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[Clinical characteristics and risk factors for bronchoscopic airway mucus hypersecretion in childhood pneumonia infected by different pathogens]. [不同病原菌感染的儿童肺炎经支气管镜检查气道粘液高分泌的临床特点及危险因素]。
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20230315-00184
J Y Cai, C Y Yan, X Q Wang, Z X Luo, J Luo, Q B Li, E M Liu, Y Deng

Objective: To investigate the risk factors for airway mucus hypersecretion in childhood pneumonia infected by different pathogens. Method: A retrospective cohort included 968 children who were hospitalized for Mycoplasma pneumoniae pneumonia (MPP), respiratory syncytial virus (RSV) pneumonia, adenovirus pneumonia and underwent bronchoscopy in Respiratory Department of Children's Hospital of Chongqing Medical University from January 2019 to December 2021 was conducted. The children were divided into two groups distinguished by airway mucus secretion according to the airway mucus hypersecretion score which were scored according to the mucus secretion under the bronchoscope. The demographic characteristics, clinical characteristics, laboratory tests and disease severity of the two groups were compared. And the risk factors for the development of airway mucus hypersecretion in two groups were analyzed. Chi square test, Mann-Whithey U test and Fisher exact test were used to analyze the differences between the two groups, and multivariate Logistic regression was used to analyze the influencing factors. Result: There were 559 males and 409 females in the 968 children, with an age of 4.0 (1.4, 6.0) years. Among the 642 children with MPP, 185 cases were in the hypersecretion group and 457 cases were in the non-hypersecretion group. There were 41 cases in the hypersecretion group and 160 cases in the non-hypersecretion group of 201 children with RSV pneumonia. In the 125 children with adenovirus pneumonia, there were 39 cases in the hypersecretion group and 86 cases in the non-hypersecretion group. In these children, the age of children in the hypersecretion group was older than that in the non-hypersecretion group (6.0 (4.0, 7.0) vs. 5.0 (3.0, 7.0) years old, 1.5 (0.5, 3.6) vs. 0.8 (0.4, 1.6) years old, 2.0 (1.2, 4.5) vs. 1.3 (0.8, 2.0) years old, U=35 295.00, 2 492.00, 1 101.00, all P<0.05). Through multivariate Logistic regression analysis it found that increased risk of airway mucus hypersecretion was present in childhood MPP with increase in peripheral blood white blood cell count (OR=3.30, 95%CI 1.51-7.93, P=0.004) or increase in neutrophil ratio (OR=2.24, 95%CI 1.16-4.33, P=0.016) or decrease in lymphocyte count (OR=3.22, 95%CI 1.66-6.31, P<0.001) or decrease in serum albumin (OR=2.00, 95%CI 1.01-3.98, P=0.047). The risk of airway mucus hypersecretion was increased in children with RSV pneumonia combined with elevated peripheral blood eosinophils (OR=3.04, 95%CI 1.02-8.93, P=0.043). Meanwhile, airway mucus hypersecretion was associated with severe pneumonia (OR=2.46, 95%CI 1.03-6.15, P=0.047) in children with RSV pneumonia. Older age was associated with increased risk of airway mucus hypersecretion in children with adenovirus pneumonia (OR=1.02

目的:探讨不同病原菌感染的儿童肺炎气道黏液高分泌的危险因素。方法:回顾性分析重庆医科大学附属儿童医院呼吸科2019年1月至2021年12月因肺炎支原体肺炎(MPP)、呼吸道合胞病毒肺炎(RSV)、腺病毒肺炎住院并行支气管镜检查的968例患儿。根据气道粘液高分泌评分将患儿分为两组,以气道粘液分泌区分,气道粘液高分泌评分根据支气管镜下气道粘液分泌进行评分。比较两组患者的人口学特征、临床特征、实验室检查及疾病严重程度。并分析两组患者发生气道粘液分泌亢进的危险因素。采用卡方检验、mann - whthey U检验和Fisher精确检验分析两组间差异,采用多因素Logistic回归分析影响因素。结果:968例患儿中,男559例,女409例,年龄4.0(1.4,6.0)岁。642例MPP患儿中,高分泌组185例,非高分泌组457例。201例RSV肺炎患儿中,高分泌组41例,非高分泌组160例。125例腺病毒肺炎患儿中,高分泌组39例,非高分泌组86例。在这些孩子中,分泌过多的儿童时代集团是比non-hypersecretion组(6.0(4.0,7.0)和5.0(3.0,7.0)岁,1.5(0.5,3.6)和0.8(0.4,1.6)岁,2.0(1.2,4.5)和1.3(0.8,2.0)岁,U = 35 295.00, 2 492.00, 1 101.00,所有= 3.30,95% ci 1.51 - -7.93, P = 0.004)或增加中性粒细胞比率(或= 2.24,95% ci 1.16 - -4.33, P = 0.016)或减少淋巴细胞计数(或= 3.22,95% ci 1.66 - -6.31 = 2.00, 95% ci 1.01 - -3.98, P = 0.047)。呼吸道合胞病毒肺炎合并外周血嗜酸性粒细胞升高的患儿气道黏液高分泌的风险增加(OR=3.04, 95%CI 1.02 ~ 8.93, P=0.043)。呼吸道合胞病毒肺炎患儿气道粘液分泌过多与重症肺炎相关(OR=2.46, 95%CI 1.03 ~ 6.15, P=0.047)。年龄较大与腺病毒肺炎患儿气道粘液分泌过多的风险增加相关(OR=1.02, 95%CI 1.00-1.04, P=0.026)。出现肺啰音、喘息或痰音的患儿(or =3.65, 95%CI 1.22 ~ 12.64, P=0.028)气道粘液分泌过多的风险增加。MPP患儿高分泌组支气管肺泡灌洗液(BALF)中性粒细胞比例较高(0.65(0.43,0.81)比0.59 (0.34,0.76),U=24 507.00, pv = 0.12 (0.06, 0.24), U=33 043.00, P6比490 (210,1 510)×106/L, 0.43(0.26, 0.62)比0.30 (0.13,0.52),U=2 043.00, 2 064.00,均为pp。MPP患儿外周血白细胞计数、中性粒细胞比例升高,淋巴细胞计数、血清白蛋白下降与气道粘液分泌增多有关。呼吸道合胞病毒肺炎患儿外周血嗜酸性粒细胞异常升高与高分泌有关。腺病毒肺炎患儿肺鸣、喘鸣和痰鸣的出现与气道粘液分泌增多有关。此外,呼吸道局部中性粒细胞浸润与肺炎支原体和RSV感染引起气道粘液高分泌的发生密切相关。
