOU Xin-lin 2023, 38(5): 349-353.  DOI: 10.19538/j.ek2023050606 Abstract ( )   Bedside functional imaging in neonatal encepha-lopathy and brain development is an important part of neurological multimodal monitoring. Cerebral hemodynamics are detected to reflect brain function，while at the same time displaying brain structure. Near-infrared spectroscopy cerebral oxygen imaging and functional ultrasound imaging are currently used clinically. Both modalities are based on the principle of neurovascular coupling，"observing" changes in cerebral blood flow through light or ultrasound，and reflecting brain functional activity according to changes in blood flow. Near-infrared cerebral oxygen imaging monitoring can realize cerebral cortex imaging，and through the international 10-20 leads，the cerebral cortex can be refined and divided. Functional ultrasound can realize functional imaging of large cortical and subcortical fields of view （several centimeters）， detecting cerebral microvascular hemodynamic changes.

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Controversial issues in therapeutic hypothermia treatment for neonates with hypoxic-ischemic encephalopathy with therapeutic ZHANG Peng, CHENG Guo-qiang 2023, 38(5): 353-356.  DOI: 10.19538/j.ek2023050607 Abstract ( )   Outcomes of hypoxic-ischemic encephalopathy （HIE） have been improved since the widespread implement-ation of therapeutic hypothermia （TH），which is the most evidence-based treatment for HIE at present，but it is also controversial. The latest clinical and experimental studies have made us understand more about the key issues of TH，including the time，depth and duration of TH，and the suitable population of TH，such as mild HIE，preterm HIE，and HIE with infection. However，there are still unresolved problems and more clinical research is still needed. In the absence of new evidence，the current therapeutic hypothermia regimen is the best in clinical practice.

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Clinical characteristics and pathogenesis of brain injury associated with neonatal sepsis HE Yu, SHI Yuan 2023, 38(5): 356-359.  DOI: 10.19538/j.ek2023050608 Abstract ( )   Neonatal sepsis might increase the risk of brain injury and long-term poor prognosis. The dysfunction of blood-brain barrier and the inflammation caused by cytotoxicity，proinflammatory factor and oxygen radicals may contribute to the pathogenesis of neonatal sepsis-related brain injury. The neonatologists should pay attention to the neonatal sepsis related brain injury，and apart from anti-infection treatment，the follow- up of the development of central nervous system should also be made. This commentary just briefly summarizes the characteristics and pathogenesis of neonatal sepsis-related brain injury，aiming to provide evidence for the future intervention.

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Research progress of brain protection in premature infants SHI Jing, MU De-zhi 2023, 38(5): 359-364.  DOI: 10.19538/j.ek2023050609 Abstract ( )   Brain injury of premature infants can lead to severe sequelae such as cerebral palsy，cognitive impairment and mental development disorder. Stabilizing brain circulation，anti-inflammation and anti-oxidation therapies are common brain protective methods for premature infants. It is still necessary to further explore the long-term impact of these therapies on the nervous system development of premature infants. This articde focuses on the research progress in brain protection in premature infants.

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NICU to NNICU：Evolution and development WANG Lai-shuan, ZHOU Wen-hao 2023, 38(5): 364-367.  DOI: 10.19538/j.ek2023050610 Abstract ( )   Brain injury remains one of the major unsolved problems in neonatal care，with survivors at high risk of lifelong neuro-disabilities. The complex interaction between pathological processes and developmental trajectory，genetic susceptibility and environmental influences after brain injury，has significant effect on the long-term neural outcomes. A coordinated，interdisciplinary approach to understand the root cause enables early detection and offers the best chance of improving outcomes and facilitate new lines of neuroprotective treatments. The Neuro-intensive Care Unit model originates from NICU，based on consistent coordinated care for infants by a multidisciplinary team，provides opportunities for specialist education and training in neonatal neurology，neuromonitoring，neuroimaging and nursing.

