中华儿科杂志最新文献

{"title":"[Analysis of clinical characteristics and prognosis of non-rhabdomyosarcoma soft tissue sarcomas in 72 children].","authors":"X X Liang, Y J Gao, J Zhao, J Ma, A A Zhang, M Wang, Q S Yang, Z Xu, Q Yuan, Y J Tang, M Z Yin, J Y Tang","doi":"10.3760/cma.j.cn112140-20260618-00495","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260618-00495","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the clinical characteristics, treatment, and prognosis factor of non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) in children. <b>Methods:</b> In this retrospective cohort study, clinical data (including age, sex, pathological type, surgical resection status, risk stratification, protocols, and survival time) of 81 children with pathologically confirmed NRSTS initially treated at Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine from January 2015 to December 2024 were collected. Survival rates was analyzed by risk group, resection completeness, and other factors. Old and new protocols (changed around 2021) were compared for survival rates. Survival analysis and univariate analysis were performed using the Kaplan-Meier method; intergroup comparison of survival rates was conducted using the Log-rank test and multivariate analysis was performed using the Cox proportional hazards regression model. <b>Results:</b> A total of 72 children with NRSTS were enrolled, including 38 males (53%) and 34 females (47%), with the age at initial diagnosis of 6.5 (3.7, 9.9) years. A total of 16 histological subtypes were identified, with synovial sarcoma being the most common (14 children (19%)). Of 47 children with genetic testing, 34 (72%) carried class1 pathogenic variants. Among all enrolled children, 21 children (29%) were stratified into low-risk group, 31 children (43%) into intermediate-risk group, and 20 children (28%) into high-risk group. With a follow-up time of 66.0 (28.3, 79.8)months, 23 events were observed, including 20 relapses or progressions, 2 second malignancies, and 1 treatment-related death; 12 deaths reported. The estimated 5-year events free survival (EFS) and overall survival (OS) rates were 66.5% (95%<i>CI</i> 55.1%-77.9%) and 85.0% (95%<i>CI</i> 76.4%-93.6%), respectively. The 5-year EFS and OS rates were 85.7% (95%<i>CI</i> 70.9%-100.0%) and 100.0% in the low-risk group, 58.2% (95%<i>CI</i> 39.8%-76.6%) and 84.8% (95%<i>CI</i> 70.7%-98.9%) in the intermediate-risk group, and 59.2% (95%<i>CI</i> 37.5%-80.9%) and 69.3% (95%<i>CI</i> 48.8%-89.8%) in the high-risk group. Prognostic analysis indicated that incomplete resection independently predicted poorer prognosis in children (EFS rate: <i>HR</i>=5.46, 95%<i>CI</i> 2.17-13.70, <i>P</i><0.001; OS rate: <i>HR</i>=10.17, 95%<i>CI</i> 2.93-35.29, <i>P</i><0.001). Among 12 low-risk children before 2021, 9 received postoperative chemoradiotherapy; among 9 children after 2021, 7 underwent observation after complete resection. The 3-year EFS and OS rates both had no statically difference between the two groups (both <i>P</i>>0.05). <b>Conclusions:</b> Surgery remains the cornerstone of treatment for pediatric NRSTS, and complete resection is the most important prognostic determinant. Most low-risk children can be cured without adjuvant chemotherapy or radiotherapy.</p>","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1225-1232"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186133","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Expert consensus on the clinical application of long-acting growth hormone (2026)].","authors":"","doi":"10.3760/cma.j.cn112140-20260817-00635","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260817-00635","url":null,"abstract":"","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1219-1224"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186502","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Two cases of type 4 severe congenital neutropenia combined with inflammatory bowel disease caused by compound heterozygous variants in G6PC3 gene].","authors":"Y M Zhang, X X Wei, M Shi, J J Xu, N Xue, B Wang, K H Zhang, L Yang, G H Liu","doi":"10.3760/cma.j.cn112140-20260414-00304","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260414-00304","url":null,"abstract":"","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1279-1282"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186585","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Clinical analysis of modified Chinese Children