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Journal of Clinical Pediatrics    2024, 42 (1): 1-14.   DOI: 10.12372/jcp.2024.23e1129 Abstract （1057）   HTML （75）    PDF（pc） （743KB）（1174）       Knowledge map    Save Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （259）   HTML （29）    PDF（pc） （1239KB）（194）       Knowledge map    Save 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 Reference | Related Articles | Metrics

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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 Abstract （258）   HTML （8）    PDF（pc） （1231KB）（145）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （218）   HTML （11）    PDF（pc） （1293KB）（250）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （210）   HTML （10）    PDF（pc） （1250KB）（130）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （202）   HTML （20）    PDF（pc） （1121KB）（153）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （201）   HTML （11）    PDF（pc） （1175KB）（138）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （188）   HTML （31）    PDF（pc） （1230KB）（145）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （187）   HTML （9）    PDF（pc） （1895KB）（324）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （178）   HTML （275）    PDF（pc） （1272KB）（143）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （173）   HTML （16）    PDF（pc） （1183KB）（140）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （170）   HTML （16）    PDF（pc） （1138KB）（189）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （170）   HTML （12）    PDF（pc） （1206KB）（311）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （169）   HTML （10）    PDF（pc） （1259KB）（169）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （168）   HTML （25）    PDF（pc） （1708KB）（109）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （168）   HTML （24）    PDF（pc） （1187KB）（117）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （167）   HTML （16）    PDF（pc） （1229KB）（188）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （165）   HTML （10）    PDF（pc） （1272KB）（111）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （163）   HTML （12）    PDF（pc） （1526KB）（96）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （163）   HTML （13）    PDF（pc） （1272KB）（106）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （161）   HTML （17）    PDF（pc） （1221KB）（110）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （161）   HTML （7）    PDF（pc） （1183KB）（164）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （156）   HTML （11）    PDF（pc） （1685KB）（137）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （156）   HTML （12）    PDF（pc） （1096KB）（189）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （152）   HTML （5）    PDF（pc） （1267KB）（130）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （151）   HTML （15）    PDF（pc） （1143KB）（377）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （145）   HTML （8）    PDF（pc） （1193KB）（78）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （145）   HTML （10）    PDF（pc） （1228KB）（131）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （143）   HTML （16）    PDF（pc） （1254KB）（182）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （143）   HTML （9）    PDF（pc） （1218KB）（87）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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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 Abstract （139）   HTML （18）    PDF（pc） （1897KB）（175）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （138）   HTML （16）    PDF（pc） （1198KB）（203）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （138）   HTML （10）    PDF（pc） （1299KB）（243）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （137）   HTML （16）    PDF（pc） （1612KB）（95）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （137）   HTML （10）    PDF（pc） （1304KB）（120）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （134）   HTML （14）    PDF（pc） （1377KB）（89）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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Journal of Clinical Pediatrics    2023, 41 (9): 703-707.   DOI: 10.12372/jcp.2023.22e1763 Abstract （133）   HTML （18）    PDF（pc） （1211KB）（107）       Knowledge map    Save Table and Figures | Reference | Related Articles | Metrics

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Progress and challenges in clinical research of umbilical cord blood transplantation for the treatment of premature infant diseases LI Fang, WANG Li Journal of Clinical Pediatrics    2023, 41 (10): 646-653.   DOI: 10.12372/jcp.2023.23e0634 Abstract （132）   HTML （15）    PDF（pc） （1443KB）（125）       Knowledge map    Save Despite great progress in the treatment of neonatal diseases, complications of preterm infants such as bronchopulmonary dysplasia, perinatal brain injury and necrotizing enterocolitis are still leading causes of neonatal mortality and long term morbidity. Exploring more effective therapies for the prevention or treatment for the complications of preterm infants are the concerns in neonatology. Stem cell therapy especially umbilical cord blood transplantation, have been explored as a new therapeutic option in premature infant diseases, with very promising preclinical and clinical results over the past 20 years. This article outlined the clinical progress, prospects and challenges of stem cells derived from umbilical cord blood in preterm diseases. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （131）   HTML （10）    PDF（pc） （1165KB）（110）       Knowledge map    Save 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. Reference | Related Articles | Metrics

