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06 June 2023, Volume 38 Issue 6 Previous Issue   

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Action plan for the rational use of antibiotics in children in China （2023—2025） National Center for Children’s Health, National Clinical Research Center for Respiratory Diseases, Chinese Pediatric Society, Chinese Medical Association, et al 2023, 38(6): 401-406.  DOI: 10.19538/j.ek2023060601 Abstract ( )   Antimicrobial resistance is a global public health threat. Antimicrobial use is closely related to antimicrobial resistance. Infectious diseases are the most common diseases in children， and antibiotics are the drugs most commonly used in children. The antimicrobial resistance in children in China is still serious. data from the monitoring network of antimicrobial prescriptions used in Chinese children show that broad-spectrum antibiotics are the main antibiotics prescribed for Chinese children. The first edition of Action Plan for the Rational Use of Antibiotics in Children in China （2017—2020） was launched five years ago. After summarizing the relevant practices on the rational use of antibiotics in Chinese children and the data from the monitoring network of antimicrobial prescriptions in Chinese children， ten specific measures have been formulated to promote the rational use of antibiotics in children in China and control antimicrobial resistance.

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Consensus on diagnosis and treatment of childhood vasculitis in China：Behcet’s syndrome Academic Group of Pediatric Rheumatology and Immunology, Society of Pediatrics of Chinese Medical Doctor Association 2023, 38(6): 407-412.  DOI: 10.19538/j.ek2023060602 Abstract ( )   Behcet’s syndrome is a kind of inflammatory vessel disease with multi-system variable， which mainly involves oral and genital mucosa， skin， digestive tract and eyes. The disease usually occurs in young adults， with about 15%~20% of childhood onset. The clinical manifestation and severity of paediatric Behcet’s syndrome are different from those in adults. Due to the lack of specific laboratory indicators， there is often a long time interval between the onset of Behcet’s syndrome and the emergence of typical clinical manifestations， so the diagnosis and treatment of paediatric Behcet’s syndrome are more challenging. Based on adult guidelines and child classification standards and combined with the opinions of some domestic rheumatology and immunology experts， this consensus aims to provide a reference basis for standardizing the diagnosis and treatment of paediatric Behcet’s syndrome in China.

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Expert consensus on the management of pediatric patients with leukemia/tumor during the SARS-CoV-2 pandemic（Guangdong Province， 2023 edition）  Hematology and Oncology Group of Pediatric Branch of Guangdong Medical Association 2023, 38(6): 413-418.  DOI: 10.19538/j.ek2023060603 Abstract ( )   Children with leukemia/tumor are immunocompromised due to the characteristics of the disease and anti-tumor treatment. These patients are susceptible to SARS-CoV-2 infection， and the severe infection can result in high death rates， posing great challenges to pediatric medical staff in this area. Given that the SARS-CoV-2 pandemic will continue to prevail in a fluctuating manner and there are questions raised by doctors needed to be answered， this consensus is to propose expert recommendations that provide a reference for clinical practice based on literature and national documents， including vaccination of SARS-CoV-2， SARS-CoV-2 testing， precautions for anti-leukemia/tumor treatment and the management of the patients with concurrent SARS-CoV-2 infection.

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Interpretation of ESPGHAN Position Paper on Management and Follow-up of Children and Adolescents with Celiac Disease ZHAO Shi-guo, ZHANG Chen-mei 2023, 38(6): 419-425.  DOI: 10.19538/j.ek2023060604 Abstract ( )   In 2022， the European Society of Paediatric Gastroenterology Hepatology and Nutrition issued a position paper on the management and follow-up of celiac disease in children and adolescents， asking 10 questions considered essential for the management and follow-up of celiac disease， including the need for follow-up， the frequency of follow-up and what should be evaluated， how to evaluate adherence to a gluten-free diet， when to expect catch-up growth， how to handle high levels of anti-tissue glutaminase antibodies， indications for small bowel biopsy， how to evaluate quality of life， how to manage gluten challenge tests in children with unclear diagnosis， how to improve communication with children or parents， and how to carry out transition to adult disease management， and so on. Based on the literature pevidence or expert opinions， 37 recommendation have been made. This paper only introduces the main contents of the position paper.

