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06 September 2025, Volume 40 Issue 9 Previous Issue   

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Attention to standardized screening，evaluation， and management of neurodevelopmental delays and disorders associated with congenital heart disease：interpretation of the 2024 AHA statement LI Chun-chun, ZHANG Chen-mei, HE Wei-mei, et al 2025, 40(9): 705-713.  DOI: 10.19538/j.ek2025090601 Abstract ( )   Neurodevelopmental delays or disorders associated with congenital heart disease （CHD） have become a global challenge；however， the concerns about them remain limited in China. The current healthcare system primarily focuses on surgical treatment for CHD， often overlooking the importance of long-term postoperative follow-up and neurodevelopmental management. This interpretation summarizes the latest advancements in the field of CHD，systematically elaborating on neuroprotection strategies and risk stratification methods， and aiming to optimize clinical management and improve neurodevelopmental outcomes of children with CHD in China.

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Interpretation of the guidelines for the diagnosis and treatment of systemic sclerosis by EULAR ZHANG Cai-hui, SONG Hong-mei 2025, 40(9): 714-719.  DOI: 10.19538/j.ek2025090602 Abstract ( )   To improve the treatment and long-term management of systemic sclerosis（SSc），the European Alliance of Associations for Rheumatology（EULAR） released updated SSc treatment guidelines in September 2024，following the 2017 guidelines. These updated guidelines continue the previous organ-targeted treatment strategies and introduce several key recommendations across eight major domains，including Raynaud’s phenomenon，digital ulcers，and pulmonary arterial hypertension. Most of the new recommendations are related to skin fibrosis and interstitial lung disease（ILD）. This article provides an interpretation of the main content of the guidelines for clinical reference.

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Improving the diagnosis and treatment level of rheumatic and autoimmune diseases in children MA Ming-sheng, ZHOU Yu, SONG Hong-mei 2025, 40(9): 720-723.  DOI: 10.19538/j.ek2025090603 Abstract ( )   Remarkable progress has been made in diagnosis and treatment level of rheumatic and autoimmune diseases in children in recent years. On the diagnostic front， the advent of biomarker-driven disease stratification has revolutionized patient management by enabling early identification of high-risk individuals and facilitating personalized treatment plans. The expanding clinical application of genetic sequencing has particularly enhanced our comprehension of monogenic lupus and other prevalent childhood autoimmune disorders. Concurrently， diagnostic classification systems are undergoing continuous refinement to keep pace with the precision medicine paradigm shift.Therapeutic advancements have been equally transformative. Early， standardized intervention has emerged as a critical factor in preventing irreversible organ damage and optimizing long-term prognosis. Modern treatment strategies now feature increasingly targeted immunosuppressive regimens， while the accelerated development of biologic agents has substantially expanded the therapeutic arsenal for pediatric rheumatic diseases. Particularly promising are novel cellular therapies that show great potential in this evolving landscape.These collective developments underscore the growing importance of molecular-guided stratified diagnosis and tailored therapeutic interventions as fundamental components in elevating the standard of care for pediatric rheumatic and autoimmune disorders.

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Renewed understanding of the diagnosis and treatment guidelines for juvenile dermatomyositis in China HE Ting-yan, YANG Jun 2025, 40(9): 724-728.  DOI: 10.19538/j.ek2025090604 Abstract ( )   Juvenile Dermatomyositis （JDM） is a chronic autoimmune disease primarily characterized by skin and muscle inflammation， requiring individualized treatment based on disease severity and major organ involved. For non-severe cases， a combination of conventional-dose glucocorticoids with methotrexate serves as the first-line therapy. Methylprednisolone pulse therapy is indicated for severe cases. Recent studies have progressively confirmed the efficacy of intravenous immunoglobulin （IVIG） in JDM. Biological agents and JAK inhibitors may be selectively used for refractory cases， while CAR-T therapy also holds therapeutic promise for these refractory patients.

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Classification diagnosis and treatment management of systemic juvenile idiopathic arthritis LUO Xi-wen, TANG Xue-mei 2025, 40(9): 728-734.  DOI: 10.19538/j.ek2025090605 Abstract ( )   Systemic juvenile idiopathic arthritis（sJIA）has a unique disease background and clinical profile，with various extra-articular manifestations and disease patterns. Its etiology remains unclear. The interaction between genetic and environmental factors contributes to the onset and progression of the disease. Inflammation control and targeted therapies are key to achieving disease remission. This review outlines the pathogenesis of sJIA，classification criteria，current research advances in diagnosis and treatment，and the main global principles for targeted therapeutic management，highlighting the importance of early diagnosis，precise intervention，and long-term follow-up in improving patient outcomes and quality of life，which helps to deepen clinicians’ overall understanding of sJIA.