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Renal pathologic classification and treatment strategy in children with lupus nephritis GAO Chunlin Journal of Clinical Pediatrics    2023, 41 (12): 887-894.   DOI: 10.12372/jcp.2023.23e0916 Abstract （130）   HTML （14）    PDF（pc） （1367KB）（120）       Knowledge map    Save Lupus nephritis (LN) is the most common glomerular disease in children, with a more severe onset and progression compared to adults. The prognosis of LN in children is worse than other glomerular diseases and has lifelong effects, although the etiology remains unclear. This article provides an overview of the current diagnosis and treatment status of LN in children, along with a brief description of the treatment for each type of LN based on the latest international guidelines. LN was diagnosed according to the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus. Renal biopsy pathology is an important diagnostic method at present. Treatment for proliferative and non-proliferative LN includes initial therapy and maintenance therapy, following recommendations from adult guidelines. Rational and proactive treatment is crucial for inducing disease remission, preventing relapse, and improving prognosis. Table and Figures | Reference | Related Articles | Metrics

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Clinical analysis of risk factors for recurrence of acute pancreatitis in children TAN Chunxiu, YANG Ying, WANG Yuanzhen, HUANG Liya Journal of Clinical Pediatrics    2023, 41 (7): 526-529.   DOI: 10.12372/jcp.2023.22e1226 Abstract （129）   HTML （3）    PDF（pc） （1273KB）（87）       Knowledge map    Save Objective To investigate the risk factors for the recurrence of acute pancreatitis in children. Methods The clinical data of children with acute pancreatitis (AP) admitted to the General Hospital of Ningxia Medical University from December 2010 to December 2020 were analyzed retrospectively. The children were divided into AP group and recurrent acute pancreatitis (RAP) group. The clinical characteristics of the two groups were compared, and the influencing factors of AP recurrence in children were analyzed. Results A total of 90 AP children (55 boys and 35 girls) were included, and the median age was 9.0 (6.0-12.0) years. In the RAP group, 24 patients recurred 6.6 (1.0-11.6) months after the first onset of AP, with a median age of 10.0 (7.0-12.0) years. The common causes of recurrence were idiopathic AP (9 cases, 37.5%), pancreatic anatomical abnormalities (7 cases, 29.2%) and biliary origin (5 cases, 20.8%). Among the 90 children with AP, 15 had severe AP, including 8 in RAP group and 7 in AP group. Among 90 AP children, the incidence of abdominal pain and vomiting were 83.3% and 32.2%. The incidence of pancreatic necrosis and pancreatic pseudocyst was 5.6% and 6.7%. Multivariate logistic regression analysis showed that abnormal pancreatic anatomy and severe AP were independent risk factors for recurrence of AP in children (P<0.05), and idiopathic AP was an independent protective factor for recurrence of AP in children (P<0.05). Conclusions Patients with severe AP and abnormal pancreatic anatomy are prone to recurrence, which should be paid attention to by clinicians. Table and Figures | Reference | Related Articles | Metrics

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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 Abstract （128）   HTML （3）    PDF（pc） （1225KB）（116）       Knowledge map    Save 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. Table and Figures | Reference | Related Articles | Metrics

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Research progress on the role of intestinal flora in sepsis WANG Yanfei, TAN Linhua Journal of Clinical Pediatrics    2023, 41 (8): 634-640.   DOI: 10.12372/jcp.2023.22e0820 Abstract （128）   HTML （13）    PDF（pc） （1259KB）（350）       Knowledge map    Save Sepsis is defined as life-threatening organ dysfunction caused by a dysregulated host response to infection which affects children's health. The intestinal flora play an important regulatory role in host metabolism and immunity and are associated with a variety of diseases. Studies have shown that sepsis and clinical treatments can lead to intestinal flora imbalance in children, which further affects the prognosis of the disease; while healthy intestinal flora can reduce the susceptibility of children to sepsis and improve the survival of sepsis, and ameliorate the damage to the function of organs. This article reviews the related research on intestinal flora and sepsis, in order to contribute to the prevention and treatment of sepsis in children. Reference | Related Articles | Metrics