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引用次数: 0
[Summary to the Sixth Special Summit on Child Health and Early Prevention of Adult Diseases]. [第六次儿童健康和早期预防成人疾病问题特别首脑会议摘要]。
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20230605-00378
L Chen, X D Yu, F Yang, M Mao, Z Y Zhao, T Y Li, F Jiang
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引用次数: 0
[Expert consensus for the management of congenital micropenis]. 【先天性小阴茎治疗的专家共识】。
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20230111-00025
先天性小阴茎是一种临床症状体征,可反映机体存在内分泌或遗传性疾病,也是患儿和家长担心的心理社会问题。其病因复杂,诊疗管理争议大,国内外尚无针对此类疾病的管理共识、指南或规范。为规范小阴茎的临床管理,尽量减少不当诊疗带来的一系列问题,经多个学组多学科专家共同讨论,并参考国内外的最新研究进展,制定了先天性小阴茎临床管理中国专家共识。.
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引用次数: 0
[Clinical features of post-neurosurgical bacterial meningitis in children]. 儿童神经外科术后细菌性脑膜炎的临床特点
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20230424-00295
L J Luo, J Wang, W J Chen, Y J Zhou, Y J Zhou, Y H Song, N Shen, Q Cao

Objective: To understand the characteristics of bacterial meningitis after pediatric neurosurgical procedures. Methods: This was a retrospective observational study. From January 2016 to December 2022, 64 children diagnosed with post-neurosurgical bacterial meningitis based on positive cerebrospinal fluid (CSF) culture in Department of Neurosurgery of Shanghai Children's Medical Center were selected as the study population. The clinical characteristics, onset time, routine biochemical indexes of cerebrospinal fluid before anti infection treatment, bacteriology characteristics and sensitivity to antibiotics of bacteria cultured from cerebrospinal fluid were analyzed. Based on the CSF culture results, the patients were divided into the Gram-positive bacteria infection group and the Gram-negative bacteria infection group. The clinical characteristics of the two groups were compared using t-tests or Wilcoxon rank-sum tests, and chi-square tests. Results: There were 64 children,42 boys and 22 girls, with onset age of 0.83 (0.50, 1.75) years. Seventy cases of post-neurosurgical bacterial meningitis occurred in the 64 children, of which 15 cases (21%) in spring, 23 cases (33%) in summer, 19 cases (27%) in autumn, and 13 cases (19%) in winter. The time of onset was 3.5 (1.0, 10.0) months after surgery; 15 cases (21%) occurred within the first month after the surgery, and 55 cases (79%) occurred after the first month. There were 38 cases (59%) showing obvious abnormal clinical manifestations, fever 36 cases (56%), vomiting 11 cases (17%). Forty-eight cases (69%) were caused by Gram-positive bacteria, with Staphylococcus epidermidis 24 cases; 22 cases (31%) were caused by Gram-negative bacteria, with Acinetobacter baumannii the prominent pathogen 7 cases. The Gram-positive bacterial infection was more common in summer than the Gram-negative bacterial infection (20 cases (42%) vs. 3 cases (14%), χ2=5.37, P=0.020), while the Gram-negative bacterial infection was more in autumn and within the first month after surgery than the Gram-positive bacterial infection (11 cases (50%) vs. 8 cases (17%), 15 cases (67%) vs. 5 cases (33%), χ2=8.48, 9.02; P=0.004, 0.003). Gram-positive bacteria resistant to vancomycin and Acinetobacter baumannii resistant to polymyxin were not found. However, Acinetobacter baumannii showed only 45% (10/22) susceptibility to carbapenem antibiotics. Conclusions: The clinical presentation of post-neurosurgical bacterial meningitis in children is atypical. Gram-positive bacteria are the main pathogens causing post-neurosurgical bacterial meningitis; Gram-negative bacterial meningitis are more likely to occur in autumn and within the first month after surgery. Acinetobacter baumannii has a high resistance rate to carbapenem antibiotics, which should be taken seriously.

