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    Journal of Clinical Pediatrics    2024, 42 (1): 1-14.   DOI: 10.12372/jcp.2024.23e1129
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    Bronchodilation test and its clinical application in children
    ZHANG Hao, CHI Yanxia
    Journal of Clinical Pediatrics    2023, 41 (5): 321-327.   DOI: 10.12372/jcp.2023.23e0206
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    Bronchodilation test (BDT) is a method to detect the reversibility of spastic airway, which is mainly used in the diagnosis, differential diagnosis, treatment and follow-up of wheezing diseases. As awareness of the importance of the bronchodilatation test has increased, its clinical use has rapidly increased. Recently, a large number of researches both nationally and internationally have focued on how to interpret the results in clinical and define the positive thresholds. The purpose of this paper is to explore new standards and opinions, sort out the idea and make recommendations that are suitable for Chinese children.

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    Clinical characteristics and genetic analysis in Chinese patients with Menke-Hennekam syndrome
    TANG Yanan, YE Xiantao, GU Xuefan, YU Yongguo, XIAO Bing, SUN Yu
    Journal of Clinical Pediatrics    2023, 41 (8): 613-617.   DOI: 10.12372/jcp.2023.22e1597
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    ObjectiveTo explore the clinical phenotype and identify genetic variations in Chinese patients with Menke-Hennekam syndrome (MKHK). Methods The clinical and genetic data of seven children with MKHK were retrospectively analyzed. Results All of the seven children are presented with psychomotor developmental delay, variable degree of intellectual disability, short stature, and facial dysmorphism (including short and upslanted palpebral fissures, telecanthi, depressed nasal bridge, short nose, long philtrum, protruding or low-set ears and micrognathia), accompanied by other manifestations (2/7 feeding problems, 4/7 visual impairment, 3/7 hearing impairment, 3/7 cerebral anomaly, 2/7 distal limb malformation). The genetic findings of patientsinvolve six different variants: five missense and one in-frame deletion), all of which arose de novo. c.5218C>T and c.5225T>A (NM_004380.3) variants have not been reported previously in literature. Conclusion MKHK is a rare autosomal dominant genetic disease, most of which are caused by heterozygous missense variation in the end of exon 30 and the beginning of exon 31 of CREBBP. This study revealed six de novo variants of CREBBP, further expanding the genetic spectrum of MKHK.

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    Resistance mechanism and treatment of carbapenem resistant Pseudomonas aeruginosa
    YU Hui
    Journal of Clinical Pediatrics    2023, 41 (8): 561-565.   DOI: 10.12372/jcp.2023.23e0487
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    Pseudomonas aeruginosa is a common opportunistic pathogen of nosocomial infection, which is widely distributed in the hospital environment and can survive for a long time. Carbapenems antibiotics play an important role in the treatment of serious infections caused by Pseudomonas aeruginosa. However, the outbreak of carbapenems resistant Pseudomonas aeruginosa is particularly prominent in recent years, which has made clinical treatment facing great challenges. This article introduces the main resistance mechanism of Pseudomonas aeruginosa to carbapenems antibiotics and summarizes the application of various clinical treatment schemes, and provides a reference clinical rational use of antibiotics and treatment of carbapenems resistant Pseudomonas aeruginosa infection

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    Clinical diagnosis and treatment of Kawasaki disease-associated macrophage activation syndrome in children
    HAO Sheng, HUANG Min
    Journal of Clinical Pediatrics    2023, 41 (7): 486-491.   DOI: 10.12372/jcp.2022.23e0317
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    Kawasaki disease is a systemic vasculitis. Macrophage activation syndrome is a severe complication of pediatric rheumatic immune diseases, which is characterized by cytokine storms and has a rapid progression and high mortality rate. Early identification of Kawasaki disease-associated macrophage activation syndrome is difficult, and its main features include persistent fever, splenomegaly, elevated ferritin levels, and decreased platelets. At present, there is no uniform diagnostic criteria for this disease, which mainly refers to the diagnostic criteria of systemic juvenile idiopathic arthritis with macrophage activation syndrome and primary hemophagocytic lymphohistiocytosis. Meanwhile, this disease should be distinguished from Kawasaki disease shock syndrome and childhood multisystem inflammatory syndrome. Glucocorticoids are the main treatment drugs, and patients with poor response can use traditional immunosuppressants such as cyclosporine. Biologic agents and targeted drugs have broad prospects.

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    Clinical analysis of 558 hospitalized children with human metapneumovirus pneumonia
    LIU Xiaolan, LUO Xiaojuan, FENG Zhiguan, LIU Chunyan, BAO Yanmin, ZHENG Yuejie
    Journal of Clinical Pediatrics    2023, 41 (6): 459-463.   DOI: 10.12372/jcp.2023.22e0294
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    Objective To analyze the clinical characteristics of human metapneumovirus (hMPV) pneumonia in children and the risk factors for severe hMPV pneumonia. Methods The clinical data of children with hMPV pneumonia hospitalized from October 2020 to March 2021 were retrospectively analyzed. The patients were divided into mild group and severe group for comparative analysis. Results A total of 558 patients (309 boys and 249 girls) with hMPV pneumonia were included, and the median age was 3.4 (1.7-4.4) years. There were 515 children in mild group and 43 in severe group. One hundred and nine children were complicated with underlying disease. Compared with the mild group, the severe group had a higher proportion of children younger than 3 years old and with a history of underlying diseases, and the difference was statistically significant (P<0.05). The main clinical manifestations of children with hMPV pneumonia were fever (85.0%), cough (96.4%), wheezing (39.8%) and pulmonary moist rales (74.0%). Extra-pulmonary manifestations such as vomiting were rare. One hundred and thirteen patients had bacterial infection, and Streptococcus pneumoniae was the commonest (54 cases). The rate of antibiotic use was higher in hospitalized children with hMPV pneumonia (50.7%, 283 cases). The proportion of wheezing, shortness of breath, wheezing sound, pleural effusion and atelectasis, the level of WBC and CRP in peripheral blood and the proportion of bacterial infection in the severe group were higher than those in the mild group, and the difference was statistically significant (P<0.05). Compared with the mild group, the proportion of oxygen therapy, alveolar lavage, antibiotics and systemic glucocorticoids use in the severe group was higher, the hospital stay was longer, and the difference was statistically significant (P<0.05). Binary logistic regression analysis showed that age <3 years old, wheezing, complicated underlying diseases and CRP≥25mg/L were risk factors for severe hMPV pneumonia (P<0.05). Conclusions The main manifestations of hMPV are fever, cough, wheezing and moist rale in the lungs which is easy to be complicated with bacterial infection, but extra-pulmonary complications are rare and the overall prognosis is good. However, for children < 3 years old, children with wheezing, underlying disease, and elevated CRP need to be alert to severe pneumonia.

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    Childhood encephalopathy: a group of diseases associated with various diseases
    ZOU Liping
    Journal of Clinical Pediatrics    2023, 41 (9): 641-643.   DOI: 10.12372/jcp.2023.23e0639
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    Encephalopathy is a serious and complex disease that affects the structure and function of the brain, which can cause severe neurological symptoms and even death. Encephalopathy can be divided into two types: acute encephalopathy (acute brain dysfunction) and chronic encephalopathy (chronic brain dysfunction). Acute encephalopathy is a heterogeneous disorder consisting of multiple syndromes that can occur in any age group, but is commonest in infants and preschoolers. Acute encephalopathy can be classified according to the pathogens of previous infections, or biological and clinicopathological features, and the etiology is complex and diverse. Among them, acute necrotizing encephalopathy needs to be identified. Chronic encephalopathy in children includes developmental encephalopathy and chronic traumatic encephalopathy in children with family abuse. Children with encephalopathy can show cognitive impairment, language retardation, psychomotor retardation, and other manifestations of brain dysfunction such as convulsions. Genetic testing is one of the important methods to help identify the cause of chronic encephalopathy in children. The article introduces the different etiology and possible clinical symptoms of encephalopathy to help clinicians diagnose early and give timely treatment.

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    Application of single cell sequencing technology in Kawasaki disease research
    HUANG Min
    Journal of Clinical Pediatrics    2023, 41 (7): 481-485.   DOI: 10.12372/jcp.2023.23e0436
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    Kawasaki disease (KD) is an acute febrile illness which mainly involves the small and medium-sized blood vessels throughout the body, predominantly occurs in children under 5 years old. Coronary artery lesions are its main complication and KD is now the commonest cause of acquired heart disease in children in developed countries. Different from traditional sequencing methods, single-cell sequencing technology can sequence genetic information at single-cell resolution, which can not only solve the problem of cell heterogeneity, but also identify cell subsets, search for biomarkers, and map cell lineages. This commentary will describe the development of single cell sequencing technology and its current application in the field of KD, and provide an outlook on its development.