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Diagnosis and management of chronic nonbacterial osteomyelitis in children MA Le, FAN Zhi-dan, HUANG Na, et al 2025, 40(9): 734-740.  DOI: 10.19538/j.ek2025090606 Abstract ( )   Chronic nonbacterial osteomyelitis is a rare autoinf-lammatory bone disease characterized by recurrent nonbacterial osteomyelitis. The lesions mainly affect long bones，spine，and anterior chest wall，presenting as nocturnal bone pain caused by single or multiple bone marrow inflammations. The disease progresses chronically and insidiously，with significant heterogeneity in clinical manifestations. Delayed treatment can lead to irreversible bone destruction. This article reviews and summarizes the disease from aspects such as pathogenesis，clinical manifestations，diagnostic criteria，differential diagnosis，and treatment strategies，aiming to improve pediatricians' understanding of the disease and provide a theoretical basis for standardized diagnosis and treatment.

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Diagnosis and treatment of childhood anti-neutrophil cytoplasmic antibody-associated vasculitis YANG Qin, WANG Mo 2025, 40(9): 740-746.  DOI: 10.19538/j.ek2025090607 Abstract ( )   Anti-neutrophil cytoplasmic antibody（ANCA）-associated vasculitis（AAV） is an autoimmune disease characterized by small-vessel inflammation，which can involve multiple organs throughout the body. The condition is extremely dangerous. With the in-depth research on pediatric AAV，more evidence has been provided for clinical guidance in the diagnosis and treatment of AAV. This article briefly elaborates on the diagnosis and treatment of pediatric AAV.

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Diagnosis and treatment of childhood-onset systemic lupus erythematosus ZHANG Yu-di, SHEN Tian, WU Xiao-chuan 2025, 40(9): 746-751.  DOI: 10.19538/j.ek2025090608 Abstract ( )   Childhood-onset systemic lupus erythematosus（cSLE） is a chronic autoimmune disease characterized by acuter onset，severer clinical manifestations，and poorer prognosis than those of adult-onset SLE. Early diagnosis and standardized treatment are crucial for improving outcomes. Currently，the diagnosis primarily relies on the 2012 SLICC and 2019 EULAR/ACR criteria，which were developed based on adult populations. Although these criteria perform well in the diagnosis of cSLE，they still have limitations in clinical practice. In the treatment a "treat-to-target" goal is emphasized and individualized treatment is conducted. Conventional therapy primarily consists of glucocorticoids combined with immunosuppressants，while the use of biologics can help reduce steroid use，lower disease activity，and decrease disease recurrences. The 2024 ACR guidelines recommend multitarget combination therapy for proliferative lupus nephritis，which shows promising results in adult patients. However，its actual efficacy and long-term safety in pediatric populations still require further validation.

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Diagnosis and treatment of children with Takayasu arteritis XU Meng, YU Guo-chen, ZHAO Xin-yuan, et al 2025, 40(9): 751-755.  DOI: 10.19538/j.ek2025090609 Abstract ( )   Takayasu arteritis is the most common large vessel vasculitis during childhood. In comparison to the diagnosis，the treatment for pediatric TAK is more challenging，and treatment strategy often refers to adult guidelines. In recent years，several academic organizations have updated their recommendations on the diagnosis and treatment of TAK，and the status of targeted drugs has gradually become more prominent，with a tendency from "salvage treatment" to "early intervention". Based on the recommendations of these guidelines/consensuses and recent studies，this article summarizes and sorts out the diagnosis and treatment of pediatric TAK in order to provide references for pediatricians' clinical decision-making；meanwhile，it is expected to conduct more researches on childhood TAK to improve the status of insufficient evidence-based evidence.