目的:了解小儿神经外科术后细菌性脑膜炎的特点。方法:回顾性观察性研究。选取2016年1月至2022年12月上海儿童医疗中心神经外科脑脊液培养阳性诊断为神经术后细菌性脑膜炎的儿童64例作为研究人群。分析脑脊液培养细菌的临床特点、发病时间、抗感染治疗前脑脊液常规生化指标、细菌学特征及对抗生素的敏感性。根据脑脊液培养结果将患者分为革兰氏阳性菌感染组和革兰氏阴性菌感染组。采用t检验或Wilcoxon秩和检验、卡方检验比较两组患者的临床特征。结果:患儿64例,男42例,女22例,发病年龄0.83(0.50,1.75)岁。64例患儿发生神经术后细菌性脑膜炎70例,其中春季15例(21%),夏季23例(33%),秋季19例(27%),冬季13例(19%)。发病时间分别为术后3.5(1.0,10.0)个月;15例(21%)发生在术后1个月内,55例(79%)发生在术后1个月内。有明显临床异常表现38例(59%),发热36例(56%),呕吐11例(17%)。革兰氏阳性菌48例(69%),表皮葡萄球菌24例;革兰氏阴性菌22例(31%),以鲍曼不动杆菌为主7例。革兰氏阳性菌感染夏季多于革兰氏阴性菌感染(20例(42%)比3例(14%),χ2=5.37, P=0.020),革兰氏阴性菌感染秋季及术后1月内多于革兰氏阳性菌感染(11例(50%)比8例(17%),15例(67%)比5例(33%),χ2=8.48, 9.02;P = 0.004, 0.003)。未发现对万古霉素耐药的革兰氏阳性菌和对多粘菌素耐药的鲍曼不动杆菌。鲍曼不动杆菌对碳青霉烯类抗生素的敏感性仅为45%(10/22)。结论:小儿神经外科术后细菌性脑膜炎的临床表现不典型。革兰氏阳性菌是引起神经外科术后细菌性脑膜炎的主要病原体;革兰氏阴性细菌性脑膜炎更可能发生在秋季和手术后第一个月内。鲍曼不动杆菌对碳青霉烯类抗生素耐药率较高,应引起高度重视。
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引用次数: 0
[A case of intellectual developmental disorder with behavioral abnormalities and craniofacial dysmorphism with or without seizures caused by PHF21A gene variation and review of literature]. [PHF21A基因变异致智力发育障碍伴行为异常、颅面畸形伴或不伴癫痫发作1例及文献复习]。
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20230221-00118
F Wu, X N Ji, M X Shen, Y Y Gao, P P Zhang, S P Li, Q Chen

Objective: To discuss the clinical and genetic features of intellectual developmental disorder with behavioral abnormalities and craniofacial dysmorphism with or without seizures (IDDBCS). Methods: The clinical and genetic records of a patient who was diagnosed with IDDBCS caused by PHF21A gene variation at Children's Hospital Capital Institute of Pediatrics in 2021 were collected retrospectively. Using " PHF21A gene" as the keyword, relevant articles were searched at CNKI, Wanfang Data and PubMed from establishment of databases to February 2023. Clinical and genetic features of IDDBCS were summarized in the combination of this case. Results: An 8 months of age boy showed overgrowth (height, weight and head circumference were all higher than the 97th percentile of children of the same age and sex) and language and motor developmental delay after birth, and gradually showed autism-like symptoms like stereotyped behavior and poor eye contact. At 8 months of age, he began to show epileptic seizures, which were in the form of a series of spastic seizures with no reaction to adrenocorticotropic hormone but a good response to vigabatrin. Physical examination showed special craniofacial appearances including a prominent high forehead, sparse eyebrows, broad nasal bridge, and downturned mouth with a tent-shaped upper lip. The patient also manifested hypotonia. Whole exome sequencing showed a de novo heterogeneous variant, PHF21A (NM_001101802.1): c.54+1G>A, and IDDBCS was diagnosed. A total of 6 articles (all English articles) were collected, involving this case and other 14 patients of IDDBCS caused by PHF21A gene variation. Clinical manifestations were intellectual disability or developmental delay (15 patients), craniofacial anomalies (15 patients), behavioral abnormalities (12 patients), seizures (9 patients), and overgrowth (8 patients). The main pathogenic variations were frameshift variations (8 patients). Conclusions: IDDBCS should be considered when patients show nervous developmental abnormalities, craniofacial anomalies, seizures and overgrowth. PHF21A gene variation detection helps to make a definite diagnosis.