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Advances in the diagnosis and treatment of juvenile systemic scleroderma ZHANG Cai-hui, SONG Hong-mei 2023, 38(6): 426-430.  DOI: 10.19538/j.ek2023060605 Abstract ( )   Juvenile systemic sclerosis （JSSc） is a chronic autoimmune disease with its pathogenesis not fully understood， and both genetic and environmental factors are involved in the development of the disease. Early diagnosis is essential， and early intervention is related to good prognosis. During diagnosis and treatment， the patient should be systematically evaluated regularly. Clinical evaluation tools such as juvenile systemic sclrosis severity score（J4S） scoring and modified Rodnan skin score（mRSS）scoring have been widely used in clinical practice. Skin ultrasound and nail fold microcircul-ation can evaluate the disease to a certain extent. A combina-tion of glucocorticoids， methotrexate， cyclophosph-amide， or mycop-henolate mofetil can be considered depen-ding on the severity of the organ involvement. In cases unresponsive to immunosuppressive agents or glucocorticoids， biological agents such as tocilizumab，rituximab or hematopo-ietic stem cell transplantation can be selected. As compared with adults， JSSc have overall better outcomes and lower mortality.

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Diagnosis and treatment of renal injury in children with anti-neutrophil cytoplasmic antibody associated vascu-litis YANG Qin, WANG Mo 2023, 38(6): 430-438.  DOI: 10.19538/j.ek2023060606 Abstract ( )   Antineutrophil Cytoplasmic Antibody associated vasculitis （AAV） is a group of small necrotizing vasculitis with little or no deposition of immune complex. It is rare in children and can involve multiple organs and is life-threatening. Kidney involvement is common and can result in ANCA-associated glomerulonephritis （AAGN）， which has a high probability of progression to end-stage renal disease and is a key factor determining the prognosis of the disease. In view of the rarity of AAV in children， there are few randomized controlled trials and guidelines for children， and most of them refer to recommendations from  adult guidelines， especially for the diagnosis and treatment of AAGN.This article aims to briefly review the research progress in the diagnosis and treatment of AAGN in recent years.

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Progress in diagnosis and treatment of neuropsychiatric systemic lupus erythematosus in children CHEN Qiao-ping, WU Xiao-chuan 2023, 38(6): 438-442.  DOI: 10.19538/j.ek2023060607 Abstract ( )   Neuropsychiatric systemic lupus erythematosus （NPSLE）is systemic lupus erythematosus （SLE） with central or peripheral nervous system involvement.NPSLE is the second leading cause of morbidity and mortality in patients with SLE， following lupus nephritis.The pathogenesis of NPSLE is complex， including the formation of autoantibodies， blood-brain barrier injury， thromboembolism， and inflammatory mediators.The most common symptom of NPSLE in children is headache. Other common symptoms include seizures， cognitive dysfunction， mood disorders， psychosis， and cerebrovascular disease.Currently， there is no unified diagnostic criteria for NPSLE， which requires comprehensive evaluation of clinical symptoms， serological tests， cerebrospinal fluid examination， imaging and other aspects， and the exclusion of other causes.Symptomatic therapy， glucocorticoid， immunosuppressant， and anticoagulant therapy are the main therapeutic methods for children with NPSLE.

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Childhood primary Sjögren’s syndrome： An update on diagnosis and treatment XU Meng, LIU Cong-cong, LIU Jin-xiang, et al 2023, 38(6): 442-450.  DOI: 10.19538/j.ek2023060608 Abstract ( )   Childhood primary Sjögren’s syndrome is less easily diagnosed in early stage because of differences in clinical presentations from those of adulthood， as well as due to unsatisfactory efficiency of current adult classification criteria in diagnosing pediatric patients. It is urgent to explore the classification/diagnostic criteria specific to children. The diagnostic role of imaging techniques and biomarkers in children need to be further elucidated. Although researches in the pathogenesis have been deepened in the past decades， clinical trials focused on the treatment of childhood primary Sjögren’s syndrome have long been neglected， and the evidence in drug efficacy is limited. Pediatric rheumatologists need to address these issues through carrying out more targeted and profound studies.