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Study on the relationship between obstructive sleep apnea and allergic rhinitis in children PU Jian, WANG Qian, LU Ying-xia, et al 2025, 40(9): 756-761.  DOI: 10.19538/j.ek2025090610 Abstract ( )   Objective　To investigate the relationship between obstructive sleep apnea（OSA） and allergic rhinitis（AR） in children，and to analyze the associated factors. Methods　A retrospective analysis was conducted on the polysomnography（PSG） data，sleep questionnaire responses，and clinical records of 1，236 pediatric patients who underwent PSG at the Sleep Medicine Centre of the Children's Hospital affiliated to the Capital Institute of Pediatrics from January 2020 to May 2023. Patients included in the study had an apnea-hypopnea index（AHI）≥1 and complete questionnaires. Exclusion criteria comprised a history of asthma，neurological and muscular diseases，craniofacial anomalies，and previous adenoid tonsillectomy. Ultimately，1，137 patients with OSA aged 0-17 years were included. These patients were categorized into mild，moderate，and severe OSA groups based on their condition，and were further divided into AR and no-AR groups according to questionnaire and clinical information. The basic characteristics and PSG data of the two groups were compared，and Logistic regression was used to analyze the risk factors for OSA aggravation，R software（version 4.2.3） employed for statistical analysis. Results　Of the 1，137 OSA patients，686 were male and 451 were female，with 476（41.9%） diagnosed with AR. The mean age of the AR group was 5.46 years，and their BMI was 16.80，which were 5.77 years and17.20 in the no-AR group；these differences were not statistically significant. The AR group had a mean minimum oxygen saturation of 0.89，which was higher than the 0.88 observed in the no-AR group（P<0.05）. Additionally，the microarousal index averaged 0.85 events/h in the AR group，lower than the 1.01 events/h in the no-AR group（P<0.05）. No significant differences were found in the distribution of AR and no-AR groups across different OSA severity levels，and there was no significant correlation between moderate to severe OSA and the presence of AR. Conclusion　The prevalence of AR is notably high among pediatric OSA patients. Furthermore，PSG parameters，specifically minimum oxygen saturation and microarousal index，are more favorable in patients with AR compared to those without AR.

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Clinical analysis of 53 cases of tracheal stenosis in children SUN Qiu-feng, LIN Li-hua, CHEN Li-li, et al 2025, 40(9): 762-766.  DOI: 10.19538/j.ek2025090611 Abstract ( )   Objective　To analyze the clinical characteristics of tracheal stenosis in pediatric patients and compare the clinical features between congenital and secondary tracheal stenosis. Methods　A retrospective analysis was conducted on clinical data of pediatric inpatients diagnosed with tracheal stenosis through bronchoscopy at the Department of Pediatrics，First Affiliated Hospital of Xiamen University from January 1，2012，to December 31，2021. Statistical analysis was performed using SPSS 28.0 software. Results　A total of 53 cases were included（30 males，23 females）with a median age of 8.7 months. Children under 1 year of age accounted for 72.2% of cases. Chronic respiratory symptoms were the predominant manifestation in 60% of patients， including chronic cough，stridor，laryngeal wheezing，and recurrent wheezing. Among the 53 cases，25 were diagnosed with congenital tracheal stenosis（CTS），all of which showed complete tracheal rings affecting various tracheal segments. Twenty-eight cases were secondary tracheal stenosis（STS），with 24 cases caused by cardiovascular compression，primarily from the aortic system，and all were external compression stenosis of the middle and lower trachea. Two cases presented with endotracheal membranous formation，resulting in luminal stenosis. Concomitant airway malformations were more prevalent in CTS compared to STS，with a significantly higher tracheal-bronchial ratio than STS. Fifty-two patients showed improvement after conservative medical management，including anti-infection therapy and airway management，and were discharged. Conclusion　Tracheal stenosis predominantly affects infants under one year of age，typically presenting with chronic respiratory symptoms. Chest CT combined with bronchoscopy can accurately evaluate pediatric tracheal stenosis. Clinical manifestations differ between congenital and secondary tracheal stenosis.

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Research progress in the treatment of pediatric invasive fungal infections HAN Hui-ling, WANG Yi 2025, 40(9): 767-772.  DOI: 10.19538/j.ek2025090612 Abstract ( )   Invasive fungal infection（IFI） is a fungal infection that invades deep tissues and organs as well as the whole body. Premature infants，newborns，and children with severe primary diseases are at high risk of invasive fungal infections. In recent years，with the increasing number of children with IFI，the increasing difficulty of pediatric IFI diagnosis，and the emergence of fungal resistance，the mortality rate has increased significantly. Pediatric IFI patients have low immunity，high complexity of drug use，atypical infection symptoms，and difficulty in early diagnosis，which increase the difficulty of clinical diagnosis and treatment in children with IFI. This article reviews the challenges faced by pediatric IFI in the treatment and the research progress in new drugs and new diagnostic and therapeutic methods，which helps to increase the attention to pediatric IFI patients，thus providing a reference for the prevention of high-risk children，the early diagnosis and treatment of pediatric IFI children，and the research direction of new drugs and new methods.  Keyword