目的:探讨伴有或不伴有癫痫发作的智力发育障碍伴行为异常和颅面畸形(IDDBCS)的临床和遗传特征。方法:回顾性收集首都儿科研究所儿童医院2021年1例因PHF21A基因变异诊断为IDDBCS的临床和遗传学记录。以“PHF21A gene”为关键词,检索自建库至2023年2月,在CNKI、万方数据、PubMed检索相关文章。结合本病例总结IDDBCS的临床及遗传学特点。结果:1例8月龄男孩出生后发育过度(身高、体重、头围均高于同年龄、同性别儿童的第97百分位),语言、运动发育迟缓,逐渐表现出刻板行为、眼神交流不良等自闭症样症状。8个月大时,他开始出现癫痫发作,表现为一系列痉挛性发作,对促肾上腺皮质激素无反应,但对维加巴特林反应良好。体格检查显示特殊颅面外观,包括突出的高额头,稀疏的眉毛,宽鼻梁,下翻的嘴巴和帐篷状的上唇。患者还表现为肌张力过低。全外显子组测序显示全新异质变异PHF21A (NM_001101802.1): c.54+1G> a,诊断为IDDBCS。共收集到6篇文献(均为英文),涉及该病例及其他14例由PHF21A基因变异引起的IDDBCS患者。临床表现为智力障碍或发育迟缓(15例)、颅面异常(15例)、行为异常(12例)、癫痫发作(9例)、生长过度(8例)。以移码变异为主(8例)。结论:当患者出现神经发育异常、颅面异常、癫痫发作和过度生长时,应考虑IDDBCS。检测PHF21A基因变异有助于明确诊断。
{"title":"[A case of intellectual developmental disorder with behavioral abnormalities and craniofacial dysmorphism with or without seizures caused by PHF21A gene variation and review of literature].","authors":"F Wu,&nbsp;X N Ji,&nbsp;M X Shen,&nbsp;Y Y Gao,&nbsp;P P Zhang,&nbsp;S P Li,&nbsp;Q Chen","doi":"10.3760/cma.j.cn112140-20230221-00118","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230221-00118","url":null,"abstract":"<p><p><b>Objective:</b> To discuss the clinical and genetic features of intellectual developmental disorder with behavioral abnormalities and craniofacial dysmorphism with or without seizures (IDDBCS). <b>Methods:</b> The clinical and genetic records of a patient who was diagnosed with IDDBCS caused by PHF21A gene variation at Children's Hospital Capital Institute of Pediatrics in 2021 were collected retrospectively. Using \" PHF21A gene\" as the keyword, relevant articles were searched at CNKI, Wanfang Data and PubMed from establishment of databases to February 2023. Clinical and genetic features of IDDBCS were summarized in the combination of this case. <b>Results:</b> An 8 months of age boy showed overgrowth (height, weight and head circumference were all higher than the 97<sup>th</sup> percentile of children of the same age and sex) and language and motor developmental delay after birth, and gradually showed autism-like symptoms like stereotyped behavior and poor eye contact. At 8 months of age, he began to show epileptic seizures, which were in the form of a series of spastic seizures with no reaction to adrenocorticotropic hormone but a good response to vigabatrin. Physical examination showed special craniofacial appearances including a prominent high forehead, sparse eyebrows, broad nasal bridge, and downturned mouth with a tent-shaped upper lip. The patient also manifested hypotonia. Whole exome sequencing showed a de novo heterogeneous variant, PHF21A (NM_001101802.1): c.54+1G>A, and IDDBCS was diagnosed. A total of 6 articles (all English articles) were collected, involving this case and other 14 patients of IDDBCS caused by PHF21A gene variation. Clinical manifestations were intellectual disability or developmental delay (15 patients), craniofacial anomalies (15 patients), behavioral abnormalities (12 patients), seizures (9 patients), and overgrowth (8 patients). The main pathogenic variations were frameshift variations (8 patients). <b>Conclusions:</b> IDDBCS should be considered when patients show nervous developmental abnormalities, craniofacial anomalies, seizures and overgrowth. PHF21A gene variation detection helps to make a definite diagnosis.</p>","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 8","pages":"726-730"},"PeriodicalIF":0.0,"publicationDate":"2023-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10300846","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 infectious encephalomyelitis caused by CARD9 gene-associated candida albicans infection]. 【CARD9基因相关白色念珠菌感染致感染性脑脊髓炎1例】。
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20230104-00005
J Wang, Y An, G Zhang
患儿 男,12岁8月龄,因“间断头痛2个月,呕吐10 d”于2021年1月6日入住西安市儿童医院神经外科。结合病史、体格检查、实验室检查、宏基因组二代测序、影像学检查,诊断为白色念珠菌致感染性脑脊髓炎,基因检测结果发现患儿携带CARD9基因NM—052813.5:c.1118G>C(p.R373P)和c.951G>A(p.R317R)复合杂合变异,其中p.R373P可致常染色体隐性遗传的家族性念珠菌2型感染。经两性霉素B静脉点滴及鞘内注射,联合伏立康唑、氟胞嘧啶口服,共治疗4.3个月,好转出院。.