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Clinical analysis of idiopathic and symptomatic occipital lobe epilepsy in children YANG Yating, CAI Yuehao, FANG Qiong, CHEN Lang, CHEN Qiaobin, LIN Zhi, WU Feifei, LIN Meng Journal of Clinical Pediatrics    2023, 41 (9): 668-673.   DOI: 10.12372/jcp.2023.22e1059 Abstract （127）   HTML （14）    PDF（pc） （1723KB）（121）       Knowledge map    Save Objective To investigate the clinical characteristics and treatment of idiopathic and symptomatic occipital lobe epilepsy in children. Methods The clinical data of children with occipital lobe epilepsy diagnosed from April 2013 to April 2022 were retrospectively analyzed. The patients were divided into idiopathic group (including early-onset group and late-onset group) and symptomatic group. The clinical characteristics, auxiliary examination results and treatment were compared between the groups. Results A total of 80 children (28 girls and 52 boys) with occipital lobe epilepsy were included. The median age of onset was 7.0 (5.0-7.0) years old and the median course of disease was 1.7 (1.0-2.5) years. There were 38 children in the early-onset group, 24 in the late-onset group and 18 in the symptomatic group. The age of onset, the proportion of head eye deflection, autonomic nervous symptoms, visual hallucinations, eye clonus, eyelid flutter and falls, and the proportion of abnormal vision and nervous system positive signs during physical examination existed significant differences among the three groups (P<0.05). The proportion of intelligence test score below normal and head MRI abnormalities, and the incidence of abnormal background, bilateral occipital discharge and eye closure sensitivity in the interictal electroencephalogram (EEG) were significantly different among three groups (P<0.05). There were statistically significant differences in the proportion of antiepileptic therapy, monotherapy and the utilization rate of sodium valproate, levetiracetam and topiramate among the three groups (P<0.05). Conclusions The clinical characteristics of idiopathic and symptomatic occipital lobe epilepsy in children are different. The corresponding treatment plan need to be formulated based on the comprehensive evaluation of clinical seizure forms, head imagings and EEG characteristics. Table and Figures | Reference | Related Articles | Metrics

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Clinical analysis of autoimmune glial fibrillary acidic protein astrocytopathy in children HOU Chi, CHEN Wenxiong, LIAO Yinting, WU Wenxiao, TIAN Yang, ZHU Haixia, PENG Bingwei, ZENG Yiru, WU Wenlin, CHEN Zongzong, LI Xiaojing Journal of Clinical Pediatrics    2023, 41 (9): 656-660.   DOI: 10.12372/jcp.2023.22e1735 Abstract （127）   HTML （15）    PDF（pc） （1421KB）（86）       Knowledge map    Save Objective To investigate the clinical characteristics, treatment and prognosis of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) in children. Methods The clinical data of children diagnosed with GFAP-A from June 2018 to July 2022 were retrospectively analyzed. Results Five patients were diagnosed with GFAP-A, including 3 boys and 2 girls, and the onset age was 11.0 (5.0-13.5) years. The commonest initial symptom was fever (4 cases), and the commonest neurological symptoms and signs were headache (5 cases) and neck rigidity with positive Kerning sign (5 cases). Cerebrospinal fluid examination (CSF) in acute stage showed elevated white blood cell count and protein level in 4 cases and protein-cell separation in 1 case. The median CSF GFAP antibody titer was 1:10 (1:10-1:100), and 1 patient had combined N-methyl-D-aspartate receptor (NMDAR) antibody. The commonest magnetic resonance imaging (MRI) findings were cerebral leptomeningeal enhancement (4 cases). In acute phase, all children received first-line immunotherapy (high-dose intravenous methylprednisolone combined with intravenous immunoglobulin), and 4 children had a good response. One patient with positive CSF NMDAR antibody had no response to first-line treatment, but improved after rituximab treatment. During a follow-up of 8.0 (4.0-36.0) months, no neurological sequelae associated with the disease were detected. Conclusions The commonest symptoms and signs of GFAP-A in children are fever, headache and meningeal irritation, and leptomeningeal enhancement is the commonest MRI change. Most children have a good response to first-line immunotherapy and have a good prognosis during follow-up. Table and Figures | Reference | Related Articles | Metrics