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Progress in pathogenesis and precise diagnosis and treatment of systemic juvenile idiopathic arthritis LUO Xi-wen, TANG Xue-mei 2023, 38(6): 450-455.  DOI: 10.19538/j.ek2023060609 Abstract ( )   Systemic juvenile idiopathic arthritis （sJIA） is a subtype of juvenile idiopathic arthritis， which has unique clinical and pathological characteristics. Macrophage activation syndrome （MAS） most frequently occurs in sJIA. It progresses rapidly and can be life-threatening. The etiology of sJIA and sJIA with MAS remains unclear，which is a major challenge in childhood rheumatism. This review summarizes the progress in the diagnosis and treatment of sJIA， emphasizing the early warning，diagnosis and precise treatment， to reduce the rate of disability and mortality and improve the prognosis of the disease.

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Progress in diagnosis and treatment of Takayasu arteritis in children HE Ting-yan, YANG Jun 2023, 38(6): 455-458.  DOI: 10.19538/j.ek2023060610 Abstract ( )   Takayasu arteritis （TA） is the most common systemic great vasculitis in children. The clinical manifest-ations， classification and diagnostic criteria and prognosis of  TA in children are different from those in adults. This article will focus on the epidemiology， pathogenesis， clinical manifestations， diagnosis， treatment and prognosis of TA in children， aiming to improve the understanding of the disease among clinicians， so as to promote early diagnosis and treatment and improve the prognosis.

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Diagnosis and treatment progress of monogenic lupus in children ZHANG Tian-yu , MA Ming-sheng 2023, 38(6): 459-463.  DOI: 10.19538/j.ek2023060611 Abstract ( )   The pathogenesis of systemic lupus erythematosus is complex， and it is currently believed that genetic susceptibility，immune and environmental factors are involved. With the development of gene detection technology， more and more monogenic lupus has been found. The different genetic variants and the causal molecular pathway are associated with distinct clinical manifestations and have different suitable targeted therapies. This article aims to summarize the clinical characteristics and possible targets for therapies of monogenic lupus in children with different pathways， hoping to provide clues for clinicians to identify patients with monogenic lupus in the early stage and to provide individualized treatment as soon as possible.

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A study on the prognostic predictive value of plasma N-terminal pro-brain natriuretic peptide combined with hypersensitive troponin T in children with septic shock GUO Zhang-yan, DU Yan-qiang, WANG Yi 2023, 38(6): 464-469.  DOI: 10.19538/j.ek2023060612 Abstract ( )   Objective　To investigate the the correlation between plasma N-terminal pro-brain natriuretic peptide combined with hypersensitive troponin T and the prognosis of children with septic shock. Methods　The clinical data of 112 children with septic shock admitted to the Department of Pediatric Intensive Care Unit of  Children’s Hospital affiliated to Xi’an Jiaotong University from January 2018 to December 2020 were retrospectively analyzed. SPSS 20.0 statistical software was used for data analysis. Prognosis was used as the dependent variable， χ2 test was used for the constituent ratio of basic variables， binary Logistic regression was used to evaluate the prognosis of each variable， receiver operating characteristic curve （ROC） was used for specific prediction of variables with predictive value， and area under the curve （AUC） > 0.5 was used as predictive value. Results　A total of 112 children with septic shock were enrolled， and 55 of them died within 28 days， with a fatality rate of 49.1%. There were no significant differences between the survival group and the death group in gender， age， primary infection site， etiological characteristics or other baseline data （all P>0.05）. Compared with death group， there were no significant differences in leukocyte， blood glucose， procalcitonin， C-reactive protein or blood albumin in survival group （all P>0.05）. In the survival group， hemoglobin， platelet count， and left ventricular ejection fraction increased， but blood lactic acid， plasma natriuretic peptide precursor， high-sensitive troponin， creatine kinase isoenzyme， glutamate aminotran-sferase， aspartate aminotransferase， creatinine， blood urea nitrogen， prothrombin time， international normalized ratio， activated partial thrombin time， etc decreased， and the differences were statistically significant （all P<0.05）. Logistic regression analysis showed that blood lactic acid， plasma N-terminal pro-brain natriuretic peptide and hypersensitive troponin T were independent prognostic risk factors for septic shock in children.ROC curve analysis showed that blood lactic acid， plasma N-terminal pro-brain natriuretic peptide and hypersensitive troponin T all had predictive value for the prognosis of children with septic shock， and the area under the ROC curve （AUC） was 0.879， 0.930， and 0.703， respectively.The combination of the three had a sensitivity of 96.5%， a specificity of 85.5%， and an AUC of 0.96 in predicting the prognosis. Conclusion　Lactate， plasma N-terminal pro-brain natriuretic peptide，and hypersensitive troponin T are independent risk factors for children with septic shock， and the combination of the three has a good predictive value for the prognosis of children with septic shock.

