中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn112140-20260311-00202

引用次数: 0

[A case of juvenile dermatomyositis complicated by T-lymphoblastic lymphoma]. [青少年皮肌炎合并t淋巴母细胞淋巴瘤1例]。

中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn112140-20260301-00168

N N Li, X L Zhao, S Q Li, S B Li, X G Wang, Y J Zhou, W T Zang, X J Yao, T Hao, L L Song, X Wang, S D Fan, Y X Cui

引用次数: 0

[Clinical efficacy and safety of lonafarnib in children with Hutchinson-Gilford progeria syndrome]. [lonafarnib治疗儿童Hutchinson-Gilford早衰综合征的疗效及安全性]。

中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn112140-20260420-00332

Y Huang, J J Wang, C J Wang, X X Tang, Q M Yu, H D Fu, L D Hu, J H Mao

{"title":"[Clinical efficacy and safety of lonafarnib in children with Hutchinson-Gilford progeria syndrome].","authors":"Y Huang, J J Wang, C J Wang, X X Tang, Q M Yu, H D Fu, L D Hu, J H Mao","doi":"10.3760/cma.j.cn112140-20260420-00332","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260420-00332","url":null,"abstract":"Objective: To evaluate the efficacy and safety of lonafarnib, in children with Hutchinson-Gilford progeria syndrome(HGPS). Methods: This was a prospective cohort study. A total of 12 children with HGPS who were treated with lonafarnib at the Children's Hospital of Zhejiang University School of Medicine between April 2022 and November 2025 were enrolled. Clinical data collected within 1 week before initial medication were taken as the baseline. The first follow-up was performed 4 to 12 months after treatment initiation, with complete laboratory tests and imaging examinations collected. The follow-up period ended in March 2026. Paired-samples t test and Wilcoxon signed-rank test were used to compare indicators including height, weight, blood lipid levels, pulse wave velocity, and areal bone mineral density before and after treatment. Results: Among the 12 children, 6 were boys and 6 girls. The age at diagnosis was 0.8 (0.3, 4.8) years, including 10 cases of classic HGPS and 2 cases of non-classic HGPS. The follow-up duration was (7±2) months. Two patients were lost to follow-up. In the 10 children who completed follow-up, height increased significantly from baseline (93 (83, 102) vs. 97 (85, 103) cm, Z=2.81, P=0.002), while no significant change was observed in height-for-age Z-score (Z=0.46, P=0.682). Low-density lipoprotein decreased significantly (2.8 (2.3, 3.5)vs. 2.5 (2.1, 2.8) mmol/L, Z=2.13, P=0.031). No statistically significant change was found in weight, liver and renal function, homeostasis model assessment of insulin resistance, pulse wave velocity, carotid intima-media thickness and areal bone mineral density before and after treatment (all P>0.05). All children experienced gastrointestinal adverse reactions of varying degrees during treatment, which were predominantly mild to moderate and relieved after dose adjustment. One child discontinued treatment due to drug allergy. Conclusions: Lonafarnib demonstrated an overall favorable safety and tolerability profile. Early gastrointestinal adverse events were mostly reversible and manageable, with no significant drug-related hepatic or renal impairment observed. Short-term follow-up showed that only low-density lipoprotein improved significantly, with no clear evidence of growth benefit, and no progressive aggravation of vascular or skeletal lesions was detected.","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 9","pages":"1009-1014"},"PeriodicalIF":0.0,"publicationDate":"2026-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148765658","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}

引用次数: 0

[Efficacy and safety of obinutuzumab in children with refractory nephrotic syndrome]. [obinutuzumab治疗难治性肾病综合征儿童的疗效和安全性]。

中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn112140-20260608-00451

X Y Liu, M Z G Zhuoga, W Bai, B G Su, K Xu, X H Zhong, F Wang, H J Xiao

{"title":"[Efficacy and safety of obinutuzumab in children with refractory nephrotic syndrome].","authors":"X Y Liu, M Z G Zhuoga, W Bai, B G Su, K Xu, X H Zhong, F Wang, H J Xiao","doi":"10.3760/cma.j.cn112140-20260608-00451","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260608-00451","url":null,"abstract":"Objective: To evaluate the efficacy and safety of obinutuzumab (OBZ) in children with refractory nephrotic syndrome (RNS) who have a poor response to rituximab (RTX) therapy. Methods: This retrospective cohort study enrolled children with primary RNS who received OBZ because of poor response to RTX at the Children's Medical Center of Peking University First Hospital from July 2024 to December 2025. For each patient, outcomes during the OBZ treatment phase were compared with those during their preceding RTX treatment phase. Comparative parameters included duration of sustained B-cell depletion, duration of urinary protein remission, tapering of glucocorticoids and immunosuppressants, relapse frequency, and adverse events. Paired t-test, Wilcoxon signed-rank test, Mann-Whitney U test, χ2 test and Fisher's exact test were adopted for statistical analysis according to the data type and distribution characteristics. Results: Thirteen children (10 males, 3 females) were enrolled. The age at disease onset was (47±13) months, and the age at first OBZ administration was (121±35) months. There were 3 cases of steroid-resistant and 10 cases of steroid-sensitive but frequently relapsing nephrotic syndrome. Compared with the RTX phase, the OBZ phase was associated with significantly longer durations of sustained B-cell depletion, sustained urinary protein remission, and annual glucocorticoid and calcineurin inhibitor (CNI) withdrawal (all P<0.05). The annual relapse frequency was significantly lower during OBZ treatment than during RTX treatment (0 (0, 0.3) vs. 1.9 (1.4, 2.6), Z=3.06, P=0.002). Regarding safety, 2 children developed mild transient infusion reactions (gastrointestinal discomfort and elevated blood pressure), which resolved with reduced infusion rate; 1 child discontinued OBZ due to concurrent pneumonia and otitis media; and 7 developed asymptomatic hypogammaglobulinemia. No severe adverse events, such as severe neutropenia, thrombocytopenia or organ dysfunction, were documented. Conclusions: For children with RNS who experience short B-cell depletion duration and frequent relapse after RTX therapy, OBZ provides more durable B-cell depletion, effectively prolongs urinary protein remission, reduces relapse risk, and facilitates safer glucocorticoid tapering. These findings suggest that OBZ may serve as a viable alternative therapeutic option for this specific pediatric population.","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 9","pages":"1015-1019"},"PeriodicalIF":0.0,"publicationDate":"2026-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148762899","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}

