"Journal of Clinical Pediatrics" is a monthly magazine. It was founded in 1983. ISSN-1000-3606 is the international magazine and CN:31-1377/R is the national standard magazine. It is a professional academic journal of Pediatrics sponsored by Shanghai Institute of Pediatrics Medicine and Xinhua Hospital affiliated to Shanghai Jiaotong University Medical College. Medical College of Shanghai Jiaotong...
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15 March 2024, Volume 42 Issue 3
  • Diagnosis and treatment philosophy and practice of macrolide-resistant Mycoplasma pneumoniae pneumonia in children
    YIN Yong, CHEN Jiande
    2024, 42(3):  177-181.  doi:10.12372/jcp.2024.24e0003
    Abstract ( 59 )   HTML( 47 )   PDF (1272KB) ( 47 )  

    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.

    Macrolide resistance in hospitalized children with Mycoplasma pneumoniae pneumonia and its clinical diagnosis and treatment
    GU Yutong, YANG Fen, YE Jianmin, HUA Li, LI Jing, ...
    2024, 42(3):  182-186.  doi:10.12372/jcp.2024.24e0025
    Abstract ( 43 )   HTML( 33 )   PDF (1396KB) ( 33 )  

    Objective To summarize the epidemiological characteristics and drug resistance rate of Mycoplasma pneumoniae (MP) infection in hospitalized children from April to October 2023, and analyze the clinical and therapeutic characteristics of MPP children during this period. Methods Throat swab samples of children with community-acquired pneumonia were collected on the day of hospitalization. The tNGS pathogen and MP macrolide-resistance gene mutation were detected, and MP positive rate and MP macrolide-resistance rate were analyzed. Patients with MPP were grouped by the admission date into non-epidemic group (June) and epidemic group (September), and further divided into resistant group and sensitive group in accordance to whether the macrolide-resistance genes were positive or not. The clinical features of the children were compared among the groups. The treatment differences were discussed comparatively between the non-epidemic resistant group and the epidemic resistant group. Results The median age of 1425 children (686 boys and 739 girls) with community-acquired pneumonia was 6 (3-8) years, and the MP positive rate was 57.1% (813 cases). The positive rate of MP varied significantly from April to October for each month (P<0.001), and MP positive rate increased month by month (12.5%-71.6%). Among 813 hospitalized children with MPP, 627 were positive for macrolide-resistance gene mutation, and the total drug resistance rate of MP was 77.1%. The drug resistance rate of MP was 93.9% in the period of non-epidemic (April to June), and significantly decreased to 71.9% in the epidemic period (August to October). The drug resistance rate of MP varied significantly from April to October for each month (P<0.001). There were 76 children in the non-epidemic group and 189 in the epidemic group. Compared with the non-epidemic group, the length of hospital stay were longer and the levels of CRP and LDH were higher in the epidemic group, with statistical significance (P<0.05). There were 64 children in the sensitive group and 201 in the resistant group. Compared with the sensitive group, the hospital stay was longer and the LDH level was higher in the resistant group, and the difference was statistically significant (P<0.05). Of the 201 children with macrolide-resistant MPP, 71 were in the non-epidemic resistant group and 130 were in the epidemic resistant group. Compared with the non-epidemic resistant group, the time to defervescence after methylprednisolone was longer and the use rate of tetracycline antibiotics was higher in the epidemic resistant group, and the difference was statistically significant (P<0.05). Conclusions The epidemic period of MP is from August to October 2023. Compared with normal days, the infection rate of MP is higher during the epidemic period, but the macrolide-resistant rate of MP in hospitalized children is lower. MPP during this epidemic period triggered stronger immune response, displayed as longer hospital stay and higher inflammatory factor level. The possibility of a new prevalent strain should be considered. This prevalent strain was less sensitive to combine treatment of macrolide plus corticosteroids, and tetracycline drugs are needed to control infection.