{"title":"[A case of infectious encephalomyelitis caused by CARD9 gene-associated candida albicans infection].","authors":"J Wang,&nbsp;Y An,&nbsp;G Zhang","doi":"10.3760/cma.j.cn112140-20230104-00005","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230104-00005","url":null,"abstract":"患儿 男,12岁8月龄,因“间断头痛2个月,呕吐10 d”于2021年1月6日入住西安市儿童医院神经外科。结合病史、体格检查、实验室检查、宏基因组二代测序、影像学检查,诊断为白色念珠菌致感染性脑脊髓炎,基因检测结果发现患儿携带CARD9基因NM—052813.5:c.1118G>C(p.R373P)和c.951G>A(p.R317R)复合杂合变异,其中p.R373P可致常染色体隐性遗传的家族性念珠菌2型感染。经两性霉素B静脉点滴及鞘内注射,联合伏立康唑、氟胞嘧啶口服,共治疗4.3个月,好转出院。.","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 8","pages":"737-739"},"PeriodicalIF":0.0,"publicationDate":"2023-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9925455","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
[Research progresses of tumor associated macrophages in neuroblastoma]. [神经母细胞瘤肿瘤相关巨噬细胞的研究进展]。
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20221117-00980
S D He, Y Su
神经母细胞瘤(NB)是儿童常见的颅外实体肿瘤。NB的发生与各种类型的免疫细胞浸润有关,特别是肿瘤相关巨噬细胞(TAM),TAM具有促进肿瘤生长并抑制免疫的功能。TAM的靶向治疗为NB患儿提供了新的治疗选择。本文对近年来NB与TAM研究领域的相关进展进行综述。.
{"title":"[Research progresses of tumor associated macrophages in neuroblastoma].","authors":"S D He,&nbsp;Y Su","doi":"10.3760/cma.j.cn112140-20221117-00980","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20221117-00980","url":null,"abstract":"神经母细胞瘤(NB)是儿童常见的颅外实体肿瘤。NB的发生与各种类型的免疫细胞浸润有关,特别是肿瘤相关巨噬细胞(TAM),TAM具有促进肿瘤生长并抑制免疫的功能。TAM的靶向治疗为NB患儿提供了新的治疗选择。本文对近年来NB与TAM研究领域的相关进展进行综述。.","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 8","pages":"760-763"},"PeriodicalIF":0.0,"publicationDate":"2023-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10300843","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
[Clinical presentation and prognosis in children over 10-year-old with primary nephrotic syndrome]. 【10岁以上原发性肾病综合征患儿的临床表现及预后分析】。
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20230104-00007
J Tu, C Y Chen, H X Yang, Y Jia, H Y Geng, H R Li

Objective: To summary the clinical presentation and prognosis of primary nephrotic syndrome (PNS) in teenagers. Methods: The clinical data, renal pathological types and prognosis of 118 children over 10-year-old with PNS treated in the Department of Nephrology of the Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2010 to December 2020 were retrospectively analyzed, with 408 children ≤10-year-old as control group synchronously. Chi-square test was used to compare the difference of clinical types, pathologic types, response to steroids and tubulointerstitial changes between the groups. The teenagers with steroid resistant nephrotic syndrome (SRNS) were divided into initial non-responder group and late non-responder group. Kaplan-Meier method was used to compare the difference of persistent proteinuria, and Fisher's exact test for the histological types. Results: There were 118 children >10-year-old, including 74 males and 44 females, with the onset age of 12.1 (10.8, 13.4) years; and 408 children ≤10-year-old with the onset age of 4.5 (3.2, 6.8) years. The proportion of SRNS was significantly higher in patients >10-year-old than those ≤10-year-old (24.6% (29/118) vs. 15.9% (65/408), χ2=4.66, P=0.031). There was no statistical difference in the pathological types between >10-year-old and ≤10-year-old (P>0.05), with minimal change disease the most common type (56.0% (14/25) vs. 60.5% (26/43)). The percentage of cases with renal tubulointerstitial lesions was significantly higher in children >10-year-old compared to those ≤10-year-old (60.0% (15/25) vs. 23.3% (10/43), χ2=9.18, P=0.002). There were 29 cases presented with SRNS in PNS over 10-year-old, including 19 initial non-responders and 10 late non-responders. Analyzed by Kaplan-Meier curve, it was shown that the percentage of persistent proteinuria after 6 months of immunosuppressive treatments was significantly higher in initial non-responders than those of the late non-responders ((22±10)% vs. 0, χ2=14.68, P<0.001); the percentage of minimal change disease was significantly higher in patients of late non-responders than those of the initial non-responders (5/6 vs. 3/13, P=0.041). Of the 63 >10-year-old with steroid-sensitive nephrotic syndrome followed up more than one year, 38 cases (60.3%) had relapse, and 14 cases (22.2%) were frequent relapse nephrotic syndrome and steroid dependent nephrotic syndrome. Among the 45 patients followed up over 18-year-old, 22 cases (48.9%) had recurrent proteinuria continued to adulthood, 3 cases of SRNS progressed to kidney insufficiency, and one of them developed into end stage kidney disease and was administrated with hemodialysis. Conclusions: Cases over 10-year-old with PNS tend to present with SRNS and renal tubulointerstitial lesions. They have a favorable prognosis,