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Diagnosis and treatment characteristics and long-term follow-up of 51 cases of infantile neuroblastoma XU Quan, YUAN Xiaojun Journal of Clinical Pediatrics    2023, 41 (9): 680-685.   DOI: 10.12372/jcp.2023.22e1756 Abstract （126）   HTML （13）    PDF（pc） （1210KB）（108）       Knowledge map    Save Objective The clinical characteristics, diagnosis and treatment characteristics and long-term curative effect of the children with neuroblastoma (NB) at the age of less than 12 months were analyzed to provide evidence-based basis for further optimizing the treatment of infantile NB patients. Methods The clinical data of infantile NB patients admitted from January 2008 to December 2018 were retrospectively analyzed, and the clinical characteristics and prognostic factors of the children were summarized. Results The median age of diagnosis in 51 NB infants (34 boys and 17 girls) was 7.5 (3.8-10.1) months, and abdominal mass (27 cases, 52.9%) was the commonest reason for medical treatment. The most frequently-occurring sites of tumors were adrenal gland (21 cases, 41.4%) and retroperitoneum (19 cases, 37.2%). Bone marrow, liver and bone were the commonest sites of metastasis. The amplification frequency of MYCN gene was 11.3% (5/44). Forty-nine NB patients underwent surgery, of whom 8 were treated with surgical resection alone, 13 were treated with surgery after chemotherapy, and 28 were treated with surgery before chemotherapy. Of the remaining patients, 1 received chemotherapy alone and 1 did not receive any treatment except follow-up. The median follow-up time was 78.5 (72.1-124.0) months. A total of 49 patients survived without an event. Two patients died. The 6-year overall survival rate and the 6-year event-free survival rate were both (96.1±2.7)%. Univariate analysis revealed that tumor staging, distant metastasis, bone marrow metastasis, bone involvement, risk grouping, MYCN amplification, serum neuron-specific enolase and lactate dehydrogenase levels at initial diagnosis were factors affecting prognosis (P<0.05). Conclusions Infantile NB patients have a good long-term prognosis. It should be expected to further reduce the intensity of chemotherapy for patients without MYCN amplification. Table and Figures | Reference | Related Articles | Metrics