    Clinical features and risk factors of macrolide-resistant severe Mycoplasma pneumoniae pneumonia in children
    CHEN Mengxue, LI Jingyang, YANG Fen, TIAN Ye, LI J...
    2024, 42(3):  187-192.  doi:10.12372/jcp.2024.24e0026
    Abstract ( 32 )   HTML( 27 )   PDF (1378KB) ( 27 )  

    Objective To explore the clinical features and risk factors of macrolide-resistant severe Mycoplasma pneumoniae pneumonia (SMPP) in children. Methods The clinical data of children with macrolide-resistant Mycoplasma pneumoniae pneumonia (MPP) who were hospitalized in the Department of Pediatric Respiratory Medicine from March to September 2023 were retrospectively analyzed. According to the severity of the disease, the patients were divided into macrolide-resistant severe group and macrolide-resistant non-severe group. The clinical characteristics of the two groups were compared, and the risk factors affecting SMPP were analyzed. Results A total of 224 children with macrolide resistant MPP were included, including 132 in the severe group (65 boys and 67 girls), with a median age of 7.0 (5.0-9.0) years. There were 92 patients in the macrolide-resistant non-severe group, 46 boys and 46 girls, with a median age of 7.0 (5.0-9.0) years. The results of binary logistic regression analysis showed that prolonged fever time, increased D-dimer level and lung consolidation were independent risk factors for predicting the occurrence of macrolide-resistant SMPP (P<0.05), while higher lactate dehydrogenase and IgE levels may be correlated with the occurrence of macrolide-resistant SMPP (P<0.05). ROC curve analysis showed that the duration of fever, lactate dehydrogenase and D-dimer had moderate diagnostic value in predicting the occurrence of macrolide-resistant SMPP (AUC>0.8, P<0.05). All patients with macrolide-resistant Mycoplasma pneumoniae pneumonia were cured and discharged after treatment. The length of hospital stay in the macrolide-resistant severe group was 7.0 (6.0-9.0) days, significantly longer than that in macrolide-resistant non-severe group [6.0 (5.0-7.0) days] (P<0.01). The hospitalization cost in the macrolide-resistant severe group was 12283 (10836-15012) yuan, which was significantly higher than that in the macrolide-resistant non-severe group [9769 (8756-11642) yuan], and the differences were statistically significant (P<0.01). Conclusions Children with macrolide-resistant SMPP experienced more prolonged fevers and hospital stays, markedly elevated inflammatory markers, and severe alterations in lung imaging, all of which raised the financial strain on their families.

    Clinical characteristics analysis of Mycoplasma pneumoniae necrotizing pneumonia and bacterial necrotizing pneumonia
    JI Xiaodan, ZHAO Shunying
    2024, 42(3):  193-197.  doi:10.12372/jcp.2024.23e0044
    Abstract ( 32 )   HTML( 20 )   PDF (1306KB) ( 20 )  

    Objective To analyze the clinical features of Mycoplasma pneumoniae necrotizing pneumonia (MPNP) and bacterial necrotizing pneumonia (BNP) and to observe the changes of inflammatory indicators. Methods The clinical data of 72 children diagnosed with MPNP or BNP from 2016 to 2021 were retrospectively analyzed. Results There were 43 children in MPNP group and 29 children in BNP group. The median age was 7.9 years in MPNP group and 1.9 years in BNP group (P<0.01). In the BNP group, the rates of shortness of breath, triple concave sign, and non-invasive ventilator use were 79.3%, 37.9%, and 51.7%, respectively, whereas in the MPNP group, the rates were 37.2%, 9.3%, and 18.6% (P<0.05). Streptococcus pneumoniae and Staphylococcus aureus were the predominant pathogens in the BNP group, making up 76% of all cases. The median C-reactive protein and procalcitonin levels in the BNP group within 3 days of disease course were 83 mg/L and 29.9 ng/mL respectively, which were higher than those in the MPNP group (14 mg/L and 0.1 ng/mL) (P<0.05). At days 4-7 and 8-14 of disease course, the median neutrophil ratio in MPNP group was 78.2% and 80.4%, while that in BNP group was 65.8% and 59.8% (P<0.05). There was no significant difference in the total number of white blood cells between the two groups at days 1-3 of disease course (P>0.05). The median blood D-dimer concentration in the MPNP group was 4.1 mg/L, which was higher than that in the BNP group (3 mg/L) (P<0.05). The pleural fluid leukocytes in MPNP group were mainly mononuclear cells, while those in the BNP group were mainly multinucleated cells. The glucose level of pleural fluid in BNP group was (2±2) mmol/L, which was significantly lower than that in the MPNP group [(6±2) mmol/L, P<0.01)]. The time of pulmonary cavity necrosis in MPNP group was (23.4±10.0) days, while that in BNP group was (10.4±5.7) days (P<0.01). In MPNP group, pulmonary embolism was observed in 8 cases, bronchoscopic plastic formation was observed in 7 cases, and sequelae (lumen stenosis and occlusion) were observed in 21 cases. Pyopneumothorax occurred in 12 cases of BNP group. Conclusions In BNP group, C-reactive protein and procalcitonin increased significantly at days 1-3 of disease course. The children in BNP group were prone to dyspnea and pyopneumothorax. In MPNP group, the proportion of neutrophil increased significantly after 4 days of disease course, the level of D-dimer in blood was higher, the occurrence of pulmonary cavity necrosis was late, and pulmonary embolism was easy to occur, and the plastic formation could be seen under bronchoscopy, and the incidence of sequelae of lumen stenosis and occlusion was high.