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Different doses of budesonide nebulized inhalation and saline nasal irrigation in children with adenoid hypertrophy：A randomized controlled study ZHANG Yun-fei, HUANG Yue, DUAN Bo, et al 2023, 38(5): 368-375.  DOI: 10.19538/j.ek2023050611 Abstract ( )   Objective    To study the clinical effect of different doses of budesonide nebulized inhalation and saline nasal irrigation in children with adenoid hypertrophy. Methods    Sixty children （aged 2～5 years） with adenoid hypertrophy attending the outpatient clinic of the Department of Otolaryngology Head and Neck Surgery of the Affiliated Hospital of Fudan University from October 2021 to February 2022 were prospectively collected and randomly assigned to three groups （n=20 for each group） according to the random number table method： high-dose budesonide group （group A，budesonide 1 mg/dose+ saline nasal irrigation），low-dose budesonide group （group B，budesonide 0.5 mg/dose+ salin nasal irrigation） and control group （group C，salin nasal irrigation），with an observation period of 8 weeks. The χ2 test，analysis of variance（ANOVA），and repeated-measures ANOVA were used to compare the clinical efficacy in the children in the three groups. Results    A total of 54 patients were finally included in the statistical analysis. Before the intervention，there were no significant differences in baseline weight，height，A/N ratio，VAS score （including nasal congestion，snoring，cough），VAS total score， arterial oxygen saturation （SaO2）data or pulse among the three groups （P>0.05），and all cases were with nasal obstruction （level Ⅲ）. With the interventions performed，VAS score for nasal congestion（F=232.089，P<0.001），snoring （F=72.520，P<0.001），cough（F=10.636，P=0.002），open-mouth breathing（F=95.325，P<0.001） and total score（F=295.633，P<0.001） all decreased significantly，and SaO2 data （F=161.575，P<0.001） gradually increased. After 8 weeks of intervention，the VAS of nasal symptoms scores and A/N values of patients in group A and group B were significantly lower than those in group C （P<0.05）. SaO2 in group A was higher than that in group C（P<0.05）. A total of 26 adverse events were reported，including 16 cases （61.54%） of cold，which was the most common adverse event. No serious adverse events occurred throughout the study，and the investigated drugs were well-tolerated and safe. Conclusion    Budesonide nebulized inhalation combined with saline nasal irrigation can effectively control the symptoms of children and improve the life quality of children with adenoid hypertrophy，and reduce the degree of adenoid hypertrophy in some children. The treatment is well-tolerated，and has certain clinical promotion value，which can bring clinical benefits to children.However，this study is a small sample size trial，and large sample and multi-center trials are needed to further verify in increaes the credibility of the clinical trial results.

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Analysis of gene mutation of macrolides resistance in children with refractory Mycoplasma pneumoniae pneumonia ZHANG Ya-Jing, MENG Chen 2023, 38(5): 376-380.  DOI: 10.19538/j.ek2023050612 Abstract ( )   Objective    To investigate the mutations of 23SrRNAV gene in bronchoalveolar lavage fluid （BALF） of children with refractory Mycoplasma pneumoniae pneumonia （RMPP），and compare the clinical data of children with and without mutations. Methods    The clinical data of 332 hospitalized children diagnosed with RMPP as subjects from October 2021 to March 2022 in the Children's Hospital Affiliated to Shandong University were analyzed retrospectively. Bronchoscopy and BALF were performed on each subject and MP-DNA in the BALF was taken to detect the 23 SrRNA V region mutation sites（A2063G，A2064G）in BALF，and they were divided into drug-resistant gene positive group and drug-resistant gene negative group. Results    Of the 332 children with MP infectious RMPP，270 cases of site mutations were detected（P<0.05）. The patients in the site mutation group were with higher WBC，NE%，CRP，LDH，D-Di and IgE levels，increased proportion of having moderate to large amount of pleural effusion and longer time of fever，and they were more likely to suffer from necrotic pneumonic. The drug resistance gene positive group mainly showed mucosal erosion，ulcer，necrosis，plastic phlegm plug and purulent BALF. Conclusion    MP drug resistance mutations may lead to or even promote the occurrence of RMPP. Drug-resistant mutated MP infection may aggravate the condition of children with RMPP，and their imaging manifestation and bronchoscopy are more severe. Bronchoscopic manifestations have certain significance for early identification of drug resistant MP infection.

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Analysis of etiological features of children with community-acquired pneumonia HU Jia-kang, HAN Zhi-ying, ZHANG Jun-yan 2023, 38(5): 381-385.  DOI: 10.19538/j.ek2023050613 Abstract ( )   Objective    To investigate the distribution and features of pathogens of children with community-acquired pneumonia （CAP） and provide evidence for clinical diagnosis and treatment. Methods    Retrospectively analyze the pathogenic characteristics of 2665 children with CAP hospitalized in the respiratory department of Shanxi Children's Hospital from January 2019 to December 2021. Results    Pathogens were detected in 1879 of 2665 children with CAP，and the detection rate was 70.5%. It included 1608 cases of single infection （85.6%）and 271 cases of mixed infection （14.4%）. The pathogen detection rates in 2019，2020 and 2021 were 67.5%（918/1361），44.7%（144/322） and 83.2%（817/982，the highest）， respectively. Streptococcus pneumoniae was detected in all age groups，and was higher in young children and preschool than in infancy and schoolage. Respiratory syncytial virus infection was mainly in the infant group，and the detection rate of Mycoplasma pneumoniae was the highest in the school-age group. The pathogen detection rates of mild and severe CAP were 69.1% and 72.3%， respectively. Conclusion    Mycoplasma pneumoniae is the main pathogen of hospitalized children with CAP in this region，with a trend of low age. There was prevalence of Mycoplasma pneumoniae in this region in 2021. Streptococcus pneumoniae is the first bacterial pathogen of CAP in children，and RSV is the first viral pathogen. The pathogen detection rate of severe CAP caused by Mycoplasma pneumoniae is higher than that of mild CAP.