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Correlation analyses between serum uric acid and diabetic ketoacidosis in children with initially diagnosed type 1 diabetes LIU Fang, CAO Bingyan, WANG Shiqi, CHEN Qiong, WEI Haiyan Journal of Clinical Pediatrics    2023, 41 (6): 424-429.   DOI: 10.12372/jcp.2023.22e1769 Abstract （125）   HTML （4）    PDF（pc） （1369KB）（100）       Knowledge map    Save Objective To explore the correlation between serum uric acid (SUA) and diabetic ketoacidosis (DKA) of children in initially diagnosed type 1 diabetes (T1DM). Methods The clinical data of children with T1DM admitted from March 2015 to June 2022 were retrospectively analyzed. The clinical characteristics of children between DKA group and non-DKA group were compared, and the correlation between SUA level and the occurrence of DKA in children with initially diagnosed T1DM was analyzed. Results A total of 358 children with T1DM (190 boys and 168 girls) were included, and the median age was 5.8 (3.0-8.9) years. There were 186 children (52.0%) in the DKA group and 172 in the non-DKA group. There were 57 children in the mild DKA group, 64 in the moderate DKA group and 65 in the severe DKA group. A total of 49 children with HUA were diagnosed, including 43 (23.1%) in the DKA group. The blood glucose, SUA level and the proportion of co-infection on admission in the DKA group were higher than those in the non-DKA group, and the C-peptide and glomerular filtration rate in the DKA group were lower than those in the non-DKA group, and the differences were statistically significant (all P<0.05). The receiver operating characteristic (ROC) curve showed that the area under the ROC curve of SUA predicting the DKA occurrence in children with initially diagnosed T1DM was 0.94 (95%CI: 0.91-0.97). When SUA≥294.2 μmol/L, the sensitivity and specificity predicting the DKA occurrence were 92.5% and 89.0%, respectively. Spearman rank correlation analysis showed that the SUA level was negatively correlated with the valueslevels of pH and HCO-3 in children with initially diagnosed T1DM (P<0.01). There were statistically significant differences in SUA levels between non-DKA group and DKA groups with different degrees (P<0.01). Pairwise comparison showed that SUA levels in mild, moderate and severe DKA groups were higher than those in non-DKA group, with statistical significance (P<0.05). Conclusions The determination of SUA level has certain clinical significance in assisting the assessment of whether the initial T1DM is combined with DKA, which is conducive to the disease assessment and can be carried out in grass-roots hospitals. Table and Figures | Reference | Related Articles | Metrics

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The application value of 5 scoring methods in the prognosis evaluation of acute respiratory distress syndrome in children QIAO Junying, ZHANG Luodan, LI Fan, ZHAO Jianchuang, GUO Shanshan, ZHANG Jingpo Journal of Clinical Pediatrics    2023, 41 (6): 442-449.   DOI: 10.12372/jcp.2023.22e1197 Abstract （124）   HTML （3）    PDF（pc） （1264KB）（297）       Knowledge map    Save Objective To explore the application value of pediatric critical illness score (PCIS), pediatric risk of mortality (PRISM) Ⅲ score, pediatric sequential organ failure assessment (pSOFA) score, radiographic assessment of lung edema (RALE) score and acute lung injury score (LIS) in the prognosis evaluation of pediatric acute respiratory distress syndrome (pARDS). Methods The clinical data of children diagnosed with ARDS admitted to the pediatric intensive care unit (PICU) from January 2015 to December 2021 were retrospectively analyzed. Results A total of 80 ARDS children with sepsis and multiple organ dysfunction were enrolled, including 45 boys and 35 girls, and the median age was 12.5 (4.0-36.3) months. There were 50 patients in the survival group and 30 patients in the death group (16 died during hospitalization and 14 died after abandonment). Compared with the survival group, the death group had higher oxygenation index (OI), higher pSOFA and PRISM Ⅲ scores at transferring to PICU and diagnosing ARDS, higher RALE and LIS scores at diagnosing ARDS, and higher proportion of vasoactive substances application and blood transfusion. Furthermore, compared with the survival group, the death group had shorter total hospital stay, PICU stay and mechanical ventilation time, and lower proportion of intrapulmonary factors, PO2/FiO2 and PCIS scores. The differences were statistically significant (P<0.05). Compared with the group without underlying diseases, the scores of pSOFA, PRISM Ⅲ, RALE, and LIS were higher and the scores of PCIS were lower at the diagnosis of ARDS in the group with underlying diseases, the differences were statistically significant (P<0.05). The AUCs of PCIS, pSOFA, PRISM Ⅲ, RALE, and LIS at the diagnosis of ARDS in predicting the death of ARDS children were 0.73, 0.89, 0.83, 0.80 and 0.82, respectively. Hosmer-Lemeshow goodness of fit test showed that PCIS had the best fitting effect between predicted mortality and actual mortality (χ2=4.16, P=0.656). Conclusions PCIS, pSOFA, PRISM Ⅲ, RALE and LIS scores all have good predictive ability for the prognosis of children with ARDS, and pSOFA score had the highest predictive value. PCIS had the best fitting effect between predicted mortality and actual mortality. Table and Figures | Reference | Related Articles | Metrics

