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    15 May 2024 Volume 42 Issue 5
      
    Commentary
    The China Neonatal Genome Project: towards a new future in the treatment of genetic diseases
    ZHOU Wenhao
    Journal of Clinical Pediatrics. 2024, 42(5):  379-383.  doi:10.12372/jcp.2024.24e0329
    Abstract ( 197 )   HTML ( 1213 )   PDF (1226KB) ( 86 )  
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    With the advancement of science and technology, screening and diagnostic techniques for neonatal genetic diseases have made great leaps forward, especially the application of next-generation sequencing (NGS) technology, which has greatly improved the efficiency and accuracy of clinical applications. However, in the face of rapid diagnosis, it has become a new challenge to provide effective treatments to improve the overall prognosis of children.This article will start from the China Neonatal Genome Project, comprehensively and in-depth discuss the current status of newborn genetic disease screening and treatment, focus on the key issues of personalized treatment research and application, and propose efforts for future directions.

    Promoting the application of new genetic testing technologies in the screening of fetal diseases with a scientific attitude
    QI Qingwei
    Journal of Clinical Pediatrics. 2024, 42(5):  384-389.  doi:10.12372/jcp.2024.24e0303
    Abstract ( 52 )   HTML ( 544 )   PDF (1274KB) ( 12 )  
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    With the development of second-generation sequencing technology, the target diseases of prenatal screening for fetal genetic diseases have gradually progressed from common chromosomal aneuploidy to chromosome microdeletion/microduplication syndromes and single-gene disorders, and the screening strategy has also gradually progressed from chromosomal abnormalities to comprehensive screening strategies for fetal chromosomal aneuploidy, copy number variations and monogenic variations. In the current clinical practice, cell-free DNA testing has become the mainstream technology for prenatal screening, but there is still an urgent need to improve the scope of the target diseases and the detection performance. It is urgent to correctly evaluate the accuracy and screening efficiency of the new genetic testing technology and define its clinical pathway. It is also important to improve the ability of the pre-testing and post-testing counselling of the practitioners, and to evaluate the feasibility of clinical application of new technologies and the accessibility of comprehensive services, and further improving the management system of prenatal screening and prenatal diagnosis are practical issues that need to be addressed urgently.

    Standard·Protocol·Guideline
    Interpretation of Catch-up growth in infants and young children with faltering growth: expert opinion to guide general clinicians
    MAO Meng, SHAO Jie, CHEN Jinjin, SHEN Lixiao
    Journal of Clinical Pediatrics. 2024, 42(5):  390-398.  doi:10.12372/jcp.2024.24e0163
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    Faltering growth is an important clinical signs for early identification of nutritional problems, and early identification and intervention of faltering growth in infants and young children is of great significance for improving their short-term and long-term health outcomes. Previous published guidelines and expert consensus lack a unified definition of faltering growth, which affects clinical identification, evaluation and standardized management. In March 2023, the Journal of Pediatric Gastroenterology and Nutrition published an article titled 'Catch-up growth in infants and young children with faltering growth: expert opinion to guide general clinicians'. It not only expounds the importance of catch-up growth, but also formulates practical consensus recommendations on how to properly define, evaluate and manage faltering growth, which has practical guiding value for clinicians. Based on the current situation of clinical practice in China, this paper interprets the recommendations in order to provide guidance and reference for clinicians to manage infants and young children with faltering growth in China.