引用次数: 0

[Progress in the diagnosis and management of hereditary nephrocalcinosis and nephrolithiasis]. [遗传性肾钙质沉着症和肾结石的诊断和治疗进展]。

中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn112140-20260518-00393

W X Liang, Y L Kang

引用次数: 0

[Practice guidelines for managing transition from adolescents to adult hood in children with inflammatory bowel disease (2026)]. [炎性肠病儿童从青少年到成年的管理实践指南（2026）]。

中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn112140-20260403-00270

引用次数: 0

[Integration and innovation of compassion and skill in modern pediatric nephrology]. 【现代儿科肾病学爱心与技能的融合与创新】。

中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn112140-20260615-00477

Q Li

引用次数: 0

[Special clue for identifying primary immunodeficiency eosinophilia]. 【鉴别原发性免疫缺陷嗜酸性粒细胞增多症的特殊线索】。

中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn11112140-20260130-00095

L M Huang, R X Dai, X D Zhao, Y F An

引用次数: 0

[Analysis of the growth trajectories of low birth weight children aged 0 to 4 years based on the superimposition by translation and rotation model]. [基于平移旋转叠加模型的0 ~ 4岁低出生体重儿生长轨迹分析]。

中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn112140-20260405-00272

B X Zhang, Y Hu, J Y Cui, J Cui, Z J Cui

{"title":"[Analysis of the growth trajectories of low birth weight children aged 0 to 4 years based on the superimposition by translation and rotation model].","authors":"B X Zhang, Y Hu, J Y Cui, J Cui, Z J Cui","doi":"10.3760/cma.j.cn112140-20260405-00272","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20260405-00272","url":null,"abstract":"Objective: To analyze the growth trajectories of low birth weight children aged 0-4 years in different intrauterine growth status. Methods: A retrospective cohort study was conducted from February 2025 to January 2026 whioh, included 430 low birth weight children who were followed up aged 0-4 years at 15 community health service centers in Xicheng District of Beijing, from January 2018 to December 2022 and had ≥3 follow-up measurements. Children were categorized by gestational age into preterm and term groups, and by intrauterine growth status into small-for-gestational-age (SGA) and appropriate-for-gestational-age (AGA) groups. Growth trajectories for weight and length (height) were fitted using the superimposition by translation and rotation (SITAR) model based on longitudinal data, and catch-up growth was evaluated using an individual-level cluster resampling method. Results: After excluding 7 preterm SGA children, 423 low birth weight children were included for analysis. Among them, there were 282 children in the preterm SGA group, 74 children in the term AGA group, 67 children in term SGA group, and 218 (51.5%) males. The gestational age of 423 children was (34.9±2.8) weeks, the birth weight was (2.1±0.3) kg,the birth length was (45.2±3.2) cm. There were 6 (4, 7) follow-ups within 4 years of age. Based on the SITAR model fitting results, the weight and height (length) of low birth weight children generally caught up to the P3 within 2 months of age; the weight reached the P25 at 6.4 months of age in boys and 5.7 months of age in girls, the deceleration of catch-up growth occurred at 19.3 and 14.7 months of age; the height (length) reached the P25 at 10.7 months of age in boys and 9.8 months of age in girls, the deceleration of catch-up growth occurred at 27.7 and 28.3 months of age; stabilizing at the P25-P50 by 48 months of age. The growth rate and trajectory of the preterm AGA group and the term AGA group were similar, the weight reached the P25 at 6.0 and 5.2 months of age in boys and girls, respectively, 6.1 and 6.6 months of age, respectively. Subsequently, the growth rate of the preterm AGA group exceeded that of the term AGA group. Moreover, the height (length) of the preterm AGA group reached the P25 earlier than that of the term AGA group (9.0, 6.4 and 16.1, 9.9 months of age). In the term AGA group and term SGA group, the growth rate and trajectory were similar within the age range of 2.9 to 6.6 months for weight and 4.5 to 16.1 months for length. The age at deceleration of length catch-up growth was earlier in SGA children (25.5 months of age in boys, 30.0 months of age in girls) than in term AGA children (whose length percentile remained below P25 at even 48 months of age). Conclusion: The peaks of catch-up growth for weight and length in low birth weight","PeriodicalId":60813,"journal":{"name":"中华儿科杂志","volume":"64 9","pages":"1020-1026"},"PeriodicalIF":0.0,"publicationDate":"2026-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148765376","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}

引用次数: 0

[A case of giggle incontinence comorbid with primary nocturnal enuresis]. [笑声失禁并发原发性夜间遗尿1例]。

中华儿科杂志 Pub Date : 2026-08-17 DOI: 10.3760/cma.j.cn112140-20260319-00223

X R Tan, J Ma

引用次数: 0

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