目的:总结青少年原发性肾病综合征(PNS)的临床表现及预后。方法:回顾性分析2010年1月至2020年12月首都儿科附属儿童医院肾内科收治的118例10岁以上PNS患儿的临床资料、肾脏病理分型及预后,同时以408例≤10岁患儿为对照组。采用卡方检验比较两组患者的临床类型、病理类型、类固醇反应及小管间质改变的差异。将青少年类固醇抵抗性肾病综合征(SRNS)分为初始无反应组和晚期无反应组。用Kaplan-Meier法比较持续性蛋白尿的差异,用Fisher精确检验比较组织学类型。结果:10岁以上儿童118例,其中男74例,女44例,发病年龄12.1(10.8,13.4)岁;10岁以下儿童408例,发病年龄为4.5(3.2,6.8)岁。>10岁患者发生SRNS的比例明显高于≤10岁患者(24.6%(29/118)比15.9% (65/408),χ2=4.66, P=0.031)。>10岁与≤10岁患儿病理分型比较,差异无统计学意义(P>0.05),以微小病变最常见(56.0%(14/25)比60.5%(26/43))。>10岁儿童肾小管间质病变发生率明显高于≤10岁儿童(60.0%(15/25)比23.3% (10/43),χ2=9.18, P=0.002)。在10岁以上的PNS患者中,有29例出现SRNS,其中19例最初无应答,10例晚期无应答。Kaplan-Meier曲线分析显示,免疫抑制治疗6个月后,初始无反应组持续蛋白尿比例显著高于晚期无反应组((22±10)% vs. 0, χ2=14.68, P0.001);晚期无反应患者的微小病变百分比明显高于初始无反应患者(5/6 vs 3/13, P=0.041)。63 >10岁的类固醇敏感性肾病综合征患者随访1年以上,38例(60.3%)复发,14例(22.2%)为频繁复发肾病综合征和类固醇依赖性肾病综合征。18岁以上随访的45例患者中,22例(48.9%)复发性蛋白尿持续至成年,3例SRNS发展为肾功能不全,1例发展为终末期肾病,给予血液透析治疗。结论:10岁以上的PNS患者往往表现为SRNS和肾小管间质病变。它们预后良好,但成年后易复发。
{"title":"[Clinical presentation and prognosis in children over 10-year-old with primary nephrotic syndrome].","authors":"J Tu,&nbsp;C Y Chen,&nbsp;H X Yang,&nbsp;Y Jia,&nbsp;H Y Geng,&nbsp;H R Li","doi":"10.3760/cma.j.cn112140-20230104-00007","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20230104-00007","url":null,"abstract":"<p><p><b>Objective:</b> To summary the clinical presentation and prognosis of primary nephrotic syndrome (PNS) in teenagers. <b>Methods:</b> The clinical data, renal pathological types and prognosis of 118 children over 10-year-old with PNS treated in the Department of Nephrology of the Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2010 to December 2020 were retrospectively analyzed, with 408 children ≤10-year-old as control group synchronously. Chi-square test was used to compare the difference of clinical types, pathologic types, response to steroids and tubulointerstitial changes between the groups. The teenagers with steroid resistant nephrotic syndrome (SRNS) were divided into initial non-responder group and late non-responder group. Kaplan-Meier method was used to compare the difference of persistent proteinuria, and Fisher's exact test for the histological types. <b>Results:</b> There were 118 children >10-year-old, including 74 males and 44 females, with the onset age of 12.1 (10.8, 13.4) years; and 408 children ≤10-year-old with the onset age of 4.5 (3.2, 6.8) years. The proportion of SRNS was significantly higher in patients >10-year-old than those ≤10-year-old (24.6% (29/118) <i>vs.</i> 15.9% (65/408), <i>χ</i><sup>2</sup>=4.66, <i>P=</i>0.031). There was no statistical difference in the pathological types between >10-year-old and ≤10-year-old (<i>P</i>>0.05), with minimal change disease the most common type (56.0% (14/25) <i>vs.</i> 60.5% (26/43)). The percentage of cases with renal tubulointerstitial lesions was significantly higher in children >10-year-old compared to those ≤10-year-old (60.0% (15/25) <i>vs.</i> 23.3% (10/43), <i>χ</i><sup>2</sup>=9.18, <i>P=</i>0.002). There were 29 cases presented with SRNS in PNS over 10-year-old, including 19 initial non-responders and 10 late non-responders. Analyzed by Kaplan-Meier curve, it was shown that the percentage of persistent proteinuria after 6 months of immunosuppressive treatments was significantly higher in initial non-responders than those of the late non-responders ((22±10)% <i>vs.</i> 0, <i>χ</i><sup>2</sup>=14.68, <i>P<</i>0.001); the percentage of minimal change disease was significantly higher in patients of late non-responders than those of the initial non-responders (5/6 <i>vs.