    Original Article
    Detection of urine glycosaminoglycans by liquid chromatography-tandem mass spectrometry in the diagnosis and follow-up of patients with mucopolysaccharidosis
    ZHAN Xia, GAO Xiaolan, JI Wenjun, CHANG Siyu, LIU Dan, Zhang Huiwen
    Journal of Clinical Pediatrics. 2024, 42(5):  399-406.  doi:10.12372/jcp.2024.24e0050
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    Objective To establish a method to determine chondroitin sulfate (CS), dermatan sulfate (DS) and heparan sulfate (HS) in urine by liquid chromatography tandem mass spectrometry (LC-MS/MS), and to investigate the application of urine glycosaminoglycans detection in the diagnosis and follow-up of mucopolysaccharidosis (MPS) patients. Methods Twenty urine samples from MPS patients and 37 urine samples from normal children were collected, and glycosaminoglycans levels in these samples were determined. Urine samples were added and dried under nitrogen. Then the samples were derivatized with methanol hydrochloride and dried under nitrogen. After reconstituted, the glycosaminoglycans were analyzed by LC-MS/MS. The linearity, limit of quantification, precision, spiked recoveries, and matrix effect were evaluated. Results The linearity of CS, DS and HS by LC-MS/MS was>0.99. The limits of quantification for CS, DS and HS were 0.5 mg/L, 1 mg/L and 1 mg/L, respectively; the intra-day imprecision ranged from 1.9% to 10.4%, and the inter-day imprecision ranged from 2.6% to 9.8%; and the recovery ranged from 85.6% to 110.4%, the relative matrix effect ranged from 84.9% to 115.5%. CS, DS and HS in urine samples of normal children were normally distributed, then the upper limit of reference interval were preliminarily determined by mean+1.64 SD, which were 16.5 mg/mmol, 1.8 mg/mmol and 1.4 mg/mmol, respectively. Urine GAGs of MPS patients were analyzed, the LC-MS/MS method was effective in detecting MPSⅠ, MPSⅡ, MPSⅢ and MPSⅥ patients. Urine samples of MPSⅠ or MPSⅡ patients who had hematopoietic stem cell transplantation showed reduced DS and HS concentration. Conclusion LC-MS/MS has good performance in detecting urinary mucopolysaccharides and is expected to be used for accurate diagnosis and treatment follow-up monitoring of MPS patients.

    Single center clinical analysis of radiofrequency ablation for children with cardiac enlargement and ventricular preexcitation
    XU Jiangshan, XU Xin, HE Shuang, LIU Qian, TIAN Jie, LYU Tiewei
    Journal of Clinical Pediatrics. 2024, 42(5):  407-413.  doi:10.12372/jcp.2024.23e1065
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    Objective To summarize the clinical characteristics of children with cardiac enlargement and ventricular preexcitation, and to evaluate the safety and effectiveness of radiofrequency ablation (RFCA) in the treatment of this type of disease. Methods A retrospective analysis was conducted on the clinical data of 6 children with cardiac enlargement and ventricular preexcitation in our hospital, and the clinical efficacy and follow-up of ablation treatment were analyzed. Results The median age of 6 children (2 males and 4 females) was 2.0 years at the time of detection of cardiac enlargement, all of whom had overt ventricular preexcitation without tachycardia manifestations and unsatisfactory recovery of cardiac size and cardiac function after regular anti-heart failure medication. All 6 children underwent RFCA, and the median age of the patients at the time of RFCA was 4.4 years. All of the children had intraoperative electrophysiologic examination confirming the presence of right-sided accessory pathway, including 4 cases of right free wall accessory pathway, 2 cases of right septal accessory pathway, and 2 cases of multiple accessory pathways. Postoperative follow-up found that all children had improved ventricular wall motion incoordination and ventricular wall kinematics compared with the previous ones, and postoperative cardiac function was firstly recovered and returned to normal in the shortest postoperative period of 1 month, while cardiac size recovered more slowly and returned to normal in the shortest period of 1.7 years. Conclusions Ventricular preexcitation with cardiac enlargement and no accompanying tachycardia is common in clinical practice. When drug treatment is not effective, RFCA can be attempted to remove the factors of bypass preexcitation. For the diagnosis and differential diagnosis of this type of disease, especially it is a safe and effective treatment. Cardiac enlargement combined with ventricular preexcitation without tachycardia is clinically common, and RFCA can be attempted to remove the factors that cause bypass preexcitation when medication is ineffective, and RFCA not only helps in the diagnosis of this type of disease, but it is also a safe and effective means of treatment.