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Research advances of the bronchiolitis obliterans syndrome following allogeneic hematopoietic stem cell transplant in children XI Bixin, HU Qun, LIU Aiguo Journal of Clinical Pediatrics    2023, 41 (8): 629-633.   DOI: 10.12372/jcp.2023.22e1137 Abstract （124）   HTML （13）    PDF（pc） （1216KB）（238）       Knowledge map    Save Bronchiolitis obliterans syndrome (BOS) is one of the rare and fatal non-infectious pulmonary complications after allogeneic haematopoietic stem cell transplantation (HSCT) in children. Mutiple risk factors such as primary graft dysfunction, graft-versus-host disease (GVHD), lymphocytic bronchiolitis, gastro-oesophageal reflux, and air pollution, particularly in children, have been reported to be the most common contributions to the bronchiolitis obliterans syndromepost-HSCT. An early diagnosis and treatment of BOS following HSCT is challenging due to the lack of obvious early symptoms, high risk of open lung biopsy, irreversible pathophysiological changes, and the failure of steroid treatment in some children. In this review, we summarize research advances in the epidemiology, pathogenesis, diagnosis, and treatment of this lethal noninfectious pulmonary complication, and provide suggestions for further improvement of diagnostic and therapeutic norms applicable to post-HSCT BOS in children. Table and Figures | Reference | Related Articles | Metrics

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Association of coefficient of glycemic variation and SNAPPE-Ⅱ with prognosis in critically ill neonates XIANG Chao, ZHANG Rong, KANG Lan, LEI Xiaoping, LIU Xingqing, DONG Wenbin Journal of Clinical Pediatrics    2023, 41 (6): 430-435.   DOI: 10.12372/jcp.2023.22e0086 Abstract （123）   HTML （6）    PDF（pc） （1382KB）（73）       Knowledge map    Save Objective To explore the association of coefficient of glycemic variation (CV) and score for neonatal acute physiology with perinatal extension-Ⅱ (SNAPPE-Ⅱ) with prognosis in critically ill neonates. Methods The clinical data of critically ill neonates admitted to the neonatal intensive care unit (NICU) from August 2018 to September 2020 were retrospectively analyzed. According to the prognosis, they were divided into death group and survival group, and the difference of clinical features between the two groups was compared. Binary logistic regression model was used to analyze the risk factors of poor prognosis in critically ill neonates. Receiver operating characteristic (ROC) curve analysis was used to evaluate the diagnostic value of CV and SNAPPE-Ⅱ in poor prognosis of critically ill neonates. Results A total of 385 critically ill newborns were included, including 228 boys and 157 girls. The median age of admission was 1.0 (1.0-24.0) hours, the mean gestational age was (35.3±4.1) weeks, and the mean weight was (2456.6±860.3) grams. There were 85 hypoglycemic and 115 hyperglycemic patients. There were 80 patients in the death group, and the median time from NICU admission to death were 5.0 (2.0-10.3) days. Binary logistic regression analysis showed that CV, SNAPPE-Ⅱ, gestational age, weight, the main diagnosis of pulmonary hemorrhage and neonatal sepsis were correlated with death of critically ill neonates (P<0.05). The area under curve (AUC) of CV predicting death of critically ill neonates was 0.72, and the AUC of SNAPPE-Ⅱwas 0.85. When CV≥0.31, the sensitivity and specificity for the diagnosis of critical neonatal death were 0.75 and 0.59. When SNAPPE-Ⅱ≥18.50, the sensitivity and specificity for the diagnosis of critical neonatal death were 0.68 and 0.93. Conclusions Both CV and SNAPPE-Ⅱ have certain predictive values for the poor prognosis of critically ill neonates. Table and Figures | Reference | Related Articles | Metrics