</i> 3/13, <i>P=</i>0.041). Of the 63 >10-year-old with steroid-sensitive nephrotic syndrome followed up more than one year, 38 cases (60.3%) had relapse, and 14 cases (22.2%) were frequent relapse nephrotic syndrome and steroid dependent nephrotic syndrome. Among the 45 patients followed up over 18-year-old, 22 cases (48.9%) had recurrent proteinuria continued to adulthood, 3 cases of SRNS progressed to kidney insufficiency, and one of them developed into end stage kidney disease and was administrated with hemodialysis. <b>Conclusions:</b> Cases over 10-year-old with PNS tend to present with SRNS and renal tubulointerstitial lesions. They have a favorable prognosis, ","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 8","pages":"708-713"},"PeriodicalIF":0.0,"publicationDate":"2023-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9916575","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
[Comparison of two child growth standards in assessing the nutritional status of children under 6 years of age]. [两种儿童生长标准评价6岁以下儿童营养状况的比较]。
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20230505-00314
S Wang, Y Mei, Z Y Yang, Q Zhang, R L Li, Y Y Wang, W H Zhao, T Xu

Objective: To compare the application of China growth standard for children under 7 years of age (China standards) and World Health Organization child growth standards (WHO standards) in evaluating the prevalence of malnutrition in children aged 0-<6 years in China. Methods: The research data came from the national special program for science & technology basic resources investigation of China, named "2019-2021 survey and application of China's nutrition and health system for children aged 0-18 years". Multi-stage stratified random sampling was used to recruit 28 districts (regions) in 14 provinces, autonomous regions or municipalities across the country. Children (n=38 848) were physically measured and questionnaires were conducted in the guardians of the children. The indicators of stunting, underweight, wasting, overweight and obesity were evaluated by China standards and WHO standards respectively. Chi-square test was used to comparing the prevalence of each nutritional status between the two standards, as well as the comparison between the two standards by gender and age. Results: Among the 38 848 children, 19 650 were boys (50.6%) and 19 198 were girls (49.4%), 19 480 urban children (50.1%) and 19 368 rural children (49.9%). The stunting, underweight and wasting cases in the study population were 2 090 children (5.4%), 1 354 children (3.5%) and 1 276 children (3.3%) according to the China standards, and 1 474 children (3.8%), 701 children (1.8%) and 824 children (2.1%) according to the WHO standards, respectively; the above rates according to the China standards were slightly higher than those to the WHO standards (χ2=111.59, 213.14, and 99.99, all P<0.001). The overweight and obesity cases in the study population were 2 186 children (5.6%) and 1 153 children (3.0%) according to the China standards, and 2 210 children (5.7%) and 1 186 children (3.1%) according to the WHO standards, with no statistically significant differences (χ2=0.14 and 0.48, P=0.709 and 0.488, respectively). Compared to the results based on WHO standards, the China standards showed a lower prevalence of overweight and obesity in boys (χ2=14.95 and 5.85, P<0.001 and =0.016, respectively), and higher prevalence of overweight in girls (χ2=12.60, P<0.001); but there was no statistically significant differences in girls' obesity prevalence between the two standards (χ2=2.62, P=0.106). Conclusions: In general, the prevalence of malnutrition among children aged 0-<6 years based on China standards is slightly higher than that on WHO standards. To evaluate the nutritional status of children, it is advisable to select appropriate child growth standards based on work requirements, norms or research objectives.

目的:比较中国7岁以下儿童生长标准(中国标准)与世界卫生组织儿童生长标准(世卫组织标准)在评价0岁以下儿童营养不良患病率中的应用情况。方法:研究数据来自国家科技基础资源调查专项“2019-2021年中国0-18岁儿童营养卫生体系调查与应用”。采用多阶段分层随机抽样方法,在全国14个省、自治区、直辖市的28个区(区)开展调查。对38 848名儿童进行体格测量,并对其监护人进行问卷调查。发育迟缓、体重不足、消瘦、超重和肥胖指标分别采用中国标准和世界卫生组织标准进行评价。采用卡方检验比较两种标准中各营养状况的患病率,以及两种标准在性别和年龄方面的比较。结果:38 848名儿童中,男孩19 650名(50.6%),女孩19 198名(49.4%),城市儿童19 480名(50.1%),农村儿童19 368名(49.9%)。研究人群发育迟缓2 090例(5.4%),体重不足1 354例(3.5%),消瘦1 276例(3.3%),WHO标准分别为1 474例(3.8%),701例(1.8%),824例(2.1%);按中国标准的上述检出率略高于按世界卫生组织标准的检出率(χ2=111.59、213.14、99.99,p < 0.001)。研究人群中超重和肥胖病例按中国标准分别为2 186例(5.6%)和1 153例(3.0%),按世界卫生组织标准分别为2 210例(5.7%)和1 186例(3.1%),差异均无统计学意义(χ2=0.14、0.48,P=0.709、0.488)。与基于WHO标准的结果相比,中国标准的男孩超重和肥胖患病率较低(χ2=14.95和5.85,Pχ2=12.60, P0.001);两种标准间女生肥胖率差异无统计学意义(χ2=2.62, P=0.106)。结论:总体而言,0- 0岁儿童营养不良患病率较高