    Association between SOCS3 expression and glucocorticoid resistance in children with primary nephrotic syndrome
    ZHUANG Yafei, CHENG Jin, WU Chen, GUAN Fengjun
    Journal of Clinical Pediatrics. 2024, 42(5):  414-418.  doi:10.12372/jcp.2024.23e0597
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    Objective To study the relationship between the expression of suppressor of cytokine signaling 3 (SOCS3) and the response to glucocorticoid(GC) treatment in children with primary nephrotic syndrome(PNS). Methods Peripheral venous blood samples were collected from 30 children with steroid-sensitive nephrotic syndrome (SSNS), 16 children with steroid-resistant nephrotic syndrome(SRNS) before and after GC treatment, and 16 healthy children. The expression of SOCS3 mRNA in peripheral blood monocytes (PBMC) and the expression of SOCS3 protein in plasma were detected. At the same time, the 24-hour urine protein, serum creatinine, blood urea nitrogen and glomerular filtration rate were measured before and after GC treatment in PNS children, and their correlation with SOCS3 expression was analyzed. Results Before GC treatment, the SSNS and SRNS groups had significantly higher mRNA and protein expression levels of SOCS3 than those of childen in the control group (P<0.05). After 6 weeks of GC treatment, the mRNA and protein levels of SOCS3 in SSNS group were lower than those before treatment (P<0.05), and the mRNA expression of SOCS3 in SRNS group showed no significant change compared with pre-treatment period (P>0.05), and the protein level of SOCS3 was increased compared to pre-treatment (P<0.05).Before and after GC treatment, the expression levels of SOCS3 mRNA and protein in the SRNS group were significantly higher than those in the SSNS group (P<0.05).The expression of SOCS3 protein in children with PNS was positively correlated with 24hUTP, SCr and BUN (P<0.05), and negatively correlated with GFR (P<0.05). Conclusion There were differences in the expression of SOCS3 between children with SRNS and SSNS, and the differences were correlated with the changes of renal function indexes. These results suggest that the up-regulation of SOCS3 expression has a potential predictive value for GC resistance.

    Clinical features and factors associated with diabetes mellitus in children-onset systemic lupus erythematosus
    LI Zhijuan, BAO Ying, HUANG Huimei, ZHANG Min, WANG Ying, NIU Yunhe, YANG Nan, SUO Lei, LIANG Nan
    Journal of Clinical Pediatrics. 2024, 42(5):  419-424.  doi:10.12372/jcp.2024.23e0068
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    Objective To analyze the clinical characteristics of diabetes mellitus complicated by systemic lupus erythematosus (SLE) in children, and to explore the independent risk factors of diabetes mellitus complicated by SLE. Methods Children with SLE complicated with diabetes mellitus hospitalized in the Department of Nephrology from January 2016 to June 2022 were selected as the diabetes mellitus group, and children with SLE not complicated with diabetes mellitus hospitalized during the same period were selected as the control group, and the clinical data of the 2 groups were collected to analyze the risk factors of SLE complicated with diabetes mellitus. Results Fifteen children (7.4%) with SLE complicated with diabetes were included, 5 males and 10 females. The median age at onset of diabetes was 12.0 (11.0~13.0) years, the median duration of SLE at the time of diabetes was 25.5 (11.5~92.5) days, and the median fasting blood glucose was 11.3 (10.8~13.2) mmol/L. There were 6 cases with family history of diabetes. At the time of diabetes mellitus 10 children were on adequate hormone (prednisone acetate 2 mg·kg-1·d-1, maximum amount ≤60 mg/d) use. The control group included 151 children with SLE (42 males and 109 females). Compared with the control group, the diabetic group was older, had higher systolic and diastolic blood pressure, higher cholesterol and uric acid levels, and lower HDL levels; the proportion of children with abnormally high systolic blood pressure, abnormally high cholesterol and uric acid, abnormally low HDL, and a family history of SLE was higher; and the time for urinary protein to turn negative was longer, and the differences were all statistically significant (P<0.05). Multifactorial logistic regression analysis revealed that age, family history of diabetes mellitus, elevated levels of uric acid and cholesterol, and lowered levels of HDL were independent risk factors for diabetes mellitus complicating SLE (P<0.05). Conclusion If children with SLE are older, have a family history of diabetes mellitus with high levels of cholesterol, low levels of HDL and hyperuricemia, the occurrence of secondary diabetes should be alerted.

    Clinical analysis of 386 cases of secondary thrombocytosis in children
    WANG Li, LIU Aiguo, WANG Yaqin, HUANG Yongjian, HOU Ling, ZHANG Ai, WANG Songmi, HU Qun
    Journal of Clinical Pediatrics. 2024, 42(5):  425-428.  doi:10.12372/jcp.2024.23e0156
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    Objective To understand the clinical characteristics of secondary thrombocythemia in children, and to help clinicians improve their diagnosis and treatment. Methods The clinical data of children with secondary thrombocythemia admitted from January to December 2021 were retrospectively analyzed to summarize the causes of the disease, age and gender distribution, and characteristics of inflammatory factors. Results Three hundred and eighty-six children with secondary thrombocythemia were included, with a mean age of onset of (2.0±0.3) years, and 294 children (76.2%) were<3 years old. The median platelet count was 626.5 (567.8-701.0)×109/L, which peaked at 8.0 (7.0-11.0) days of the disease course and then gradually declined.143 children were regularly monitored for blood counts during the recovery period, and platelets returned to normal at 10.0 (7.0-14.0) days of the disease course. The most common cause of secondary thrombocythemia was infection (94.6%), and the three most common pathogens of infection were Mycoplasma pneumoniae, fungi, and adenovirus. The levels of interleukin (IL)-1β, IL-2 receptor, IL-6, IL-8, IL-10, and tumor necrosis factor (TNF)-α were higher in children with secondary thrombocythemia than in those with normal platelets, and the difference was statistically significant (P<0.05). Conclusion The secondary thrombocythemia is most common in children under 3 years of age, and the most common cause is infection. Platelets can be normalized after treatment for the primary disease, and no inhibition of platelet aggregation and anticoagulation is needed in all children except Kawasaki disease.