Leukemia Group-Acute Myeloid Leukemia 2019 regimen in treating pediatric acute myeloid leukemia].","authors":"W Fan, J J Li, L Zhang, Z X Zhang, Z L Song, R H Tang, M Yue, B H Zou, H H Qin, T Hu, J H Li, S Q Feng, L T Xuan, M Z Hu, D F Shao, X N Chen, M N Zhai, D Q Luo, R Liu","doi":"10.3760/cma.j.cn112140-20260828-00670","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260828-00670","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; To evaluate the clinical efficiency and prognosis of a modified regimen based on the Chinese Children Leukemia Group-Acute Myeloid Leukemia 2019 (CCLG-AML2019) protocol for childhood acute myeloid leukaemia (AML) (non-acute promyelocytic leukaemia, non-APL). &lt;b&gt;Methods:&lt;/b&gt; In this retrospective cohort study, clinical data including sex, age, French-American-British (FAB) classification, genetics, molecular biology, treatment, therapeutic efficacy and prognosis of 41 newly diagnosed non-APL AML children treated at Capital Institute of Pediatrics, Capital Pediatric Medical Center, Capital Medical University from November 2019 to December 2023 were analyzed. All children received chemotherapy according to the CCLG-AML2019 protocol. For high-risk (HR) children with persistent high-level minimal residual disease (MRD) after induction therapy, a cladribine-containing intensified salvage chemotherapy regimen was introduced in the third course. According to the risk stratification criteria, children were divided into high-risk and non-high-risk groups. High-risk children received allogeneic hematopoietic stem cell transplantation (allo-HSCT) after 3 or 4 courses of chemotherapy. Children were divided into two groups according to age (&lt;2 years and ≥2 years group), and the clinical characteristics and prognosis between the two groups were compared using Fisher's exact test. Kaplan-Meier method was used to estimate the 5-year overall survival (OS) rate and event-free survival (EFS) rate, and Cox regression was employed for univariate analysis. &lt;b&gt;Results:&lt;/b&gt; Among the 41 children, there were 22 males and 19 females, with an age of 5.8 (2.6, 8.0) years. Twenty-four children (59%) were classified as the high-risk group, and 17 (41%) as the non-high-risk group. The follow-up duration was 51(38, 62) months. The 5-year OS rate was (85±7)%, and the 5-year EFS rate was (79±7)%. Non-HR children achieved 100% for both, whereas in the high-risk group, the corresponding rates were (75±10)% and (65±11)%, respectively. The difference in EFS rate between the two groups had statistically difference (&lt;i&gt;P&lt;/i&gt;=0.018). There were 10 children in the &lt;2 years age group, among them FAB M5 or M7 subtypes were observed in 9 children, KMT2A gene rearrangements were observed in 5 children, and high-risk stratification were defined in 8 children. Core binding factor (CBF)-AML (19 children) had a KIT gene variation in 10 children, and with no relapse. During follow-up, 5 deaths occurred (2 due to relapse, 2 due to transplant-related complications, and 1 due to infection). &lt;b&gt;Conclusions:&lt;/b&gt; The CCLG-AML2019 regimen offers favorable short-term survival for pediatric non-APL AML, especially in non-HR children. HR children still face the risks of relapse and transplant-related mortality after allo-HSCT. Children aged &lt;2 years may benefit from chemotherapy combined with targeted therapy. CBF-AML has a favorable overall prognosis, and the high frequency of KIT g","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1233-1239"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186406","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Expert consensus on the use of thrombopoietin receptor agonists in pediatric primary immune thrombocytopenia (2026)].","authors":"","doi":"10.3760/cma.j.cn112140-20260525-00409","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260525-00409","url":null,"abstract":"","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1209-1218"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186490","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Advances in the effects of obesity on anti-tumor necrosis factor therapy in children with inflammatory bowel disease].","authors":"M Y Zhai, Y Y Luo","doi":"10.3760/cma.j.cn112140-20260703-00529","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260703-00529","url":null,"abstract":"","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1299-1302"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149185959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Clinical