    A study of allergen-specific IgE in children in Suzhou from 2018 to 2021
    WANG Pengli, XU Lina, JIANG Wujun, LI Pengyun, MA ...
    2024, 42(3):  198-203.  doi:10.12372/jcp.2024.23e1083
    Abstract ( 26 )   HTML( 10 )   PDF (1422KB) ( 10 )  

    Objective To investigate the changes of allergen-specific IgE in children with allergic diseases in Suzhou from 2018 to 2021. Additionally, the study will examine the characteristics of allergen sensitization in connection to age and gender, as well as changes in lifestyle and environment. Methods A retrospective analysis of the detection of common inhalant and food-specific IgE in suspected allergic children aged 0-18 years from 2018 to 2021 was conducted. Results A total of 43028 suspected allergic children were included, with a positive sIgE rate of 64.4% (27706/43028). Before the COVID-19 pandemic, the positive rate was 60.6% in 2018 and 58.6% in 2019. It was 70.1% in 2020 and 67.2% in 2021 during the COVID-19 pandemic. There was a significant difference in the positive rate among different years (P<0.001). The top 4 inhalant allergens with the highest sIgE positivity in suspected allergic children were dust mite mix, house dust, mold mix, and cat hair; the top 4 food allergens were egg, milk, beef, and soybean. The positive rates of all inhaled (except cockroaches) and food allergen-specific IgE detected from 2020 to 2021 were higher than those from 2018 to 2019, and the differences were statistically significant (P<0.05). Among male subjects, the sIgE positivity rates for tree mix, common ragweed, mugwort, dust mite mix, house dust, cat hair, dog epithelium, and cocklebur were higher than those in female subjects, with statistically significant differences (P<0.05). The sIgE positivity rates for milk, peanut, beef, lamb, fish mix, shrimp, and crab were higher in male subjects than in female subjects, with statistically significant differences (P<0.05). The sIgE positivity rates for inhalant (except for crab) and food allergens differed significantly among different age groups (P<0.05). Conclusion During the COVID-19 pandemic, the positive rates of inhaled (except cockroach) and food allergen-specific IgE increased in children with suspected allergic diseases, and the positive rates were different in different genders and age groups. The trend of multi-sensitization increased.