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Analysis of risk factors for ulcerative colitis complicated with Clostridium difficile infection in children WANG Rui-zi, XUE Fu-min, YU Zhi-dan, et al 2023, 38(5): 386-390.  DOI: 10.19538/j.ek2023050614 Abstract ( )   Objective    To investigate the prevalence and clinical features of children with ulcerative colitis （UC） complicated with Clostridioides difficile infection （CDI），and identify the risk factors for UC complicated with CDI. Methods    From January 2012 to November 2022，the clinical data of 107 children with UC admitted to the Department of Gastroenterology，Children's Hospital Affiliated to Zhengzhou University，who had completed C. difficile testing were collected and divided into two groups according to the occurrence of CDI. Clinical characteristics of the two groups were analyzed，and risk factors were analyzed. Results    There were 56 males （52.3%） and 51 females （47.7%） with UC，24 of whom showed CDI，with a positive rate of 22.4%. Compared with the non-CDI group，the proportion of antibiotics > 7 days （d） in the CDI group was significantly higher （P=0.001）. The serum albumin in CDI group was significantly lower than that in non-CDI group （P=0.015）. In CDI group，the proportion of hormone induction （> 4 weeks） + mesalazine maintenance therapy，hormone induction （> 4 weeks） + immunosuppressive maintenance therapy and infliximab clonal antibody therapy were higher （all P < 0.05）. Multivariate analysis showed that antibiotic use > 7 days，hormone-induced （> 4 weeks） + mesalazine maintenance therapy，hormone-induced （> 4 weeks） + immunosuppressive maintenance therapy，and infliximab clonal antibody therapy were independent risk factors for CDI. Conclusion    Antibiotic use for more than 7 days，mesalazine induction and maintenance therapy，immunosup-pressive maintena-nce therapy，infliximab therapy ，clinical type and low serum albumin are predictors of CDI in children with UC. Antibiotic use > 7 days，and immunosuppressant use are independent risk factors，which can increase the risk of CDI in children with UC and increase the probability of Clostridium difficile infection in children with UC，requiring vigilance or preventive treatment.

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Research progress of soluble form of triggering receptors expressed on myeloid cells -1 in neonatal septicemia LU Ya-nan, WAN Jun, ZHANG Xiao-mei 2023, 38(5): 391-394.  DOI: 10.19538/j.ek2023050615 Abstract ( )   Neonatal septicemia is a common severe infectious disease，which progresses rapidly and is an important cause of death in the neonatal period. Soluble form of Triggering Receptors Expressed on Myeloid cell-1 （sTREM-1） is a soluble subtype of Triggering Receptors Expressed on Myeloid cell-1 （TREM-1），which is highly expressed in inflammatory response. It can activate cell pathways and amplify inflammatory response by collaborating with multiple cell receptors. Sufficient evidence has confirmed that sTREM-1 plays an important role in the early recognition，disease progression and prognosis evaluation of neonatal septicemia. This article will review the biological characteristics，signal pathway and role of sTREM-1 in neonatal septicemia.

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Clinical and immunological features of X-linked agammaglobulinemia complicated with ichthyosis vulgaris in children MAO Xiao-lan, DENG Meng-yue, LI Yue, et al 2023, 38(5): 395-400.  DOI: 10.19538/j.ek2023050616 Abstract ( )   X-linked agammaglobulinaemia （XLA） is a primary immunodeficiency disease caused by mutations in the BTK gene. Ichthyosis vulgaris （IV） is an inherited skin disorder with mutations in filaggrin （FLG） gene with incomplete penetrance. There have been no reports on comorbidity of XLA and IV at present. This is the first report on XLA complicated with IV，which was found in the Children's Hospital Affiliated to Chongqing Medical university in May of 2017. The diagnosis of XLA was delayed due to the atypical clinical phenotype. In clinical practice，physicians should pay attention to the variation of XLA phenotype spectrum，and be alert to atypical phenotypes. Immunological examinations and gene sequencing are helpful for disease diaghosis. WES has great significance for the diagnosis of atypical genetic diseases.