    Correlation between overweight and obesity and lung function in children with asthma
    LI Dan, ZHANG Rui, LIU Feng, ZHAO Deyu
    Journal of Clinical Pediatrics. 2024, 42(5):  429-433.  doi:10.12372/jcp.2024.23e0554
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    Objective To study the correlation between overweight and obesity and lung function in children with asthma. Methods A total of 368 children with asthma attending the respiratory outpatient clinic from July 2022 to September 2022 were selected and categorized into 235 cases (63.8%) in the non-overweight and obese group, 57 cases (15.5%) in the overweight group, and 76 cases (20.7%) in the obese group according to the body mass index (BMI), and the indexes of lung function were tested in the three groups of children. These included forced vital capacity (FVC), forced expiratory volume at the end of the first second (FEV1), one-second rate (FEV1/FVC), forced expiratory flow rate (FEF) accounted for 25%, 50%, 75% of forced vital capacity (FEF25, FEF50, FEF75), forced expiratory flow rate in the middle period (FEF25-75), peak expiratory flow (PEF). Results Among overweight and obese children, the proportion of poorly controlled asthma was higher, and the difference was statistically significant when compared with the non-overweight and obese group (P<0.05). Among 368 children with asthma, BMI values were negatively correlated with FEV1/FVC (r=-0.22, P<0.05) and positively correlated with each of the FVC, FEV1, FEF25, FEF50, FEF75, and FEF25-75 (P<0.05). In children with acute exacerbation and remission, the results of correlation between BMI values and lung function indicators were the same as before. Conclusions The proportion of poorly controlled asthma was higher in overweight and obese children. With the increase of BMI, the FEV1/FVC of children with asthma showed a decreasing trend. Therefore, the negative effects of overweight and obesity on children with asthma were mainly manifested in the central airway.

    Mediating role of social skills between motor skills and comprehensible language in 12-month-old children
    JIAN Lanying, LUO Yan, ZHU Yan, SHEN Chengwei, TU Kexin, ZHANG Qiong, MEI Xinjie, QIN Huiling, JIN Lifang
    Journal of Clinical Pediatrics. 2024, 42(5):  434-438.  doi:10.12372/jcp.2024.23e0754
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    Objective To explore the relationship between motor skills and comprehensible language development, and to examine the mediating effects of social skills on them in 12-month-old children. Methods Twelve-month-old infants in the birth cohort who underwent regular follow-up at the Child Health Clinic of Guiyang Maternal and Child Health Hospital from January 2022 to May 2023 were used as the study subjects. A questionnaire survey was performed to collect demographic information. Ages and Stages Questionnaire-Third Edition (ASQ-3) was used to evaluate infants’ motor and social skills. Comprehensible language was evaluated by using Chinese Communicative Development Inventory-Putonghua version (PCDI). Results In 12-month-old children,gross motor, fine motor and social skills were positively correlated with comprehensible language. After adjustment for covariates, social skill played a complete mediating role between gross motor [β=0.27, 95%CI(0.17-0.39)], fine motor [β=0.45, 95%CI(0.30-0.62)] and comprehensible language development in 12-month-old children. Conclusion In 12-month-old children, motor skills can indirectly contribute to the development of comprehensible language through social skills.