    Pathogenic characteristics of neonatal sepsis and influence factors of gram-negative bacterial infection: based on a 5-year retrospective clinical study
    BIAN Zhaonan, ZHA Xinyi, ZHANG Xi, CHEN Xuting, CH...
    2024, 42(3):  204-210.  doi:10.12372/jcp.2024.23e0661
    Abstract ( 20 )   HTML( 6 )   PDF (1478KB) ( 6 )  

    Objective To investigate the distribution and drug resistance of bacteria in neonatal sepsis and to identify the influence factor for gram-negative (G-) bacterial infection. Methods The clinical data of patients diagnosed with neonatal sepsis admitted from January 1, 2018 to December 31, 2022 were collected, and the characteristics and drug resistance of major pathogenic bacteria were analyzed. Based on the pathogenic bacteria's gram staining results, all patients were categorized into groups of gram-positive (G+) and G- bacteria. The influencing factors for G- bacterial infection were analyzed by binary logistic regression, and the value of each factor was evaluated according to the area under the receiver operating characteristic curve (AUC). Results A total of 208 strains of pathogenic bacteria were detected in 201 neonatal sepsis patients, of which 126 (60.6%) were G+ bacteria and 82 (39.4%) were G- bacteria. Coagulase negative staphylococci (CoNS) was the most common G+ bacteria with high resistance to penicillin, erythromycin and oxacillin. Klebsiella pneumoniae was the most common G- bacteria with high resistance to penicillins and cephalosporins. Comparing the proportion of pathogenic bacteria in different years, it was found that the proportion of G- bacteria showed an increasing trend in the past five years. According to the results of pathogen gram staining, 201 patients were divided into G+ bacteria group (n=119) and G- bacteria group (n=82). Univariate analysis and binary logistic regression analysis showed that hospital-acquired infection, white blood cell count (WBC) < 5×109/L, C-reactive protein (CRP) and procalcitonin (PCT) were the influential factors in differentiating G- bacterial infection (P<0.05). The AUC of the combination of the above influencing factors for identifying G- bacterial infection was 0.888 (95%CI: 0.840~0.936, P<0.01). Conclusions CoNS and Klebsiella pneumoniae were the main pathogens of neonatal sepsis. The proportion of G- bacterial infection showed an increasing trend from 2018 to 2022. Hospital-acquired infection, WBC<5×109/L and higher CRP and PCT levels were risk factors for G- bacterial infection. The combination of hospital-acquired infection, WBC<5×109/L, CRP and PCT had high predictive value for G- bacterial infection.

    Correlation between cognitive function and electroclinical characteristics of benign childhood epilepsy with centrotemporal spikes
    CHEN Shicai, DUAN Lifen, SUN Ying, SHAO Juwei, LI ...
    2024, 42(3):  211-217.  doi:10.12372/jcp.2024.22e1748
    Abstract ( 24 )   HTML( 7 )   PDF (1320KB) ( 7 )  

    Objective To investigate the association of cognitive function and clinical factors with electroencephalogram epileptiform discharges in benign childhood epilepsy with centrotemporal spikes (BECT). Methods The clinical data of children with BECT monitored by long-term video electroencephalogram from January 2019 to July 2022 were retrospectively analyzed, and the healthy children who underwent physical examination in outpatient department during the same period were selected as control group. Chinese Revised Wechsler Intelligence Scale for Children (C-WISC) test was conducted in both groups of children. The intelligence quotient of the two groups was compared, and the relationship between clinical factors, electroencephalogram epileptiform discharges and total intelligence quotient (FIQ), verbal intelligence quotient (VIQ) and performance intelligence quotient (PIQ) in children with BECT were analyzed. Results A total of 42 patients (18 boys and 24 girls) with typical BECT were included, with an average age of (9.4±2.0) years and an average onset age of (7.4±2.6) years. The control group consisted of 45 healthy children, including 28 boys and 17 girls, with an average age of (9.7±2.2) years. FIQ, VIQ, scores of classification and vocabulary in VIQ sub-items, as well as wood block map in PIQ sub-items in BECT group were lower compared with the control group, with statistical significance (P<0.05). FIQ and VIQ in <10-year-old group were lower than those in ≥10-year-old group among 42 children with BECT, and the differences was statistically significant (P<0.05). There was a statistically significant difference in VIQ among different discharge side groups (P<0.05), and the score of bilateral discharge group was lower. The onset age of children with BECT was significantly positively correlated with FIQ (r=0.39), VIQ (r=0.57) and scores of knowledge (r=0.49), classification (r=0.35), vocabulary (rs=0.62), comprehension (r=0.43) and digit span (r=0.37) in VIQ sub-items. The course of disease was significantly negatively correlated with the scores of knowledge (rs=-0.31) in VIQ sub-items (P<0.05). Compared with the group with onset age ≥10 years old, the scores of knowledge and vocabulary in VIQ sub-items in the group with onset age <10 years old were lower, and the difference was statistically significant (P<0.05). There were statistically significant differences in the scores of knowledge and digit span in VIQ sub-items among left, right and bilateral groups (P<0.05). Conclusions The average intelligence level of BECT children is in the normal range, but there is an imbalance in the intellectual structure, mainly manifested as impaired language function. Early onset age, bilateral cerebral hemispheres discharges and long course of disease are the main factors that affect the language function and lead to the decline of cognitive function in children with BECT.