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    Application of exhaled nitric oxide in the diagnosis and treatment of respiratory allergic diseases in children
    WU Jinhong
    Journal of Clinical Pediatrics    2023, 41 (5): 328-332.   DOI: 10.12372/jcp.2022.23e0182
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    Expiratory nitric oxide detection is a non-invasive method to assess the state of eosinophilic airway inflammation and can predict the responsiveness of respiratory allergic diseases to corticosteroid treatment. It is mainly used for the diagnosis, differential diagnosis, treatment, and predicting prognosis and management of respiratory allergic diseases.

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    Genetic characteristics of TRPV4-related congenital skeletal disorder
    ZHANG Wenyan, YAO Ziming, ZHANG Xuejun, ZHANG Yaodong, WANG Lingfei, HU Xuyun, HAO Chanjuan
    Journal of Clinical Pediatrics    2023, 41 (7): 530-536.   DOI: 10.12372/jcp.2023.22e1509
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    Objective To identify the genetic causes of four patients with different severity of congenital skeletal disorder by genetic diagnosis, and to summarize the clinical characteristics and analyze the genotype-phenotype. Methods The clinical data of four patients were collected. The peripheral blood of the patients and their parents were collected and DNA was extracted. Whole exome sequencing of patients was performed and variants were classified following the interpretation standards and guidelines of the American College of Medical Genetics and Genomics (ACMG) and the Association for Molecular Pathology. Putative pathogenic variants were verified by Sanger sequencing. Results The four patients all carried TRPV4 heterozygous variants. Two missense variants were inherited from affected parents, and one deletion insertion variant and missense variant were de novo: c.2077G>A (p.Val693Met), c.1199G>A (p.Arg400Gln), c.1657delinsACTA (p.Tyr553delinsThrAsn) and c.259G>A (p.Glu87Lys). None of these variants have been previously reported. Patients 1-3 had varying degrees of short stature, and all 4 had congenital scoliosis and other skeletal deformities. They were diagnosed as mild Metatropic Dysplasia, Autosomal Dominant Brachyomia type 3, Parastremmatic Dysplasia with Metatropic Dysplasia, and classic Metatropic Dysplasia respectively. Their parents with the same variant also had mild bone deformity. Conclusions The phenotypes of congenital skeletal disorders caused by different variants in TRPV4 gene were widely heterogeneous. Patients often present with overlapping skeletal system abnormalities. Therefore, differential diagnosis and clinical intervention can be conducted according to the molecular diagnosis results.

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    Clinical characteristics and diagnosis of lupus nephritis in children
    XIA Zhengkun, WANG Ren
    Journal of Clinical Pediatrics    2023, 41 (12): 881-886.   DOI: 10.12372/jcp.2023.23e0979
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    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by immune dysregulation and autoantibody formation. With the progress of treatment, the survival rate of SLE patients has improved significantly. Even so, lupus nephritis (LN) is associated with high morbidity and mortality. At present, there is a lack of data on LN in children, and its diagnosis, treatment and monitoring are mainly based on guidelines for adults. Treatment is mainly based on hormones and immunosuppressants. Recently, biologics have been used to treat LN with good results and no obvious adverse reactions. This article reviews the progress of epidemiology, clinical features, pathogenesis, diagnosis and treatment of LN in children.

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    Clinical applications of human milk oligosaccharides in infants
    JIANG Lu, CAI Wei
    Journal of Clinical Pediatrics    2023, 41 (7): 556-560.   DOI: 10.12372/jcp.2023.23e0465
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    Human milk oligosaccharides (HMOs) are unique and abundant complex carbohydrates in breast milk. As the third most abundant component in breast milk, they have gradually received attention from scholars both domestically and internationally. With the rapid development of analytical techniques and multiomics, the research field of HMOs has made great progress and made great breakthroughs in the past few years. However, we are still in the initial stage of revealing the role and mechanisms of HMOs. However, the conclusions are mainly from foreign clinical studies, and need to be further verified by high-quality randomized clinical trials. In addition, we need to consider its effectiveness in different diseases, environment, and populations. Here, we summarize the roles of HMOs in regulating gut microbiome development, intestinal health, immune system development, and brain development in infants. Additionally, we explore the potential future applications of HMOs in infants in China.

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    Clinical analysis of the criteria of obstructive ventilation dysfunction in children in Shanghai
    WU Yufen, DONG Wenfang, PAN Chunhong, ZHANG Hao
    Journal of Clinical Pediatrics    2023, 41 (5): 339-345.   DOI: 10.12372/jcp.2023.23e0183
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    Objective To analyze the clinical rationality of pulmonary function parameters associated with obstructive ventilation dysfunction in children. Methods Children aged 6-18 years who attended the respiratory department and completed pulmonary function tests from January to December 2021 were selected and grouped at an age interval of one year. The relevant lung function parameters such as forced vital capacity (FVC), maximum vital capacity (VCmax), forced expiratory volume in the first second (FEV1), FEV1/FVC, FEV1/VCmax were detected. The clinical consistency between measured and measured/predicted value of FEV1/FVC, measured and measured/predicted value of FEV1/VCmax, respectively, in the interpretation of obstructive lesions was compared. Results A total of 7025 children were enrolled, including 4543 males and 2482 females with median age of (8.6±2.5) years old, with the largest number of children aged 6-10 years old. Except for 10-11 and 11-12 age group, the volume correlation parameters FVC, VCmax and FEV1 were greater in males than in females, and their absolute values increased with the increase of age. There was a slight difference between VCmax and FVC in all age groups, and VCmax was slightly higher than FVC. The measured and predicted values of FEV1/FVC in females were higher than those in males except 15-16 and 16-17 age group. The measured and predicted values of FEV1/VCmax for females were higher than those for males except for 13-14 and 16-17 age group. When the measured value of FEV1/FVC < 80% and the measured/predicted value of FEV1/FVC < 92%, the interpretation inconsistency rate is 6.8%, and the interpretation inconsistency rate between the measured value of FEV1/FVC and the measured value of FEV1/VCmax is 5.0%. Finally, the ideal FEV1/VCmax measured/predicted value is 93.9%. Conclusions The measured/predicted value of FEV1/VCmax <93.9% can be used as a clinical reference standard for the interpretation of obstructive ventilation dysfunction in children.

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    Clinical characteristics and genetic analysis of SETBP1 haploinsufficiency disorder
    WAN Ruiping, HUANG Xiaofei, YE Xingguang, WU Yanling, DAI Jiemin, LIU Zhigang
    Journal of Clinical Pediatrics    2023, 41 (6): 450-454.   DOI: 10.12372/jcp.2023.22e1155
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    Objective To summarize the clinical phenotype and genetic variation characteristics of patients with SETBP1 haploinsufficiency disorder (SETBP1-HD). Methods The genetic variations and clinical characteristics of three children with SETBP1-HD from January 2019 to December 2021 were retrospectively analyzed. Results Three SETBP1-HD children, 1 boy and 2 girls (identical twins), were 5 years old and 5 months old respectively. All patients presented moderate intellectual disabilities, motor and language developmental retardation. Compared with the verbal expression ability, the language comprehension ability of case 1 was better, and the child also had attention deficits. Case 2 and case 3 had febrile seizures. No obvious autism-like manifestations were observed in the 3 cases, and no specific manifestations were observed on head MRI scan and video electroencephalogram. De novo heterozygous variations in SETBP1 gene were found in all patients, including a frameshift variation (c.607delG/p.Gly203Valfs*4) from case 1 and a nonsense variation (c.1873C>T/p.Arg625*) from case 2 and case 3. Conclusions SETBP1-HD is a different phenotype from Schinzel-Giedion syndrome. The main clinical features of SETBP1-HD are intellectual disabilities, speech and language disorder, and motor developmental retardation. Speech and language disorders are the core symptoms of the disease. Some patients may have behavioral problems and convulsive seizures. Loss of function variation in SETBP1 resulting in haploinsufficiency is the cause of this disease. Patients can benefit partially from supportive treatment such as rehabilitation training, behavioral therapy, and anticonvulsant medication. Prenatal diagnosis is an important measure to prevent this disease.

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    Effect of SARS-CoV-2 infection on pulmonary function in children with asthma
    WANG Jinrong, MIAO Yu, MA Guangzeng, CAO Luofei
    Journal of Clinical Pediatrics    2023, 41 (5): 333-338.   DOI: 10.12372/jcp.2022.23e0163
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    The pandemic of COVID-19 raises new challenges in the management of pediatric asthma. We analyzed airway injury in children with asthma from molecular mechanism to pathological alterations and explored the effect of SARS-CoV-2 on pulmonary function, which will inform clinicians in the diagnosis and management of childhood asthma in the context of the new coronavirus epidemic.