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Research progress on risk factors and comorbidity mechanisms of pediatric allergic airway diseases and tic disorders SHEN Wen-xin, YU Si-jia, SHAN Li-shen 2025, 40(9): 773-778.  DOI: 10.19538/j.ek2025090613 Abstract ( )   Pediatric allergic airway diseases and tic disorders，as chronic conditions，are increasingly prevalent，which not only affect the quality of life but also impose substantial economic and social burdens. Clinical studies have revealed a significant correlation between tic disorders and allergic diseases，both of which are associated with immune system imbalances. The symptoms involve various parts or organs，and the pathogenesis is complex，severely impacting the quality of life. Potential factors influencing the development of these diseases include maternal depression and anxiety，maternal allergy history，maternal tobacco exposure history，sleep disorders，and environmental pollution. Furthermore，gut microbiome dysbiosis and T-cell immune imbalance may be potential factors underlying the comorbidity of the two conditions. Exploring and understanding these shared risk factors and comorbidity mechanisms increases the cognition about these two diseases，which is of great significance for the prevention and treatment of allergic airway diseases and  tic disorders.

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Plasmapheresis in the treatment of toxic epidermal necrolysis in children： report on 3 cases YE Feng, LIANG Yu-feng, TAO Jian-ping, et al 2025, 40(9): 779-783.  DOI: 10.19538/j.ek2025090614 Abstract ( )   From January 2022 to December 2023，the Pediatric Intensive Care Unit（PICU） of the Women and Children's Medical Center Affiliated to Guangzhou Medical University admitted 3 children with toxic epidermal necrolysis（TEN），aged 4，8 and 13 respectively. The areas of skin lesion were 50%，70% and 90% respectively，and the involved sites were the eyes，nasal cavity，oral cavity，external genitalia，urethral orifice and perianal mucosa respectively. The SCORTEN score and the in-hospital mortality rate assessment were 2/12%，2/12%，and 3/32%，respectively. After ineffective treatment with glucocorticoids and intravenous immunoglobulin（IVIG），2 to 5 sessions（1.5 to 2.0 times the plasma volume each time） of plasma exchange（PE） treatment were given respectively，with an interval of 48 hours between each two sessions. After treatment，the skin lesions of all three cases improved significantly，and the inflammatory factors decreased significantly，while no ocular complications such as visual field defects occurred. The article holds that PE may be an effective treatment method for children with TEN.

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Efficacy and safety analysis of 9-year combined drug therapy for a case of familial male-limited precocious puberty CHEN Meng, YAO Hui, LAN Tian 2025, 40(9): 784-787.  DOI: 10.19538/j.ek2025090615 Abstract ( )   Currently，there are few reports on the long-term efficacy in familial male-limited precocious puberty（FMPP） in China. This article reports the clinical characteristics，genetic testing result，and the treatment effects and safety during a 9-year follow-up of a child with FMPP caused by an activating mutation of the LHCGR gene. The male patient presented with penis enlargement and accelerated growth at one year and six months old. Based on his serum testosterone level，LHRH stimulation test result，bone age and other auxiliary examinations，he was initially diagnosed with peripheral precocious puberty. Genetic detection revealed a missense mutation of the LHCGR gene exon11 in the patient and his mother，resulting in the mutation of amino acid 532 from leucine to arginine（p.L532A，a suspected new mutation site），and the boy was finally diagnosed with FMPP. After combined treatment with letrozole，medroxyprogesterone，and triptorelin acetate for more than 9 years，the child achieved a satisfactory final height without any obvious adverse reactions. This study accumulates experience in the diagnosis and treatment of FMPP and expands the mutation spectrum of the LHCGR gene.

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Central precocious puberty with vasculitis-related moyamoya syndrome：one case report XIE Dan-dan, CHEN Qiu-li, MA Hua-mei, et al 2025, 40(9): 788-791.  DOI: 10.19538/j.ek2025090616 Abstract ( )   A 3-year-old and 1-month-old female child visited the Department of Pediatric Endocrinology of the First Affiliated Hospital of Sun Yat-sen University in November 2020 due to“breast development with accelerated growth for 1.5 years”. After examination，she was diagnosed with central precocious puberty（CPP）. After admission，pituitary magnetic resonance imaging（MRI），magnetic resonance angiography（MRA），magnetic resonance spectroscopy（MRS），diffusion-weighted imaging（DWI）+susceptibility weighted imaging（SWI） and other examinations were performed，and the diagnosis was vasculitis-related smoke syndrome. Due to poor control of bone age（BA） in the child after treatment with gonadotropin-releasing hormone analog（GnRHa），17-hydroxyprogesterone（17OHP） was checked. The level of 17OHP was elevated，and the adrenocorticotrophic hormone（ACTH） stimulation test was performed，which indicated the possibility of non-classical congenital adrenal hyperplasia（NCCAH）. This case of CPP accompanied by vasculitisassociated smoke syndrome is rare，suggesting that vasculitis-associated smoke syndrome can also be a cause of CPP.