    Clinical features and long-term prognosis of 25 patients with systemic juvenile idiopathic arthritis complicated with macrophage activation syndrome
    LIU Ping, JIANG Chunya, WEI Lei, WANG Xiangli, JIN...
    2024, 42(3):  218-223.  doi:10.12372/jcp.2024.22e1773
    Abstract ( 19 )   HTML( 7 )   PDF (1305KB) ( 7 )  

    Objective To investigate the clinical features and long-term prognosis of systemic juvenile idiopathic arthritis (sJIA) complicated with macrophage activation syndrome (MAS). Methods The clinical data of sJIA children complicated with MAS treated from August 2014 to August 2020 were retrospectively analyzed. The sJIA children without MAS diagnosed and treated in the same period were classified as sJIA without MAS group. The clinical manifestations and long-term prognosis were compared between the two groups. Results The median onset age of the 25 patients (11 boys and 14 girls) in sJIA with MAS group was 5.8 (2.6-10.0) years. The median onset age of 38 patients (18 boys and 20 girls) in sJIA without MAS group was 7.0 (5.1-9.0) years. Twenty-one patients had MAS in the early stage of sJIA. Compared with the sJIA without MAS group, the incidence of hepatosplenomegaly, respiratory system involvement, central nervous system involvement and bleeding in the sJIA with MAS group was higher, and the difference was statistically significant (P<0.05). Methemoglobinemia was the most common abnormal laboratory index in sJIA patients with MAS (100%), followed by elevated aspartate aminotransferase (24 cases) and decreased fibrinogen (23 cases). All patients were treated with glucocorticoids, 22 with intravenous immunoglobulin and 22 with cyclosporin A. Tocilizumab was added to 11 patients, 4 of them were treated with tocilizumab within 2 to 3 weeks of MAS activity, and 7 were treated with tocilizumab when sJIA was still in an unstable state after MAS remission. The median follow-up time was 40.0 (26.5-60.5) months. The results of follow-up to August 31, 2022 showed that there were 11 cases of monophasic course, 5 cases of recurrent course, and 9 cases of persistent course in the sJIA with MAS group, and there was no statistically significant difference in long-term prognosis compared with the sJIA without MAS group (P>0.05). Growth retardation occurred in 4 sJIA children with MAS who received glucocorticoid therapy for a long duration. Hormonal cataract occurred in 3 sJIA children with MAS. Conclusions For sJIA children complicated with MAS, if early identification, effective treatment and standardized management can be given in time, the long-term prognosis is similar to that of children without MAS.

    Clinical characteristics, genotypes and follow-up analysis of children with isovaleric academia
    SUN Yuning, LIANG Lili, DING Si, LIU Yuchao, CHEN ...
    2024, 42(3):  224-229.  doi:10.12372/jcp.2024.23e1218
    Abstract ( 21 )   HTML( 11 )   PDF (1350KB) ( 11 )  