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    Nutritional management in acute illness of children with inborn errors of metabolism
    QIU Wenjuan, DU Taozi, XIA Yu
    Journal of Clinical Pediatrics    2023, 41 (6): 401-405.   DOI: 10.12372/jcp.2023.23e0366
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    Inborn errors of metabolism (IEMs) are a group of monogenic diseases caused by defects in enzyme, receptor, cofactor or transporter during biochemical metabolism of amino acids, carbohydrates, organic acids, fatty acids and mitochondrial energy metabolism. The metabolic emergencies due to disorders in IEM leads to high mortality and disability rates in children. Improving nutrition management in acute illness is of great significance to the prognosis of IEM. Based on the latest guidelines and expert consensus of IEM at home and abroad, combined with clinical practice, we introduced the principles and protocols of nutrition management in the acute illness of IEM, which aims to improve the nutrition management and long-term prognosis of IEM.

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    Clinical analysis of fulminant myocarditis in 12 children
    SUN Juan, LI Haiying, JIA Peisheng, WANG Huaili
    Journal of Clinical Pediatrics    2023, 41 (9): 692-696.   DOI: 10.12372/jcp.2023.23e0168
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    Objective To analyze the clinical characteristics of fulminant myocarditis (FM) in children, and to provide references for the clinical decision-making of pediatricians. Methods The clinical data of children with FM who were hospitalized in the pediatric intensive care unit (PICU) from October 2019 to October 2022 were retrospectively analyzed. Results Twelve children with FM (5 boys and 7 girls) were included. The median age was 10.0 (5.6-12.6) years, the median time from onset of disease to treatment was 5.0 (3.0-6.0) days, and the median length of hospital stay was 12.0 (11.0-16.8) days. Twelve patients had no specific symptoms, 11 had circulatory symptoms, and 1 had gastrointestinal symptoms only. The onset symptoms were as follows: 8 children had digestive symptoms, 5 children had neurological symptoms, 2 children had both digestive and neurological symptoms, and 1 child had respiratory symptoms. The levels of myocardial troponinⅠ(cTnⅠ), brain natriuretic peptide (BNP) and lactate dehydrogenase (LDH) were elevated in all children. The median cTnⅠ level was 5.5 (1.4-12.6) ng/L, the median BNP level was 11630.0 (6440.0-28152.0) pg/mL, and the median LDH level was 642.0 (465.5-1194.3) U/L. The myocardial troponin T (cTnT) level was elevated in 11 patients (1 child undetected), and the median cTnT level was 1.2 (0.3-3.9) ng/L. The levels of serum creatine kinase (CK) and creatine kinase isoenzyme (CK-MB) were elevated in 9 patients, the median CK level was 348.5 (99.3-674.8) U/L, and the median CK-MB level was 35.4 (24.5-97.2) U/L. The D-dimer levels were elevated in 10 patients, and the median D-dimer level was 1.7 (0.6-3.3) mg/L. Electrocardiogram and echocardiography were abnormal in all patients after admission. Three patients were treated with extracorporeal membrane oxygenation (ECMO). Ten patients (83.3%) were discharged after improvement, and 2 died. Conclusions The onset symptoms of FM in children are atypical. The positive rates of myocardial enzyme, electrocardiogram and echocardiography were high. ECMO is an effective method to treat FM.

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    Research progress on early screening and diagnosis of Crohn's disease in children
    Reviewer: WANG Chenhui, Reviser: YANG Hui
    Journal of Clinical Pediatrics    2023, 41 (9): 708-714.   DOI: 10.12372/jcp.2023.22e0604
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    Crohn's disease (CD) is a chronic nonspecific gastrointestinal inflammatory disease. The onset of CD in children is insidious, the clinical manifestations are lack of specificity, and early diagnosis is difficult, which leads to the increased complications, surgical rates and disability rates. Early screening and diagnosis, timely intervention can improve the therapeutic effect and prognosis of children, and improve the life quality of children. This article reviews the progress of early screening and diagnosis of CD in children.

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    Advances in studies on risk factors and predictors of patent hemodynamically significant ductus arteriosus in premature infants
    ZHAO Caiyan, SUN Xuan, CHEN Ling
    Journal of Clinical Pediatrics    2023, 41 (6): 475-479.   DOI: 10.12372/jcp.2023.22e0327
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    Objective Patent ductus arteriosus (PDA) is a common complication in premature infants. When it progresses to PDA with significant hemodynamics (hsPDA), it can seriously affect neonatal outcomes. Recent studies have found that some perinatal factors, platelet-related parameters, brain natriuretic peptide and ultrasound indicators can predict the occurrence of hsPDA and form an artificial intelligence prediction model. However, some predictors are still controversial, and further research is needed to build a sensitive, accurate and easy-to-use prediction model for targeted treatment of high-risk children to improve the prognosis of premature babies.

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    Clinical and genetic analysis of Wiedemann-Steiner syndrome caused by KMT2A gene mutation in three cases
    LIU Suying, LI Fang, MA Hongwei
    Journal of Clinical Pediatrics    2023, 41 (8): 618-623.   DOI: 10.12372/jcp.2023.22e0472
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    Objective To report the clinical and genetic characteristics of three cases of Wiedemann-Steiner syndrome (WDSTS) caused by KMT2A gene mutation, and improve the understanding of the disease and the efficiency of diagnosis and treatment. Methods The clinical data of three cases of WDSTS caused by KMT2A gene mutation in three families diagnosed in Shengjing Hospital of China Medical University from December 2019 to September 2021 were reviewed, and the clinical and genetic characteristics of WDSTS patients reported in the literature were reviewed and summarized. Results All three cases were diagnosed in infancy, with the youngest age of diagnosis of two months old, and the reasons for consultation were growth retardation and anorexia, respectively. Similar to those in previous literature, the clinical manifestations were special facial features, malnutrition (3/3), feeding difficulties, sleep disorders, hirsutism (2/3), and developmental delay (1/3). The clinical manifestations that had not been reported before were umbilical hernia and inguinal hernia. All three cases were de novo frameshift variants, which occurred in exon 3 and 27 in hot spot variants region and exon 11 in non-hot spot variants region, respectively. Conclusions Wiedemann-Steiner syndrome should be considered in children with malnutrition, feeding difficulty and developmental delay, combined with special facial features and specific hirsute. High-throughput exome sequencing should be used to facilitate early diagnosis. De novo frameshift variants of KMT2A gene are common, with hot spots in exon 27 and 3. The three unreported frameshift variants in this study enrich the mutation spectrum of this gene. In addition, patients with variants in the CXXC region of the KMT2A gene may have a more severe clinical phenotype.

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    Clinical analysis of 195 children with invasive pulmonary fungal infection
    XU Beixue, LIU Quanbo
    Journal of Clinical Pediatrics    2023, 41 (8): 584-588.   DOI: 10.12372/jcp.2023.22e0925
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    Objective To analyze the clinical data of 195 children with invasive pulmonary fungal infection (IPFI), and to provide help for early identification and diagnosis of IPFI in children. Methods The clinical data of 195 children with IPFI were retrospectively analysed. Results Among the 195 cases, 123 were male and 72 were female, the median age was 9.5 (0.9-62.0) months. 52 were neonates (<28 d), 79 were infants (28 d-3 years), and 64 were elder children (3-18 years). The most common diseases of newborn were premature infants (75.0%), the most common diseases of infant were immunodeficiency (20.3%), and haematological diseases were more common in elder children (31.3%). The differences in the proportions of two-combination antibiotic use, haemodialysis or dialysis, indwelling gastric tube, use of glucocorticoids, chemotherapy or other immunosuppressive treatments, and parenteral nutrition were statistically significant between different age groups (P<0.05). The main pathogen was Candida albicans (69.2%), and there were significant differences in the proportion of Candida albicans, Candida tropicalis and Aspergillus among different age groups (P<0.05). Candida had a high resistance to azoles and a low resistance to amphotericin B and 5-fluorocytosine. Conclusions Invasive pulmonary fungal infections in children were predominant in infants and young children, with differences in the underlying disease and invasiveness factors in different age groups, with Candida being the predominant pathogen, and the distribution of strains of the organisms may be related to age, with a high rate of resistance to azoles inCandida.