analysis of 4 children with tubulointerstitial nephritis and uveitis syndrome].","authors":"N N Wang, J Tu, C Y Chen","doi":"10.3760/cma.j.cn112140-20260711-00547","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260711-00547","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the clinical and pathological characteristics, treatment, and prognosis of tubulointerstitial nephritis and uveitis (TINU) syndrome in children. <b>Methods:</b> This case series study analyzed the clinical characteristics, laboratory examinations, renal biopsy findings, treatment courses, and outcomes of 4 children diagnosed with TINU syndrome, who were hospitalized in the Department of Nephrology, Capital Center for Children's Health affiliated to Capital Medical University between November 2017 and April 2024. <b>Results:</b> Four children with TINU syndrome included 2 males and 2 females. Their ages at diagnosis were 13 years and 11 months, 13 years and 6 months, 13 years and 7 months, and 11 years and 8 months respectively. Three patients had a history of infection or drug exposure before disease onset. Ocular symptoms preceded renal involvement by 1 month in 1 case, whereas uveitis developed 8.0, 0.5 and 14.0 months after renal involvement in the other 3 cases. All patients had acute kidney injury (AKI), including stage 2 AKI in 3 patients and stage 3 AKI in 1 patient. Glycosuria and elevated urinary β2-microglobulin levels occurred, and varying degrees of proteinuria and leukocyturia occurred in 3 patients. Serum creatinine levels at disease onset were markedly elevated at 130.0, 103.4, 188.4 and 105.7 μmol/L, and estimated glomerular filtration rates were reduced to 50.5, 56.4, 28.9 and 53.8 ml/(min·1.73 m²). Renal biopsies showed acute interstitial nephritis, and the predominant ocular manifestation was bilateral anterior uveitis. All patients received systemic glucocorticoid therapy and topical corticosteroid eye drops, and 1 patient was additionally treated with mycophenolate mofetil. During follow-up periods of 57, 82, 14 and 66 months, respectively, 1 patient progressed to stage 2 chronic kidney disease. Proteinuria resolved and ocular symptoms recurred only in 1 patient. <b>Conclusions:</b> Renal involvement generally has a favorable prognosis in children with TINU syndrome. However renal function may not fully recover in some patients, and uveitis may persist or recur, underscoring the need for renal and ophthalmic combined screening and long-term multidisciplinary follow-up.</p>","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1274-1278"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186321","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Clinical analysis of 5 cases of pediatric respiratory viral sepsis].","authors":"Y Long, T Y Yuan, L Zhao, J Xu, R Y Niu, H M Fu","doi":"10.3760/cma.j.cn112140-20260702-00524","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260702-00524","url":null,"abstract":"<p><p><b>Objective:</b> To analyze the clinical characteristics of pediatric respiratory viral sepsis and to improve clinicians' awareness of this disorder. <b>Methods:</b> Case series study. The study retrospectively reviewed the clinical data of 5 children with respiratory viral sepsis treated at Department of Pulmonary and Critical Care Medicine, Kunming children's hospital between March 2023 and January 2026, analyzing their clinical manifestations, laboratory findings, treatment courses, and outcomes. <b>Results:</b> Among the 5 cases of respiratory viral sepsis, there were 2 males and 3 females, and the ages were 3 years and 8 months, 4 years, 4 years and 7 months, 5 years and 2 months, and 8 years and 10 months respectively. All 5 cases had fever, 3 cases had cough, 2 cases had breathing difficulties and 1 each case of skin mottling, thrombocytopenia, and convulsions and consciousness disorder. Laboratory tests showed that the white blood cell count was (2.06-11.86)×10⁹/L, platelet count (26-262)×10⁹/L, C-reactive protein 0.62-68.15 mg/L, procalcitonin 0.09-0.35 μg/L and interleukin-6 0.50-19.64 ng/L. Pathogen examination showed 3 cases of influenza A virus infection, 1 case of adenovirus infection, and 1 case of adenovirus combined with parainfluenza virus infection. All 5 patients recovered and were discharged after comprehensive treatment including antiviral therapy, respiratory support, and immunomodulation, with a hospital stay of 8-59 d. <b>Conclusions:</b> Respiratory viral infections can cause multiple organ dysfunction in children and meet the diagnostic criteria for sepsis. Greater clinical attention should be paid to the diagnosis, recognition, and management of viral sepsis in children.