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Comparative analysis of clinical characteristics of bronchitis obliterans and bronchiolitis obliterans LIU Chu, DAI Ji-hong, GENG Gang, et al 2023, 38(6): 470-476.  DOI: 10.19538/j.ek2023060613 Abstract ( )   Objective 　To analyze the clinical characteristics of bronchitis obliterans in children， and to discuss the differences between its clinical characteristics and those of bronchiolitis obliterans， so as to achieve early recognition and treatment of bronchitis obliterans. Methods　The clinical data of those children who were diagnosed with bronchitis obliterans and bronchiolitis obliterans in Children's Hospital Affiliated to Chongqing Medical University from January 2021 to December 2021 were collected. The patients were divided into simple bronchitis obliterans group ， simple bronchiolitis obliterans group and bronchiolitis obliterans combined with bronchiolitis obliterans group （referred to as combination group）. The clinical characteristics， laboratory examination， imaging examination results and treatment were compared. Results　The onset age of simple bronchitis obliterans group  was higher than that of the other two groups. The symptoms of wheezing， repeated wheezing， shortness of breath， activity intolerance， dyspnea and three concave signs in simple bronchiolitis obliterans group were significantly more obvious than those in simple bronchitis obliterans group ; the symptoms of wheezing， repeated wheezing， shortness of breath and activity intolerance in combination group were more obvious than those in simple bronchitis obliterans group ; the reduction of breath sound in simple bronchitis obliterans group  was more obvious than that in simple bronchiolitis obliterans group; The total number of cells， nuclei， neutrophils， lymphocytes， phagocytes and pleural effusion in alveolar lavage fluid of simple bronchitis obliterans group  were more than those of simple bronchiolitis obliterans group; the number of lymphocytes in lavage fluid of simple bronchitis obliterans group  was more than that of combination group; the degree of pulmonary dysfunction of simple bronchitis obliterans group  was less severe than that of the other two groups， and the use of invasive ventilator was less than that of the other two groups. Conclusion　The clinical manifestations of children with bronchitis obliterans are similar to those of bronchiolitis obliterans， but the manifestations of bronchitis obliterans are generally less severe than those of bronchiolitis obliterans， and the degree of pulmonary dysfunction is also less severe. Bronchoscopy and the total number of cells and neutrophils in alveolar lavage fluid may be clues for identification. Bronchoscopic lavage therapy and targeted and aggressive anti-infective therapy may be helpful in preventing further development of bronchial occlusion in these patients.

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A case report of lysineuria protein intolerance YI Ying-qing, ZHONG Li-li, WANG Zhen, et al 2023, 38(6): 477-480.  DOI: 10.19538/j.ek2023060614 Abstract ( )   A 6-month-old male child was admitted to hospital due to cough for more than 1 month. The child's growth and development lags behind that of children of the same age. Hepatosplenomegaly existed， and lactate dehydrogenase and alpha-fetoprotein were checked for multiple times and were significantly higher than normal values. Urine orotic acid and uracil slightly increased， and blood ammonia was normal. Chest CT showed interstitial pneumonia. Electron microscope of liver biopsy showed that lipid droplets of different sizes were deposited in some hepatocytes， and many pigment particles were deposited in some hepatocytes. Whole-exome sequencing revealed a compound heterozygous variation in the SLC7A7 gene， and the final diagnosis of lysinuric protein intolerance was made. The disease is a rare autosomal recessive disease. Early diagnosis and timely treatment of the disease can significantly improve the prognosis of children. However， because it often involves multiple systems and lacks specificity in clinical manifestations， it is easy to be missed or misdiagnosed.  Diagnosed cases of small infants are rare. This article summarizes the clinical characteristics， diagnosis and treatment of this patient， and by reviewing relevant literature， provides a reference for the early diagnosis of such disease and the prevention and treatment of  its complications.