    Clinical analysis of the first refractory status epilepticus in pediatric intensive care unit
    ZHOU Yiping, DOU Jiaying, CUI Yun, SHI Jingyi, SUN Ting, ZHANG Yucai
    Journal of Clinical Pediatrics. 2024, 42(5):  439-444.  doi:10.12372/jcp.2024.23e0242
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    Objective To investigate the clinical characteristics and prognosis of refractory status epilepticus (RSE) patients admitted to the pediatric intensive care unit (PICU). Methods Clinical data of children with RSE admitted to the PICU from January 2017 to December 2021 were retrospectively collected, and they were divided into survival and death groups based on survival status. Gender, age, body mass index, etiology, Glasgow Coma Score (GCS) at admission, head imaging, electroencephalogram, antiepileptic drugs, and modified Rankin Scale (mRS) at discharge were summarized. The clinical characteristics, treatment and prognosis of children with RSE were analyzed. Results A total of 80 children with RSE were admitted, 39 (48.8%) were male, median age at onset was 38 (12-80.8) months, including 12 (15%) cases of super-refractory status epilepticus sustained (SRSE).The causes of RSE were severe encephalitis in 50 cases, epileptic syndromes in 9 cases, intracranial tumors/haemorrhages in 7 cases, immune encephalitis in 4 cases, hereditary-metabolic encephalopathy in 4 cases, septic encephalopathy in 3 cases, and septic meningitis in 3 cases. Mechanical ventilation was required in 53 cases (66.3%), and the median duration of mechanical ventilation was 96 (54-239) h. Seventy-seven cases were examined by cranial magnetic resonance imaging, of which 61 cases (79.2%) were abnormal. The median duration of convulsive seizures was 19 (2~46) h, the median length of stay in the PICU was 8.5 (5-17.8) d, and there were 19 deaths (hospitalization morbidity and mortality rate of 23.8%). 61 patients survived to be discharged with a good recovery of neurological function (mRS < 3) in 23 cases (37.7%). Comparing the survival and death groups, there was a significant difference in the proportion of admission GCS scores and imaging abnormalities (both P<0.05). Conclusion The etiology of new-onset RSE in this group of children was mainly severe encephalitis, and the GCS scores and cranial imaging results on admission were of reference value for prognostic judgment.

    Delirium in pediatric intensive care unit with mechanical ventilation: analysis of incidence and influencing factors
    XU Lili, MA Zhushengying, QIAN Wen, XU Yaya, ZHU Yueniu, ZHU Xiaodong, GE Xiaohua
    Journal of Clinical Pediatrics. 2024, 42(5):  445-449.  doi:10.12372/jcp.2024.23e0408
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    Objective To investigate the prevalence of delirium (PD) in children receiving mechanical ventilation (MV) treatment in the pediatric intensive care unit (PICU) and its related influencing factors. Methods The clinical data of children receiving MV treatment in the PICU from July 2022 to March 2023 were retrospectively analyzed. Results 149 children received MV, with a median age of 2.0(1.0-6.0) years, 74 males and 75 females. Among them, 86 children (57.7%) were aged ≤2 years, and 34 children (22.8%) were repeatedly intubated during treatment. Among 149 children, 81 (54.4%) had delirium. Compared with the non-delirium group, the delirium group was younger, had a lower proportion of prone position ventilation, a higher proportion of age ≤2 years, a longer PICU length of stay, and a higher proportion of PICU length of stay >14 days, with statistically significant differences (P<0.05). Compared with the non-delirium group, the delirium group had a higher proportion of oxygenation index ≤150mmHg on the first day of MV, a longer total MV time, and a lower proportion of total MV time ≤168 h, with statistically significant differences (P<0.05). Multivariate logistic regression analysis showed that PICU length of stay > 14 days and oxygenation index ≤ 150 mmHg on the first day of MV were independent risk factors for delirium in children with MV (P<0.05), while total MV time ≤ 168 h was a protective factor for delirium in children with MV (P<0.05). Conclusions The prevalence of delirium in children receiving MV treatment in PICU is higher, which is closely related to hypoxic injury. Long-term MV and PICU hospitalization are also important factors contributing to the development of delirium.

    Clinical analysis of five patients with 17α-hydroxylase deficiency from three families
    MA Xiuqi, TANG Yijun, CHEN Yao, LIU Qian, ZHANG Xiaofang, CHEN Lin, ZHANG Yi, WANG Xike, WANG Xiumin
    Journal of Clinical Pediatrics. 2024, 42(5):  450-455.  doi:10.12372/jcp.2024.23e0665
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    Objective To investigate and summarize the diagnosis and treatment of 17α-hydroxylase deficiency. Methods Clinical data of a total of 5 cases of 17α-hydroxylase deficiency (17OHD) from 3 families admitted to the Department of Pediatric Endocrinology and Genetic Metabolism from November 2015 to February 2023 were retrospectively analyzed, and relevant literature was reviewed. Results Hypertension, hypokalemia and low sex hormone levels were present in all 5 cases, 4 cases had no secondary sexual development, and 2 children with 46, XY underwent gonadal biopsy and bilateral orchiectomy, and postoperative gonadal pathology showed dysplasia of testis. Genetic analysis showed that 3 cases had homozygous variants of c.985_987delinsAA, which resulted in amino acid shift line mutation (p.Tyr329Lysfs*90), and the other two cases had compound heterozygous varaiants of c.785T>G and c.1193C>T. Hypokalemia and hypertension were controlled by glucocorticoid therapy. Conclusions Early recognition and diagnosis of 17α-hydroxylase deficiency and timely glucocorticoid replacement therapy can achieve a satisfactory outcome and improve the quality of life of the children. Gene sequencing helps to clarify the molecular diagnosis of this rare disease.