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    Clinical and genetic analysis of early onset very long chain acyl-CoA dehydrogenase deficiency: a report of three cases
    LI Yanjun, ZHANG Yonghong, CHEN Yan, QIU Wenjun, HAN Lianshu, ZHU Tianwen
    Journal of Clinical Pediatrics    2023, 41 (5): 381-386.   DOI: 10.12372/jcp.2023.22e0453
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    Objective To explore the clinical features and genetic characteristics for early onset very long chain acyl-coenzyme A dehydrogenase deficiency (VLCADD) and to improve the understanding of this disease. Methods The genotypes, clinical phenotypes and prognosis of 3 neonates with early-onset VLCADD diagnosed and treated in the neonatal intensive care unit of Xinhua Hospital, Shanghai Jiao Tong University School of Medicine from September 2017 to April 2020 were retrospectively analyzed. Results Three children (2 boys and 1 girl) with early onset VLCADD were diagnosed by molecular method, and none of them had a positive family history. All three patients presented with metabolic crisis or digestive tract symptoms during the neonatal period. Two children were found to have increased C14:1 during the neonatal screening, and 1 case was not screened. All three children had ACADVL gene variations, which were complex heterozygotes, and the variations were from parents, among which c.1615C > T and c.231-232insAATG were not reported. One patient was breastfed after birth and had respiratory and cardiac arrest at 2 days of age. The other two children were fed special milk powder rich in medium-chain triacylglycerol immediately after the diagnosis due to abnormalities found in the neonatal screening, and sudden death occurred at 3 and 4 months of age, respectively. Conclusions Early onset VLCADD is one of the potential sudden death diseases in neonatal period and infancy. The prognosis is still poor despite early diagnosis and treatment. Therefore, prenatal diagnosis of fetuses from families with previous birth of VLCADD is an effective way to avoid the birth of early onset VLCADD children.

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    Report a case of α-ketoadipic aciduria caused by compound heterozygous variant of DHTKD1 gene
    WANG Hongxia, PAN Xiang, LU Jun
    Journal of Clinical Pediatrics    2023, 41 (8): 624-628.   DOI: 10.12372/jcp.2023.22e0486
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    Alpha-ketoadipic aciduria is a genetic metabolic disease caused by the disorder of lysine, hydroxylysine and tryptophan degradation metabolism. The clinical manifestations of the disease vary in severity and affect a wide range of systems. The main clinical manifestations include growth retardation, hypotonia, epilepsy, ataxia, microcephaly and abnormal behavior. The patient was 2 years and 8 months old and presented with convulsions and behavioral abnor malities. Urine organic acid analysis showed that α-ketoadipic acid was significantly increased. Genetic testing revealed that DHTKD1 gene had pathogenic compound heterozygous variation. Combined with the clinical and genetic characteristics of the child, the diagnosis of α-ketoadipic aciduria was confirmed. The patient's condition improved after symptomatic treatment with a low lysine and protein diet and rehabilitation. This is the first report of α-ketoadipic aciduria in Chinese medical database, which expands the genetic spectrum of α-ketoadipic aciduria and provides reference for clinical diagnosis and treatment of the disease.

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    Characteristics of 900 hospitalized children of asthma attack
    LIU Qiuyi, ZHANG Peiling, GUO Rong, YAN Li, GENG Gang, LIU Zheng, TIAN Daiyin, DAI Jihong, LUO Zhengxiu, FU Zhou, NIU Chao
    Journal of Clinical Pediatrics    2023, 41 (5): 353-359.   DOI: 10.12372/jcp.2023.22e0955
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    Objective To analyze the characteristics of hospitalized children with asthma exacerbation, and provide clinical reference for the diagnose and treatment of asthma. Methods Clinical data of children with bronchial asthma admitted to the Respiratory Center of Children's Hospital of Chongqing Medical University from January 2011 to June 2021 were retrospectively analyzed. Results 900 children were enrolled in the study, including 615 boys and 285 girls, with a median age of 2.3 (1.3-3.9) years. In the study, 559 toddlers, 246 preschool children, and 92 school-age children were enrolled, and significant differences were found in the proportions of boys and in the time distribution of initial diagnosis as asthma among the three age groups (P<0.05). The proportion of children first diagnosed with asthma after admission was 63.5%. The percentage of children admitted for mild acute asthma attacks was 76.6%, with more infants (475 cases, 52.8%) followed by preschool children (180 cases, 20.0%). Among 900 children, 501 (55.7%) had pathogenic bacteria detected by sputum culture, with the highest proportion of Streptococcus pneumoniae (26.9%), followed by Moraxella catarrhalis and Haemophilus influenzae. The positive proportion of four viruses detected in the study, including respiratory syncytial virus, adenovirus, influenza virus, parainfluenza virus, was 25.0%, and of which respiratory syncytial virus had the highest detection rate (16.3%). The difference in the proportions of asthma children combined with allergic rhinitis or sinusitis among the three age groups was statistically significant (P < 0.01). A total of 224 children were tested for allergen sIgE, of which 119 children (53.1%) were positive for inhalant allergens and 76 children (33.9%) were positive for foodborne allergens. Dust mite (27.7%) was the most important allergens, followed by house dust (8.9%). There were significant differences in the positive rates of most inhalant allergens (e.g., dust mite, house dust, cat and dog hair, tree allergens, mulberry, etc.) between the three age groups (P < 0.05). Conclusions Mild acute asthma attacks was the most important cause for hospitalization in children under 6 years old with asthma being firstly diagnosed after admission. Respiratory tract infection was the most important trigger of acute exacerbation of asthma. For children with asthma, allergen screening and allergic rhinitis prevention and treatment should be emphasized.

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    Advances in the pathogenesis of very early onset inflammatory bowel disease
    ZHUANG Yan, HUANG Ruiwen
    Journal of Clinical Pediatrics    2023, 41 (7): 549-555.   DOI: 10.12372/jcp.2023.22e0397
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    Very early onset inflammatory bowel disease (VEO-IBD) refers to those patients who have developed disease before the age of 6, and their incidence and prevalence have increased rapidly in recent years. With the development of gene sequencing technologies and platforms, the pathogenesis of VEO-IBD has been found to be related to the monogenic variations involved in multiple pathways of immunity. Whether the monogenic variation is the main cause of VEO-IBD is still controversial, but identifying the specific variant type can guide specific treatment to some extent. This paper introduces the possible mechanisms of gene variation and environmental exposure in the pathogenesis of VEO-IBD, focusing on four aspects of monogenic variation associated with VEO-IBD, in order to provide directions for early diagnosis and precise treatment.

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    Research progress on characterization and therapeutics for recurrent medulloblastoma in children
    ZHANG Zaiyu, WU Yuxin, LIANG Ping
    Journal of Clinical Pediatrics    2023, 41 (10): 708-714.   DOI: 10.12372/jcp.2023.22e1112
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    Medulloblastoma (MB) is a common intracranial malignancy in children with a high recurrence rate despite standardized multimodal initial treatment. The prognosis of recurrent children is very poor even after intensive treatment. In recent years, the in-depth understanding of the clinical and biological characteristics of MB recurrence can guide the clinical design of relevant treatment strategies and implement of clinical trials. This paper reviews the research progress of the above contents, hoping to promote the speed of its clinical transformation and application, and finally benefit the clinical practice.

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    Clinical and genetic characteristics of Kallmann syndrome
    TANG Yijun, ZHANG Qianwen, WANG Yirou, CHEN Yao, LI Xin, LI Juan, WANG Jian, WANG Xiumin
    Journal of Clinical Pediatrics    2023, 41 (7): 537-542.   DOI: 10.12372/jcp.2023.22e1053
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    Objective To analyze the clinical and genotypic characteristics of children with Kallmann syndrome (KS), and to strengthen clinicians' understanding of the disease. Methods The clinical characteristics, whole-exome gene sequencing results, and treatment outcomes of 20 KS patients were retrospectively analyzed. Results Among the 20 patients, 17 were boys and 3 were girls. The mean age at diagnosis was 13.13 years old. Male children presented micropenis with small testicular size and history of bilateral cryptorchidism, while female patients presented primary amenorrhea and retardation of breast development. The results of Chinese odor recognition test indicated that 93.75% (15/16) children had olfactory disorder. Children with KS may have other systemic manifestations, including neurodevelopmental delay, mental retardation, phalangeal abnormalities, hearing impairment, and congenital cleft lip and palate. Among the 19 patients who underwent whole-exome gene sequencing, 12 variation sites in 6 genes were found, including FGFR1, CHD7, SOX10, KAL1, PROKR2 and SOX2. The molecular diagnosis rate was 63.16% (12/19). Testicular size, penile length, penile girth, T, LH and FSH were significantly improved after receiving pulsatile GnRH pump treatment, and there were no obvious adverse reactions. Conclusions The application of Chinese odor recognition tests can more accurately evaluate olfactory function in children with KS. The whole-exome gene sequencing technology is helpful to improve the molecular diagnosis of the disease, so as to diagnose the children with this rare disease as early as possible. Pulsatile GnRH pump therapy promotes sexual development in children with KS without obvious adverse effects.