    Objective To explore the clinical features, gene variation and follow-up results of children with isovaleric acidemia (IVA), and to provide evidence for early recognition, diagnosis and treatment of IVA. Methods The clinical data of children diagnosed with IVA in the Department of Pediatric Endocrinology from April 2012 to October 2023 were retrospectively analyzed. Results Of the 34 children with IVA (22 boys and 12 girls), 15 had acute neonatal type, 3 had chronic intermittent type, and 16 had asymptomatic type confirmed by neonatal screening. Compared with asymptomatic group, blood isovalerylcarnitine (C5) level, C5/ acetylcarnitine (C2) ratio, C5/ propionylcarnitine (C3) ratio and urine isovalerylglycine level in the group with clinical symptoms (including acute neonatal type and chronic intermittent type) were significantly increased, with statistical significance (P<0.05). A total of 40 mutations were identified in 34 patients, most of which were missense mutations (30/40, 75.0%) and c.1208A>G (n=8) was the most frequent variant. There was no significant correlation between clinical manifestation and type of gene variation. Four patients, all of whom belonged to the acute neonatal type, died during follow-up. Thirty surviving children had considerably lower urinary isovalerylglycine levels at the most recent follow-up, with a statistically significant difference from before therapy (P<0.05). Conclusions Clinical manifestations of IVA lack specificity. Elevated C5, C5/C2 ratio and C5/C3 ratio in plasm and isovalerylglycine in urine are typical hallmarks of IVA. Newborn screening is pivotal for early diagnosis, treatment, and prognosis.

    Clinical characteristics and genetic variation analysis of Shwachman-Diamond syndrome in seven children
    WANG Ruifang, LIANG Lili, ZHANG Kaichuang, YANG Yi...
    2024, 42(3):  230-237.  doi:10.12372/jcp.2024.23e1219
    Abstract ( 20 )   HTML( 9 )   PDF (1320KB) ( 9 )  

    Objective To investigate the clinical phenotype and genetic variant characteristics of children with Shwachman-Diamond syndrome (SDS). Methods From January 2018 to September 2023, seven children with SDS under long-term follow-up in the Department of Pediatric Endocrine and Genetics were selected as the research objects. The clinical data of children were gathered and peripheral blood samples were collected for exome sequencing (ES), and lineage validation of the variants was performed by Sanger sequencing. Results Among the 7 children with SDS, 3 were boys and 4 were girls. The median age at first diagnosis was 3.0 (0.9-4.0) years. Six patients carried SBDS defects and one case carried EFL1 defect. Among the six patients with SBDS deficiency, five of them carried compound heterozygous variants (two cases with the variants of c.258+2T>C/c.183_184delinsCT, one case with c.258+2T>C/c.40A>G, one case with c.258+2T>C/c.184A>T, one case with c.258+2T>C/ heterozygous deletion of exon 3), and the remaining one patient carried SBDS homozygous variation (c.258+2T>C). Patients with SBDS deficiency were initially diagnosed with short stature (6/6, 100%), and combined with chronic diarrhea (3/6, 50%) and recurrent respiratory infections (1/6, 16.7%). Through examination, all the six patients with SBDS deficiency (100%) were found to have neutropenia and elevated liver enzymes. Four cases had skeletal dysplasia and 3 cases had exocrine pancreatic dysfunction. One SDS patient was confirmed to carry compound heterozygous variants of EFL1 (c.2260C>T/c.316G>A). The main clinical manifestations included short stature and abnormal skeletal development, without pancreatic and blood system involvement. Conclusions Children with SBDS deficiency presented clinical phenotypic heterogeneity. The phenotypic spectrum and variation spectrum of SDS in China were enriched, and an SDS patient with EFL1 variant was reported for the first time in the Chinese population. For children with short stature and symptoms such as neutropenia, exocrine pancreatic dysfunction, and skeletal deformities, genetic testing should be completed to avoid missing the diagnosis of SDS.

    Clinical features and treatment of anti-melanoma differentiation-associated gene 5 antibody-positive juvenile dermatomyositis
    ZHANG Jin, CHEN Jiang, MIN Yue, SONG Xiaoxiang, FE...
    2024, 42(3):  238-242.  doi:10.12372/jcp.2024.22e1028
    Abstract ( 16 )   HTML( 5 )   PDF (1555KB) ( 5 )  