    Clinical Report
    Acute cerebellar ataxia associated with SARS-CoV-2 infection in children: a case report
    ZHANG Yan, WANG Caihong, WANG Jinhui, YU Hui, LIN Caimei
    Journal of Clinical Pediatrics. 2024, 42(5):  456-460.  doi:10.12372/jcp.2024.23e0227
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    To investigate the clinical characteristics and treatment of acute cerebellar ataxia in children associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The clinical features, neuroimages, therapy and prognosis of a girl with acute cerebellar ataxia associated with SARS-CoV-2 infection who was hospitalized in Children′s Hospital of Fudan University at Xiamen were retrospectively analyzed and the related literature were summarized. The patient was a girl aged 4 years and 11 months. She was admitted due to cough for 2 weeks and fever for 3 days. On the 3rd day of admission, she was treated by intravenous human immunoglobulin injection with a total amount of 2g/kg (divided into 4 days) and with methylprednisolone 2mg/kg. After significant improvement, she was discharged from hospital with oral prednisone acetate at 1mg/kg/d. At 11 days of follow-up, the girl had returned to normal, and prednisone was stopped. Three articles and 3 children with acute cerebellar ataxia associated with SARS-CoV-2 infection were reported. The age of onset was 5 years old, 13 years old, and 15 years old, respectively. All 3 cases were males, and the onset was 1-2 weeks after SARS-CoV-2 infection. All of them had ataxia gait. Two patients treated with intravenous methylprednisolone, and they were recovered to normal within 2 months. Acute cerebellar ataxia caused by SARS-CoV-2 infection usually appears 1 to 2 weeks after infection, and the clinical manifestations were ataxic gait, which was mostly caused by post-infection immune response, and the prognosis was good.

    Literature Review
    Research progress on mechanism of lipid metabolism in asthma
    CHEN Shaotian, YANG Nan
    Journal of Clinical Pediatrics. 2024, 42(5):  461-466.  doi:10.12372/jcp.2024.23e0201
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    Asthma is one of the most common chronic respiratory diseases, affecting all age group. Its prevalence and social healthcare costs are increasing every year. The results of numerous studies in recent years have conclusively demonstrated the role of lipid molecules as powerful signaling molecules that regulate a variety of cellular biological processes. By regulating different cells in the airways of asthma patients, they influence the occurrence and development of the disease. Therefore, this article summarizes the potential metabolic biomarkers in asthma, and explores the mechanisms of lipid metabolism in asthma pathogenesis on different cells to provide ideas for personalized asthma treatment from the lipidomics and asthma studies that have been developed in recent years.

    Re-recognition of the complement involvement in the pathogenesis of kidney diseases in children
    WANG Peipei, ZHANG Pei, GAO Chunlin, XIA Zhengkun
    Journal of Clinical Pediatrics. 2024, 42(5):  467-473.  doi:10.12372/jcp.2024.23e0595
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    Complement is an important component of innate immunity, as well as a bridge to regulate adaptive immunity. It is an important substance that mediates cellular inflammatory response and induces apoptosis. The cascade of the complement response is stictly regulated by the stringent control of the complennent regulatory proteins. Should the complement overactivated or regulate abnormally, it can cause damage to the host cells. In the glomerular microenvironment, dysregulation of the complement system can lead to C3 glomerulopathy and atypical hemolytic uremic syndrome. In addition, complement is also hyperactivated in many other inflammatory kidney diseases, such as ANCA-associated glomerulonephritis, lupus nephritis, IgA nephropathy, membranous nephropathy, and diabetic nephropathy. In addition, it may mediate renal allograft injury by playing a role in ischemia-reperfusion injury and rejection. This article reviews the role of complement in the pathogenesis of glomerular diseases and related targeted therapy.