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    Long-term management of severe coronary artery lesions in Kawasaki disease
    LIU Fang, LIN Yixiang
    Journal of Clinical Pediatrics    2023, 41 (7): 492-497.   DOI: 10.12372/jcp.2022.23e0331
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    Kawasaki disease coronary artery lesions (CAL) has become one of the most common acquired cardiovascular diseases in China. The treatment and long-term management of severe CAL is very challenging. This article summarizes and prospects the management of severe CAL by focusing on diagnosis and evaluation methods, treatment progress and follow-up programs.

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    Risk factors analysis of coronary artery aneurysm occurrence in children with Kawasaki disease
    ZHOU Cuizhen, SONG Sirui, CHEN Liqin, HUANG Min
    Journal of Clinical Pediatrics    2023, 41 (7): 498-501.   DOI: 10.12372/jcp.2023.23e0285
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    Objective To investigate the risk factors of coronary artery aneurysm (CAA) occurrence in children with Kawasaki disease (KD). Methods The clinical data of children with KD hospitalized from January 2020 to December 2021 were retrospectively analyzed. According to the presence or absence of CAA, the children were divided into CAA group and non-CAA group, and the clinical characteristics between the two groups were compared. The risk factors affecting the occurrence of CAA and the predictive value of related indicators for KD complicated with CAA were analyzed. Results A total of 557 children with KD (346 boys and 211 girls) were included, and the median age was 25.0 (13.0-45.2) months. CAA was found in 75 patients (13.5%) and intravenous immunoglobulin (IVIG) resistance was observed in 39 (7.0%) patients. Multivariate logistic regression analysis showed that age ≤1 year, IVIG resistance and elevated platelet count (PLT) were independent risk factors for KD complicated with CAA, and elevated albumin (ALB) was a protective factor for KD complicated with CAA (P<0.05). ROC curve analysis showed that the area under ROC curve (AUC) of PLT and ALB predicting KD with CAA was 0.64 (0.57-0.73) and 0.65 (0.58-0.72), respectively. The sensitivity and specificity of PLT>551×109/L for predicting KD with CAA were 63.0% and 67.0%, and the sensitivity and specificity of ALB<36.5U/L for predicting KD with CAA were 68.0% and 56.0%. Conclusions Age≤1 year, IVIG resistance, PLT>551×109/L and ALB<36.5U/L had certain predictive effects on the occurrence of CAA in children with Kawasaki disease.

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    Clinical features, treatments, and outcomes of neonatal arrhythmia
    CHEN Wenwen, DAI Shuzhen, XU Liping
    Journal of Clinical Pediatrics    2023, 41 (7): 507-513.   DOI: 10.12372/jcp.2023.22e0320
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    Objective To evaluate the etiology, treatment and outcome of neonatal arrhythmia. Methods The clinical data and short-term follow-up results of neonates diagnosed with arrhythmia from June 2017 to May 2020 were retrospectively analyzed. Results Twenty-three cases with neonatal arrhythmia (11 boys and 12 girls) were included. The mean gestational age was (36.2±2.9) weeks and the mean birth weight was (2784.8±727.7) grams. There were 12 full-term and 11 preterm infants. Abnormal fetal heart rhythm of 8 cases were observed in the prenatal period. There were 9 cases of ectopic beats, 12 cases of tachycardia and 2 cases of conduction block. The main causes of arrhythmia were cardiac structural abnormality, neonatal respiratory distress syndrome and asphyxia at birth. Fourteen cases did not have clear etiology or clinical symptoms. Twelve cases with tachycardia were treated with anti-arrhythmia drugs or electrical cardioversion, while those without clinical symptoms (6 cases of supraventricular premature beats, 3 cases of ventricular premature beats and 1 case of atrioventricular block) did not receive anti-arrhythmia therapy. Four neonates died (17.4%) eventually. Serum levels of creatine kinase, creatine kinase-MB isoenzyme, cardiac troponin T and B-type natriuretic peptide in death group were significantly higher than those in survival group, and the difference was statistically significant (P<0.05). Of the 19 infants who survived until discharge, 11 showed abnormal rhythm on dynamic electrocardiogram, and 2 were treated with oral anti-arrhythmia drug after discharge. By 1 year of age, none of the patients had been readmitted to the hospital for symptomatic arrhythmia. Conclusions Arrhythmia may occur in newborns with underlying diseases, or without clear cause or clinical symptoms. The prognosis of patients with elevated myocardial markers is poor. Rapid arrhythmia should be considered as soon as possible. For those without clear cause or symptoms, those with good heart function, no intervention should be involved, but need to be closely followed.

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    Role of vascular smooth muscle cell phenotypic switching in cardiovascular diseases in children
    XU Xiao, ZHAO Lin, GONG Fangqi
    Journal of Clinical Pediatrics    2023, 41 (5): 394-400.   DOI: 10.12372/jcp.2023.22e0555
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    Vascular smooth muscle cells (VSMCs) are predominant constituents of the vessel walls and contribute to maintaining vascular function of the blood vessels. VSMC phenotypic switching from a quiescent contractile phenotype to a proliferative and migratory phenotype induced by several pathological stimulations, which is involved in the pathogenesis of a serious cardiovascular disease (CVDs). In recent years, cardiovascular health in children has become an increasingly prominent problem, posing a heavy burdens on economic development. In this review, we summarized the research on VSMCs phenotypic switching in pediatric CVDs such as Kawasaki disease, aneurysm, primary hypertension, pulmonary hypertension, coarctation of the aorta, and Takayasu arteritis, which is aim to provide potential targets for the prevention and treatment of CVDs in children.

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    Summary of clinical characteristics of 23 children with buckwheat allergy
    DU Yuyang, WANG Xiaoyang, SHAO Mingjun, LIU Chuanhe
    Journal of Clinical Pediatrics    2023, 41 (5): 360-365.   DOI: 10.12372/jcp.2023.22e1639
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    Objective To analyze and summarize the clinical manifestation of buckwheat allergy in children in order to increase the awareness and diagnosis of buckwheat allergy in children. Methods Children with buckwheat allergy were enrolled from outpatients in the Department of Allergy, Children’s Hospital of Capital Institute of Pediatrics during July 2019 through April 2022. Clinical data was collected, including skin prick test (SPT), serum allergen specific immunoglobin E(IgE) and peripheral eosinophil detection. Results A total of 23 cases (14 males and 9 females) were enrolled, with a median age of 7.8 (5.5-10) years. The children developed allergic symptoms after intake of buckwheat products, skin exposure of buckwheat or inhalation of buckwheat flour. Allergic symptoms occurred within 10 minutes in sixteen cases, within 10 minutes to 30 minutes in twenty-two cases, and within 30 minutes to 2 hours in six cases after exposure to buckwheat allergen. The most common clinical manifestations were skin symptom (21/23 cases, 91.3%), followed by respiratory symptom (15/23 cases, 65.2%), digestive symptom (5/23 cases, 21.7%) and neurological symptom (1/21 cases, 4.3%). 16 cases had severe anaphylaxis. 21 cases were tested positive in buckwheat SPT, and the risk of anaphylaxis was increased when the SPT level was “++++” (P<0.01). The detection level of serum total IgE in 23 cases was 120 to 1489 kU/L. All the cases had a history of allergic rhinitis, eczema and family allergies. 18 of them have diagnosed with bronchial asthma. 22 of them had a history of using buckwheat husk pillows. Conclusions Buckwheat allergy in children has lately onset in school-aged children and adolescent, with varied clinical manifestations and high rate of anaphylaxis. Buckwheat husk pillows may be an important way for buckwheat sensitization. Skin prick test is of great value in the diagnosis and evaluation of buckwheat allergy.

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    Diagnosis and treatment philosophy and practice of macrolide-resistant Mycoplasma pneumoniae pneumonia in children
    YIN Yong, CHEN Jiande
    Journal of Clinical Pediatrics    2024, 42 (3): 177-181.   DOI: 10.12372/jcp.2024.24e0003
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    Since July 2023, China and even the world have experienced a peak outbreak of Mycoplasma pneumoniae pneumonia, which has brought a heavy burden to children's health, families, and society. Although macrolide antibiotics are the first choice recommended by the guidelines, the majority of hospitalized children with Mycoplasma pneumoniae pneumonia in this epidemic are resistant to macrolides. This has brought great challenges to the traditional diagnosis and treatment model, especially the increasing number of pulmonary embolism cases, which requires clinicians to think about and practice new diagnosis and treatment concepts.