    Objective To summarize the clinical features of anti-melanoma differentiation-associated gene 5 antibody (MDA5)-positive juvenile dermatomyositis (JDM), and to study its treatment and evaluation. Methods The basic information, clinical manifestations, laboratory and imaging examinations, treatment and prognosis of 5 children with anti-MDA5-positive JDM who were hospitalized from January 2019 to May 2022 were retrospectively analyzed. Results All 5 patients (3 boys and 2 girls) had typical Gottron rash/sign, 3 had skin ulcer, 2 had skin calcification, and 1 had digital necrosis. All the 5 patients had normal muscle strength, but abnormal muscle MRI. Myositis specific antibodies were positive for anti-MDA5 antibodies in all children, and 3 patients were positive for anti-Ro-52. All 5 patients showed interstitial lung disease on high-resolution CT. Only 1 patient had shortness of breath, 2 had elevated KL-6, and 2 had digestive tract involvement. All 5 patients were treated with corticosteroids, and 4 were treated with cyclophosphamide as the initial immunosuppressant, and all of them were partially relieved within 2 months. Conclusions The onset age of anti-MDA5-related JDM is relatively low, the clinical manifestations are primarily typical rash, muscle involvement is not evident, primarily clinically amyopathic dermatomyositis, and the incidence of interstitial lung disease is high. Anti-MDA5 antibody titer, high-resolution CT, KL-6 examination is particularly important for diagnosis and evaluation of disease efficacy. The main treatment is methylprednisolone combined with immunosuppressive agents. The combined treatment with tofaciib is effective.

    Allogeneic hematopoietic stem cell transplantation for treatment of dyskeratosis congenita: a report of 3 cases and literature review
    WANG Yingjie, CHEN Zhiwei, MAI Yumiao, SUN Pan, JI...
    2024, 42(3):  243-248.  doi:10.12372/jcp.2024.23e0266
    Abstract ( 22 )   HTML( 10 )   PDF (1299KB) ( 10 )  

    Objective To investigate the efficacy of allogeneic hematopoietic stem cell transplantation (HSCT) in the treatment of children with dyskeratosis congenita (DKC), and to provide a basis for exploring the optimal transplantation program. Methods The clinical data of 3 children with DKC treated by HSCT in our hospital from June 2019 to June 2022 were retrospectively analyzed and the related literatures were reviewed. Results Three patients all underwent HSCT with a fludarabine-based reduced-intensity conditioning (RIC) regimen. The median count of mononuclear cells (MNC) and CD34+ cells was 13.7(8.3-17.3)×108/kg and 8.2(6.3-13.1)×106/kg respectively. A combination of methotrexate, cyclosporin A and mycophenolate mofetil was used to prevent graft-versus-host disease (GVHD). All 3 patients were able to tolerate the HSCT pretreatment. The median time of neutrophil implantation was 11(10-11) days, and the median time of platelet implantation was 11(10-13) days, and no primary implantation failure occurred. Two patients developed acute GVHD of different severity, 1 developed chronic GVHD, and 1 developed reversible posterior leukoencephalopathy syndrome (RPLS) after transplantation. The follow-up was completed in January 2023 and the median follow-up time was 19(11-42) months. All 3 patients survived, and 1 had chronic GVHD. At present, all 3 children were free from blood transfusion, and no DKC-related gene mutation was detected in blood cells after transplantation. The growth and development of the patients was similar to that of normal children of the same age, and no combined solid tumor has been found. Conclusions The pretreatment regimen of " Flu+low-dose cyclophosphamide (CTX) + anti-thymocyte globulin (ATG)" for HSCT treatment in DKC patients was tolerable, and no primary implantation failure occurred. GVHD is still the main factor affecting the survival of children, and it is necessary to explore the clinical study of individualized treatment for DKC.