</p>","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1270-1273"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Pathogenesis and treatment advances of CBFA2T3::GLIS2 fusion gene-driven acute megakaryoblastic leukemia].","authors":"J J Zhao, S Y Hu","doi":"10.3760/cma.j.cn112140-20260616-00486","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260616-00486","url":null,"abstract":"","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1288-1291"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186514","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Clinical analysis of 21 children with Xp11.2 translocation renal cell carcinoma].","authors":"Q H Yao, H H Li, H M Sun, Y F Liu, D Wang","doi":"10.3760/cma.j.cn112140-20260623-00502","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260623-00502","url":null,"abstract":"&lt;p&gt;&lt;p&gt;&lt;b&gt;Objective:&lt;/b&gt; To summary the clinical characteristics, treatment and prognosis of children with Xp11.2 translocation renal cell carcinoma. &lt;b&gt;Methods:&lt;/b&gt; In this case-series study, clinical data including sex, age, clinical manifestations, staging, treatment modalities, and follow-up outcomes of 21 children with Xp11.2 translocation renal cell carcinoma who first visit and received treatment and follow-up at the First Affiliated Hospital of Zhengzhou University from June 2019 to June 2025 were collected. The Kaplan-Meier method was used for survival analysis, and the Log-rank test was applied for univariate prognostic analysis. &lt;b&gt;Results:&lt;/b&gt; Among the 21 children, 12 were males, 9 were females. The age at diagnosis was 15 (12, 16) years. Gross hematuria and flank pain were the main frequent symptoms, which occurred on 10 and 5 children respectively. According to the American Joint Committee on Cancer (AJCC) staging system, there were 7 children of stage Ⅰ, 2 children of stage Ⅱ, 9 children of stage Ⅲ, 3 children of stage Ⅳ. Children with stage Ⅰ-Ⅱ received surgical treatment only. With follow-up time of 19 (8, 52) months, none experienced disease recurrence. The 3-year event-free survival (EFS) and overall survival (OS) rates were both 100%. Among 9 children with stage Ⅲ, 4 received surgery alone, 2 of whom experienced recurrence; 5 received surgery plus tyrosine kinase inhibitor (TKI), 1 of whom developed recurrence. Among 3 children of stage Ⅳ, 1 had extensive metastasis and was treated with sunitinib only, subsequently progressed and died; 1 received surgery plus sunitinib and later developed disease progression; 1 achieved partial remission after surgery combined with programmed death-1 (PD-1) inhibitors and axitinib therapy. The progression or recurrence time was 7.0(6.5, 9.5) months. Among the 5 relapsed or progressive children, 1 child died; 4 children were treated with PD-1 inhibitor combined with axitinib, with 3 achieving complete remission and 1 obtaining stable disease. The follow-up duration for stage Ⅲ-Ⅳ children was 30.0 (12.8, 44.5) months, and the 3-year EFS and OS rates were 55% (95%&lt;i&gt;CI&lt;/i&gt; 23%-78%) and 91% (95%&lt;i&gt;CI&lt;/i&gt; 49%-97%), respectively. The follow-up duration for all 21 children was 28 (11, 44) months, with 3-year EFS and OS rates of 73% (95%&lt;i&gt;CI&lt;/i&gt; 38%-91%) and 94% (95%&lt;i&gt;CI&lt;/i&gt; 67%-99%), respectively. AJCC stage Ⅲ-Ⅳ and the presence of regional lymph node metastasis were both associated with disease progression and recurrence (both &lt;i&gt;P&lt;/i&gt;&lt;0.05). &lt;b&gt;Conclusions:&lt;/b&gt; Children of Xp11.2 tRCC predominantly presents primarily with gross hematuria. Children with stage Ⅰ-Ⅱ can be treated with surgery alone, while those with stage Ⅲ-Ⅳ can receive surgical resection combined with tyrosine kinase inhibitor therapy. The overall prognosis is favorable. AJCC stage Ⅲ-Ⅳ and the presence of regional lymph node metastasis are poor prognostic factors. PD-1 inhibitor combined with axitinib therapy can be used for the t","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 11","pages":"1240-1244"},"PeriodicalIF":0.0,"publicationDate":"2026-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186370","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}