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    Clinical characteristics of bacterial meningitis/meningoencephalitis in children aged 5 years and over: an analysis of 39 cases
    ZHANG Jianzhao, SUN Jing, LI Guannan, ZHENG Ping, LI Dongqing, LIU Zhao, MA Caihui, YANG Jian
    Journal of Clinical Pediatrics    2023, 41 (5): 376-380.   DOI: 10.12372/jcp.2023.22e1493
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    Objective To analyze the clinical characteristics of bacterial meningitis/ meningoencephalitis in children aged 5 years and above. Methods The clinical data of hospitalized children with bacterial meningitis/meningoencephalitis in the department of neurology in the hospital were retrospectively analyzed. Results A total of 39 (7.8%) children, 28 males and 11 females, aged 5-15 years with bacterial meningitis/meningoencephalitis were enrolled. The etiology included: nasal infection in 11 cases (28.2%), ear infection in 5 cases (12.8%), trauma in 3 cases (7.7%), immunodeficiency disease in 3 cases (7.7%), facial cellulitis in 2 cases (5.1%), anatomical abnormalities (dermoid cysts) in 1 case (2.7%), central nervous system tumors (lymphoma) in 1 case (2.7%), and unknown causes in 13 cases (33.3%). Symptoms include fever, headache, vomiting, disturbance of consciousness, convulsions, paralysis, facial paralysis and convulsive persistence. Pathogens were found in cerebrospinal fluid of 15 children (38.5%), including Neisseria gonorrhoeae, Streptococcus pneumoniae, Pseudomonas aeruginosa, Ralstonia pipiensis, Micrococcus kristinae, Rosemonas, Staphylococcus aureus, Streptococcus intermedius, Streptococcus constellatus and Listeria monocytogenes. Among them, pathogens of 10 cases were found by second generation sequencing, and 6 cases were found by cerebrospinal fluid culture. There were 17 cases (43.6%) with abnormal magnetic resonance imaging of the skull. Complications or sequelae included: subdural effusion in 35 cases (89.7%), subdural pus in 6 cases (15.4%), cerebral hernia in 6 cases (15.4%), hydrocephalus in 3 cases (7.7%), speech impairment in 2 cases (5.1%), and death in 1 case (2.7%). Conclusions The etiology of bacterial meningitis/meningoencephalitis in children aged 5 years or older should be actively sought, such as intracranial endothelioid cysts, primary tumors of the central nervous system, cellulitis of the head and face, sinusitis, immunodeficiency diseases, and trauma.

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    Advances in the study of bedaquiline and delamanid in the treatment of drug-resistant tuberculosis in children
    WANG Xiyu, XU Hongmei
    Journal of Clinical Pediatrics    2023, 41 (5): 387-393.   DOI: 10.12372/jcp.2023.22e1064
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    Tuberculosis (TB) is one of the major global public health concerns, and drug-resistant TB is one of the major obstacles to achieve the goal of ending TB due to the long treatment period, complicated regimens, multiple adverse effects and poor efficacy. In recent years, bedaquiline and delamanid have gradually become promising new drugs, but there is lack of researches on the application of these drugs in children with drug-resistant tuberculosis in China. This article reviews the efficacy and safety of bedaquiline and delamanid in drug-resistant tuberculosis and the progress of their use in children, in order to help construct new and effective regimens for the treatment of drug-resistant tuberculosis in children.

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    Clinical manifestations and immune mechanism of invasive pulmonary mycosis in children
    QIN Tao, XU Hongmei
    Journal of Clinical Pediatrics    2023, 41 (8): 566-570.   DOI: 10.12372/jcp.2023.23e0435
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    Invasive pulmonary mycosis is a fungal infection of the bronchi and lungs, causing inflammation of the airway mucosa and pulmonary inflammatory granulomas. In severe cases, necrotizing pneumonia may occur, and even hematogenous dissemination to other organs. Its occurrence and development depend on the interaction between external fungal pathogenic factors and the patient's immune function. Children with primary immune dysfunction, hematological malignancies, receiving hematopoietic stem cell transplantation or high-intensity immunosuppressive therapy, long-term invasive catheterization, or severe infections may be susceptible to fungi. This article discusses the immunological pathogenesis and clinical characteristics of invasive pulmonary mycosis in children, hoping to provide theoretical basis for the early detection of the disease.

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    Predictive role of IL-17A in refractory Mycoplasma pneumoniae pneumonia in children
    GAO Longfei, ZHANG Jingli, WU Xiaojie, WU Huifang, DUAN Chenchu, KANG Juncong, ZHANG Zhongping
    Journal of Clinical Pediatrics    2023, 41 (5): 366-369.   DOI: 10.12372/jcp.2023.22e0032
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    Objective To explore the predictive value of blood interleukin (IL)-17A in children with refractory Mycoplasma pneumoniae pneumonia (RMPP). Methods Clinical data of children with Mycoplasma pneumoniae pneumonia (MPP) admitted from January 2019 to October 2021 were retrospectively analyzed. The enrolled children were divided into the MPP and the RMPP group. The differences of infection-related indexes between the two groups were compared, and the value of these indicators in predicting RMPP was evaluated by ROC curves. Results A total of 63 children with MPP were included in the study, including 42 cases in MPP group and 21 cases in RMPP group. There were 43 males and 20 females with a median age of 4.9(1.8-12.0) years and no significant differences was found in gender and age between the two groups (P>0.05). The proportion of lobar consolidation, pleural effusion, moxifloxacin, glucocorticoid and bronchoscopy in RMPP group were higher than those in MPP group (P<0.05). Also, IL-17A, IL-18, LDH and ferritin levels in RMPP group were higher (P<0.05). The area under ROC curve (AUC) of IL-18 and IL-17A in predicting RMPP were 0.85, 0.81, 0.80 and 0.69 in the RMPP group, respectively, with corresponding cut-off values of 472.0 pg/mL, 13.4 pg/mL,389.0 UI/mL, and 251.0 pg/mL, respectively. Conclusions Serum IL-17A≥13.4 pg/mL and IL-18≥472 pg/mL were relatively accurate predictors of refractory mycoplasma pneumonia in children, and IL-18 has higher predictive efficacy.

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    Clinical application of non-invasive high-frequency oscillatory ventilation in premature infants
    ZHU Xingwang, SHI Yuan
    Journal of Clinical Pediatrics    2023, 41 (10): 641-645.   DOI: 10.12372/jcp.2023.23e0789
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    Noninvasive high-frequency oscillatory ventilation(NHFOV) is based on the application of a continuous flow, generating a constant distending positive pressure with superimposed oscillations, delivered all over the spontaneous breathing cycle. NHFOV matches together the advantages of high-frequency ventilation (no need for synchronisation, high efficacy in removing CO2) and nasal continuous positive airway pressure (NCPAP, non-invasive interface, increase in functional residual capacity allowing oxygenation to improve). As it represents another “brick in the wall” of the non-invasive respiratory support, it has been increasing applied in neonatal intensive care unit (NICU). At present, there is no consensus guidelines on the application of NHFOV, the application of NHFOV is largely based on the local practices and comfort. This article summarizes the physiology of NHFOV, indications, ventilator device, interfaces, parameter settings, and clinical evidence based on a PubMed literature search.

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    Prospect of gene therapy for developmental and epileptic encephalopathy
    JI Taoyun
    Journal of Clinical Pediatrics    2023, 41 (9): 650-655.   DOI: 10.12372/jcp.2023.23e0540
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    Developmental and epileptic encephalopathy (DEE) is a group of heterogeneous disorders characterized by early-onset epilepsy, abnormal electroencephalography and developmental retardation or regression. The etiology of DEE is complex, with high disability rate and fatality rate. With the development of next-generation sequencing technology, more and more genetic causes related to DEE have been discovered, which also deepens the acknowledgement on the pathogenesis of DEE. These researches provide a basis for exploring different treatment methods, especially gene therapy. It is expected that gene therapy will be carried out in the future to improve the prognosis of DEE.

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    Myelin oligodendrocyte glycoprotein antibody-associated disease in children with prominent manifestation of optic neuritis caused by Mycoplasma pneumoniae infection: a case report
    GUO Fang, KANG Lei, DU Feifan, JIA Yanhong, XU Meixian
    Journal of Clinical Pediatrics    2023, 41 (10): 703-707.   DOI: 10.12372/jcp.2023.22e0638
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    Objective To investigate the clinical manifestations, treatment and prognosis of pediatric Mycoplasma pneumoniae (MP)-associated optic neuritis (ON). Methods The clinical data of 1 child with MP-ON were retrospectively analyzed, and the cases of MP-ON in domestic and foreign databases were retrieved and analyzed. Results The patient was a 10-year-old prepubertal girl, and her onset symptoms were fever and headache. Her serum MP antibody titer was >1:1280. After intravenous infusion of azithromycin, the child's fever and headache were quickly relieved. On day 23 of the disease course, the patient developed acute vision loss with pain of eye rotation, visual field defects, papilledema, and abnormal visual evoked potentials. There were no other manifestations of nervous system involvement and no manifestations of encephalopathy. Laboratory test found positive serum myelin oligodendrocyte glycoprotein-antibody (MOG-Ab). Cerebrospinal fluid and MRI results were abnormal. After intravenous infusion of high dose methylprednisolone combined with human immunoglobulin, the vision of the child returned to the pre-onset level, and no recurrence was observed 3 months later. Conclusions MP infection can be complicated by ON, which is related to demyelinating changes caused by autoimmunity or immune complex. Intractable headache has suggestive significance. Serum antibodies detection of central nervous system demyelinating disease is beneficial to clinical classification and guidance of treatment.