    Umbilical cord blood transplantation in the treatment of metachromatic leukodystrophy: a report of 3 cases
    GUAN Liqiang, JIANG Fan, CHEN Jiao, LIU Zhouyang, ...
    2024, 42(3):  249-252.  doi:10.12372/jcp.2024.22e1424
    Abstract ( 21 )   HTML( 5 )   PDF (1259KB) ( 5 )  

    Objective To investigate the safety and efficacy of umbilical cord blood transplantation (UCBT) in the treatment of metachromatic leukodystrophy (MLD). Methods The clinical data of 3 MLD children treated with unrelated UCBT from April to September 2019 were retrospectively analyzed. Results All the 3 children showed walking disorder, progressive motor function regression, and 2 of them showed mental retardation. Lysosomal arylsulfatase A (ARSA) decreased in all of them. Head MRI of all the 3 children showed abnormal signals of different degrees in the white matter of the brain and they all had compound heterozygous mutations of ARSA gene. All the 3 children were treated with unrelated UCBT, and the pre-treatment regimen was fludarabine + cyclophosphamide + rabbit anti-human thymocyte immunoglobulin + busulfan, and the prevention of graft-versus-host disease (GVHD) was treated with cyclosporine + mycophenolate mofetil + methotrexate. All the 3 children were successfully implanted, and the whole blood of all children reached complete donor chimerism within 1 month after transplantation. Head MRI showed that the progression of brain parenchymal lesions gradually stopped, and ARSA levels gradually returned to normal. All 3 patients developed infection, 2 developed acute GVHD and 1 developed chronic GVHD. After anti-infection and regulatory immunotherapy, they all improved. The last follow-up was in August 2022, and all 3 patients survived. Long-term efficacy and neurological recovery still require long-term follow-up.Conclusion UCBT is a safe and effective treatment to prevent the progression of MLD.

    Clinical and etiological analysis of adenovirus infection and liver damage in children
    ZHANG Ying, XU Hongmei
    2024, 42(3):  259-263.  doi:10.12372/jcp.2024.22e0909
    Abstract ( 29 )   HTML( 13 )   PDF (1337KB) ( 13 )  

    Human adenovirus infection in children is a common clinical disease that may be life-threatening. Since its discovery, the virus has been found all over the world and can occur in all four seasons. Recently, there have been a number of cases of unexplained severe hepatitis in children. Some of the children were treated with liver transplantation due to their critical condition. The occurrence of the disease may be related to adenovirus infection. This article summarizes the cases of adenovirus infection complicated by liver damage in children that have been reported in the last ten years, and explores their etiology.

    Advances in the application of anti-IgE monoclonal antibodies combined with allergen immunotherapy
    LI Shuxian, LIU Jinling, HE Jing, CHEN Zhimin
    2024, 42(3):  264-269.  doi:10.12372/jcp.2024.22e1077
    Abstract ( 18 )   HTML( 8 )   PDF (1334KB) ( 8 )  

    Anti-IgE monoclonal antibodies have been approved for the treatment of asthma and chronic urticaria, and anti-IgE monoclonal antibodies combined with allergen immunotherapy (AIT) have attracted widespread attention as an adjunctive therapy for allergic diseases. Studies have shown that the addition of anti-IgE monoclonal antibodies during or before AIT treatment significantly reduces the frequency and severity of AIT adverse reactions, significantly improves the symptom scores, and shortens the time required to achieve maintenance doses. Larger, high-quality, placebo-controlled trials are still needed to better identify the beneficiaries of anti-IgE monoclonal antibodies in conjunction with AIT, as well as the optimal dose and duration of treatment, and to assess long-term benefits and conduct cost-benefit analysis. This article reviews the application of anti-IgE monoclonal antibodies as adjunctive therapy in AIT.

    Gene therapy for mucopolysaccharidosis type Ⅱ
    CHEN Guoqing, ZHANG Huiwen
    2024, 42(3):  270-276.  doi:10.12372/jcp.2024.23e0682
    Abstract ( 18 )   HTML( 7 )   PDF (1330KB) ( 7 )  

    The two major treatments for mucopolysaccharidosis type Ⅱ (MPS Ⅱ) are hematopoietic stem cell transplantation (HSCT) and enzyme replacement therapy (ERT). The restoration of central nervous system function is limited by these two therapies, although they are successful in controlling the worsening of physical symptoms. The main limitation is that the blood-brain barrier (BBB) prevents large molecules, including enzymes, from entering the brain, which can affect the effectiveness of treatment. Gene therapy is an emerging therapy for MPS Ⅱ. This article will describe the current preclinical research status and ongoing clinical trials of MPS Ⅱ gene therapy to demonstrate the latest advances in this field.

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