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    Clinical analysis of 15 children with primary nephrotic syndrome complicated with adrenal crisis
    SUN Zhicai, LIU Yuling, LI Xiaolin, PAN Xiaofen
    Journal of Clinical Pediatrics    2023, 41 (8): 610-612.   DOI: 10.12372/jcp.2023.22e0900
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    Objective To investigate the clinical characteristics and prognosis of primary nephrotic syndrome (PNS) complicated with adrenal crisis (AC) in children. Methods Clinical data of 15 patients with primary nephrotic syndrome complicated with adrenal crisis in the department of Pediatrics, Boai Hospital, Zhongshan City from January 2010 to March 2022 were retrospectively analyzed. Results The average age of the patients was 6.25±1.81 years old (4.75-12 years old), including 9 males and 6 females. The course of disease was 2.74±1.33 years (1-6 years). Inducement: Glucocorticoid dose reduction or withdrawal in 8 cases, respiratory tract infections in 5 cases, and no obvious triggers in 2 cases. Clinical manifestations: Gastrointestinal symptoms such as abdominal pain, nausea, and vomiting were found in 11 cases, circulatory failure manifestations such as low blood pressure and oliguria in 7 cases, hyponatremia in 8 cases, hyperkalemia in 4 cases, and hypoglycaemia in 2 cases, All the children were accompanied by neurological symptoms of varying degrees (such as dizziness, debilitation, malaise or irritability, etc.). Serum cortisol: Random serum cortisol level of 9 children with digestive tract or early shock symptoms was 37.6±15.7 nmol/ L (15.3-52.7 nmol/ L), fasting serum cortisol level was 56.3±18.9 nmol/ L (21.6-73.4 nmol/L) at 8 am in 6 patients. Treatment and prognosis: Symptoms were effectively controlled in all children after 2-3 days of intravenous hydrocortisone treatment. After follow-up to June 2022, 11 cases of PNS were cured, 4 cases of PNS were still under treatment, and there was no recurrence of AC. Conclusion Primary nephrotic syndrome in children with adrenal crisis is mainly caused by hormone abatement or infection, and the symptoms of digestive and circulatory system are the main manifestations. Early identification and timely treatment have a good prognosis.

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    The clinical treatment characteristics of fulminant type 1 diabetes mellitus
    LI Jiru, MA Zhushengying, QIAN Wen, QIU Huinan, XU Lili, ZHU Xiaodong
    Journal of Clinical Pediatrics    2023, 41 (6): 417-423.   DOI: 10.12372/jcp.2023.22e1428
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    Objective To investigate the clinical diagnosis and treatment of fulminant type 1 diabetes mellitus (FT1DM) in pediatrcis. Methods The clinical data of children with newly diagnosed diabetes mellitus complicated with ketoacidosis (DKA) admitted to the pediatric intensive care unit from January 2015 to June 2022 were retrospectively analyzed. The subjects were divided into FT1DM group and typical type 1 diabetes mellitus (TT1DM) combined with DKA group, and the clinical characteristics and treatment of the two groups were compared. Results A total of 90 children (42 boys and 48 girls) with initial T1DM complicated with DKA were included, and the median age was 87.0 (39.0-125.0) months. There were 85 children in TT1DM combined with DKA group and 5 in FT1DM group. None of the enrolled children died or had acute pancreatitis. The onset time of children in FT1DM group was significantly shorter than that of TT1DM combined with DKA group, and the difference was statistically significant (P<0.05). FT1DM group had more significant dehydration, influenza-like symptoms, chest tightness, fatigue and lethargy, and the proportion of tachycardia and tachypnea was significantly higher than that of TT1DM combined with DKA group, and the difference was statistically significant (P<0.05). Compared with TT1DM combined with DKA group, FT1DM group had lower levels of serum sodium, glycosylated hemoglobin (HbAlc), fasting C-peptide, sodium×HbAlc, and lower positive proportions of anti-glutamic acid decarboxylase antibody (GADA) and protein tyrosine phosphatase antibody (IA-2A); serum potassium, fasting blood glucose, creatinine, blood glucose/HbAlc and potassium/HbAlc were higher in FT1DM group, and the differences were statistically significant (P<0.05). Compared with TT1DM combined with DKA group, the fluid resuscitation dose and total therapeutic dose of insulin in FT1DM group were higher and the time to correct ketosis was longer at 24/48 hours after admission, and the difference was statistically significant (P<0.05). Conclusions FT1DM is rare in pediatric patients with newly diagnosed diabetes mellitus, has a sudden onset of disease, has a more severe disorder of glucose metabolism, and can affect multiple systems. Children with FT1DM require longer treatment periods, more exogenous insulin injections, and fluid for resuscitation and expansion.

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    Analysis of prognostic factors and survival status of group 4 medulloblastoma in children
    WU Yuefang, SUN Yanling, WU Wanshui, DU Shuxu, LI Miao, SUN Liming
    Journal of Clinical Pediatrics    2023, 41 (9): 686-691.   DOI: 10.12372/jcp.2023.22e1634
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    Objective To investigate the survival status and prognostic factors of group 4 medulloblastoma (MB) in children. Methods The clinical data of children with group 4 MB admitted to the Department of Pediatrics from May 2016 to August 2020 (follow-up until August 2022) were retrospectively analyzed. The Kaplan-Meier method was used to calculate the overall survival (OS) rate and progression-free survival (PFS) rate. Log-rank test was used to compare the difference in survival rate between groups, and Cox regression model was used to analyze the factors affecting prognosis. Results A total of 145 children (106 boys and 39 girls) with group 4 MB were included, and the median age of diagnosis was 7.5 (5.7-9.6) years old. There were 91 children in M0 stage and 54 children in M+ stage (1 in M1 stage, 12 in M2 stage and 41 in M3 stage). The pathologic types were classic in 127 cases, desmoplastic/nodular (DN) in 8 cases, anaplastic/large cell (LC/A) in 8 cases, extensive nodularity (EN) in 1 case, and none of somatotype (NOS) in 1 case. The median follow-up time was 47.9 (36.5-59.3) months, and 37 children had tumor recurrence. The 5-year OS and PFS rates were (80.8±3.4) % and (55.4±4.7) %, respectively. Cox regression analysis indicated that M+ stage, MYCN amplification and Chr12p+ variation were independent risk factors for prognosis (P<0.05). Conclusions Group 4 MB children with M+ stage or MYCN amplification have a relatively poor prognosis. Chr12p + may be related to the prognosis of children, but it needs to be confirmed by clinical studies with larger samples.

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    Journal of Clinical Pediatrics    2023, 41 (9): 703-707.   DOI: 10.12372/jcp.2023.22e1763
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    Relationship between blood lipids and age, coronary artery disease and its severity in the acute stage of Kawasaki disease
    HE Fangyuan, HE Xuehua, YUAN Yonghua, ZHU Liurong, WU Yi, XIA Xiaohui
    Journal of Clinical Pediatrics    2023, 41 (6): 455-458.   DOI: 10.12372/jcp.2023.22e1030
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    Objective To investigate the relationship between the changes of blood lipid and age, coronary artery disease and its severity in children with Kawasaki disease (KD). Methods A total of 241 children with acute KD admitted from January 2018 to December 2020 were selected as the study subjects (KD group), and 30 healthy children who underwent physical examination in our hospital were selected as the control group. The levels of serum total cholesterol (TC), triglyceride (TG), low density lipoprotein (LDL-C) and high density lipoprotein (HDL-C) were compared between the two groups. The differences in blood lipid levels among KD children of different ages, with or without coronary artery lesions (CAL) and with different severity of CAL were compared. Results There were 241 patients (135 boys and 106 girls) in KD group, and the median age was 2.17 (1.04-4.00) years. Forty children were <1 year old, 120 were 1-3 years old, and 81 were >3 years old. There were 94 children in the CAL group and 147 in the non-CAL group. In the CAL group, 68 children were grade Ⅱ, 17 were grade Ⅲ and 9 were grade Ⅳ. The control group included 30 patients (14 boys and 16 girls) and the median age was 3.00 (2.00-5.00) years. The TG and LDL-C levels of KD children in the acute stage were significantly higher than those in the control group, while the levels of TC and HDL-C were significantly lower than those in the control group, and there were statistically significant differences (P<0.05). There were statistically significant differences in TG among different age groups (P<0.05), and TG showed a decreasing trend with the increase of age. TG in the CAL group was significantly higher than that in the non-CAL group, and the difference was statistically significant (P<0.05). There were statistically significant differences in TG among CAL groups with different severity (P<0.05). Conclusions In the acute stage of KD, the levels of TC and HDL-C were decreased, while the levels of TG and LDL-C were increased. The TG level was correlated with the age, whether CAL was complicated or not, and the severity of CAL in KD children.

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    Effects of stress hyperglycemia on organ function in critically ill children
    WU Jie, WANG Quan
    Journal of Clinical Pediatrics    2023, 41 (6): 406-410.   DOI: 10.12372/jcp.2022.23e0258
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    Stress-induced hyperglycemia (SHG) may occur in severe children under stress. It is currently believed that the pathogenesis of SHG is related to abnormal regulation of endocrine hormones, massive release of cytokines, and insulin resistance in critical cases. Continuous elevated blood glucose levels can cause mitochondrial dysfunction, activation of inflammatory pathways, and oxidative stress. Although the association between SHG and poor prognosis has received widespread attention in recent years, strategies to strictly control blood glucose combined with intensive insulin therapy do not seem to benefit critically ill children. In general, blood glcose not exceeding 180 mg/dL (10.0 mmol/L) is appropriate. This article reviews the progress of research on the definition, pathogenesis and harm of SHG in order to improve clinicians’ understanding.

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    Clinical characteristics of mitochondrial encephalomyopathy in children
    ZHUO Muqing, LI Xiaojing, PENG Bingwei, ZHU Haixia, TIAN Yang, ZHENG Kelu, GAO Yuanyuan, WU Wenxiao, WU Wenlin, CHEN Zongzong, CHEN Wenxiong, CAO Binbin
    Journal of Clinical Pediatrics    2023, 41 (9): 661-667.   DOI: 10.12372/jcp.2023.22e1600
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    Objective To explore the clinical features of mitochondrial encephalomyopathy (ME) in children in southern China. Methods The clinical data of children diagnosed with ME from January 2015 to August 2022 were retrospectively analyzed. Results Thirty-six children with ME (22 boys and 14 girls) were included, and the onset age was 6.8 (2.1-10.8) years old. The onset symptoms were stroke-like episodes (24 cases), exercise intolerance (7 cases), ptosis (3 cases) and mental retardation (2 cases). Neuromuscular symptoms during the course of the disease included seizures (17 cases), headache (11 cases), paralysis (11 cases), ataxia (10 cases), ptosis of the upper eyelid (7 cases), mental/motor retardation (7 cases), blurred vision (6 cases), disturbance of consciousness (3 cases), and psychobehavioral abnormality (2 cases). Other non-neuromuscular symptoms included fever (7 cases), vomiting (6 cases), slow weight gain (5 cases), abdominal pain (2 cases), urinary retention (1 case), intestinal obstruction (1 case), and respiratory failure after sedation (1 case). The clinical phenotypes included mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) (16 cases), Leigh syndrome (LS) (11 cases), myoclonus epilepsy with ragged-red fibers (MERRF) (3 cases), Kearns-Sayre syndrome (KSS) (2 cases), and unclassifiable types (4 cases). The blood lactic acid of resting phase was increased in 71.4% of the children, and cerebrospinal fluid lactic acid was increased in 95.2% of the children. There was statistically significant difference between blood lactic acid of resting phase and cerebrospinal fluid lactic acid (P<0.05). Abnormal brain MRI was found in 31 children, mostly involving the parietal lobe (15 cases), occipital lobe (14 cases), basal ganglia (13 cases), brainstem (10 cases) and thalamus (8 cases). Long segmental spinal cord abnormal signals were found in 2 cases of LS. Typical broken red fibers were seen by muscle biopsy in 2 children. Genetic examination found mtDNA variations in 81.8% of the children. Conclusions The onset age of ME in children is around the age of 6. Stroke like attack is the commonest onset symptom. MELAS is the commonest clinical phenotype. The levels of resting blood lactic acid and cerebral spinal fluid lactic acid may increase. Head MRI shows that ME often involves the parietal and occipital lobe, basal ganglia, brainstem and thalamus. The results of genetic examination are mainly mtDNA variations.

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    Beware of the pitfalls in diagnosis and treatment of autoimmune encephalitis in children
    ZHANG Weihua, ZOU Liping, REN Haitao, GUAN Hongzhi
    Journal of Clinical Pediatrics    2023, 41 (9): 644-649.   DOI: 10.12372/jcp.2023.23e0455
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    Over the past 15 years, the development of antibody detection technology led to the increased awareness of autoimmune encephalitis. However, the innovation has also brought about a certain proportion of misdiagnosis, and some patients have been treated improperly as a result. The main reasons for misdiagnosis include lax implementation of diagnostic criteria, incorrect interpretation of antibody test results, and insufficient differential diagnosis. Compared with adults, the symptoms of children with autoimmune encephalitis are more difficult to identify, and there are antibody spectrum and characteristics that are different from those of adults. The differential diagnosis of the disease involves a wider range, and its diagnosis and treatment face greater challenges. This article will summarize the diagnosis and treatment process of autoimmune encephalitis in children based on the current situation in China. The related pitfalls of diagnosis and treatment are interpreted and analyzed in order to help pediatricians improve accurate diagnosis and treatment of autoimmune encephalitis.

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    Related factors analysis of clinical outcome after pulmonary hemorrhage in very low birth weight infants
    ZHU Wen, ZOU Yunsu, WU Yue, LU Keyu, CHENG Rui, TONG Meiling, YANG Yang
    Journal of Clinical Pediatrics    2023, 41 (10): 658-664.   DOI: 10.12372/jcp.2023.22e0562
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    Objective To explore the important prognostic factors of pulmonary hemorrhage in very low birth weight (VLBW) infants. Methods The clinical data of VLBW infants diagnosed with pulmonary hemorrhage admitted to NICU from January 1, 2018 to December 31, 2021 were retrospectively analyzed. Results A total of 64 VLBW neonates were included, 39 in the death group and 25 in the survival group. In the death group, there were 21 neonates with gestational age <28 weeks, 17 with gestational age of 28-31+6 weeks, and 1 with gestational age of 32-33+6 weeks. There were 17 neonates with birth weight <1000 g, 13 with 1000-1250 g, and 9 with 1250-1500 g. Compared with the survival group, the death group had lower birth weight and gestational age, lower age of pulmonary hemorrhage occurrence, higher rates of gestational age <28 weeks and resuscitation by tracheal intubation, and higher rates of Ⅲ-Ⅳ respiratory distress syndrome, symptomatic ductus arteriosus and early-onset sepsis, with statistical significance (P<0.05). Compared with the survival group, the death group had higher peak inspiratory pressure within 24h after birth, higher fraction of inspired oxygen before pulmonary hemorrhage, and higher fraction of inspired oxygen and peak inspiratory pressure after pulmonary hemorrhage; In the death group, the proportion of arterial blood BE<-5mmol/L after birth was higher, and the proportion of arterial blood pH<7.1, BE<-5 mmol/L, PaCO2>50 mmHg was higher when pulmonary hemorrhage occurred; Prothrombin time (PT) and thrombin time were prolonged during pulmonary hemorrhage in the death group. The differences were statistically significant (P<0.05). Binary logistic regression analysis showed that resuscitation by tracheal intubation and PT≥30 s during pulmonary hemorrhage were independent risk factors for death in children with pulmonary hemorrhage (P<0.05). Log-rank test showed that the median survival time in the resuscitation group was shorter than that in the non-resuscitation group, and the median survival time in the PT≥30 s group was shorter than that in the PT<30 s group, the difference was statistically significant (P<0.001). Conclusions In clinical work, attention should be paid to the intrauterine hypoxia of VLBW infants, the reasonable application of tracheal intubation resuscitation after birth, and the active correction of coagulation dysfunction after bleeding, so as to reduce the mortality of pulmonary hemorrhage.

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    Analysis of prognostic risk factors of IgA vasculitis with nephritis in children
    NIE Xiaojing, CHEN Yi
    Journal of Clinical Pediatrics    2023, 41 (12): 895-902.   DOI: 10.12372/jcp.2023.23e0697
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    IgA vasculitis (IgAV) is the most common systemic small vessel vasculitis in children, and it is called IgA vasculitis with nephritis (IgAVN) when it affects the kidneys. Renal damage eventually lead to chronic kidney disease in about 20% children with IgAVN, and accounting for 1%~2% of all childhood end-stage renal disease (ESRD). Therefore, it is important to identify the risk factors for the prognosis of IgAVN in children and give timely and effective intervention for the prognosis of IgAVN. This article reviews the risk factors affecting the prognosis of IgAVN in children, in order to provide reference for clinical decision-making.

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