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引用次数: 0
[Clinical characteristics of 42 children with focal atrial tachycardia originated from the right atrial appendage]. [小儿局灶性房性心动过速42例临床特点]。
Pub Date : 2023-08-02 DOI: 10.3760/cma.j.cn112140-20221227-01067
Y Zhang, X M Li, H Jiang, Y Q Jin, M T Li, Y L Gu, H M Zhou

Objective: To investigate the feature and treatment of atrial tachycardia (AT) originated from right atrial appendage (RAA) in children. Methods: The data of 42 children with AT originated from RAA, who were admitted the First Hospital of Tsinghua University from January 2010 to September 2022 were analyzed retrospectively.The clinical characteristics, treatment and efficacy were analyzed. The children were divided into tachycardia cardiomyopathy group and normal cardiac function group. The differences in the ablation age and the heart rate during AT between two groups were compared by independent sample t-test. Results: Among 42 children, there were 20 males and 22 females. The age of onset was 2.7 (0.6, 5.1) years. Their age at radiofrequency ablation was (6.5±3.6) years, and the weight was (23.4±10.0) kg. Thirty-two children (76%) had sustained AT. The incidence of tachycardia cardiomyopathy was 43% (18/42). Compared to that of the normal cardiac function group, the ablation age and the heart rate at atrial tachycardia of the tachycardia cardiomyopathy group were higher ((8.1±3.8) vs. (5.3±3.1) years, t=-2.63, P=0.012; (173±41) vs. (150±30) beats per minute, t=-2.05, P=0.047. Thirty-eight children (90%) responded poorly to two or more antiarrhythmic drugs. The immediate success rate of radiofrequency ablation (RFCA) was 57% (24/42), and the AT recurrence rate was 17% (4/24). Twenty-two children underwent RAA resection, and their AT were all converted to sinus rhythm after the surgery. During the RAA resection, 10 cases of right atrial appendage aneurysm were found, 9/18 of which failed the RFCA. Conclusions: The AT originated from the RAA in children tend to present with sustained AT, respond poorly to antiarrhythmic drugs, and has a low success rate of RFCA as well as high recurrence rate. Resection of the RAA is a safe and effective complementary treatment.

目的:探讨儿童右心房附件性房性心动过速(AT)的特点及治疗方法。方法:回顾性分析2010年1月至2022年9月清华大学第一医院收治的42例RAA源性AT患儿的资料。分析其临床特点、治疗方法及疗效。将患儿分为心动过速心肌病组和心功能正常组。采用独立样本t检验比较两组消融年龄和AT期间心率的差异。结果:42例患儿中,男20例,女22例。发病年龄为2.7(0.6,5.1)岁。患者射频消融年龄(6.5±3.6)岁,体重(23.4±10.0)kg。32例患儿(76%)持续AT。心动过速心肌病的发生率为43%(18/42)。与心功能正常组相比,心动过速心肌病组消融年龄和房性心动过速心率均增高((8.1±3.8)∶(5.3±3.1)年,t=-2.63, P=0.012;(173±41)vs(150±30)次/分,t=-2.05, P=0.047。38名儿童(90%)对两种或两种以上抗心律失常药物反应不佳。射频消融(RFCA)即刻成功率为57% (24/42),AT复发率为17%(4/24)。22例患儿行RAA切除术,术后AT均转化为窦性心律。在RAA切除术中发现10例右心房附件动脉瘤,其中9/18例RFCA手术失败。结论:儿童RAA源性AT多表现为持续性AT,抗心律失常药物反应差,RFCA成功率低,复发率高。RAA切除术是一种安全有效的辅助治疗方法。
{"title":"[Clinical characteristics of 42 children with focal atrial tachycardia originated from the right atrial appendage].","authors":"Y Zhang,&nbsp;X M Li,&nbsp;H Jiang,&nbsp;Y Q Jin,&nbsp;M T Li,&nbsp;Y L Gu,&nbsp;H M Zhou","doi":"10.3760/cma.j.cn112140-20221227-01067","DOIUrl":"https://doi.org/10.3760/cma.j.cn112140-20221227-01067","url":null,"abstract":"<p><p><b>Objective:</b> To investigate the feature and treatment of atrial tachycardia (AT) originated from right atrial appendage (RAA) in children. <b>Methods:</b> The data of 42 children with AT originated from RAA, who were admitted the First Hospital of Tsinghua University from January 2010 to September 2022 were analyzed retrospectively.The clinical characteristics, treatment and efficacy were analyzed. The children were divided into tachycardia cardiomyopathy group and normal cardiac function group. The differences in the ablation age and the heart rate during AT between two groups were compared by independent sample <i>t</i>-test. <b>Results:</b> Among 42 children, there were 20 males and 22 females. The age of onset was 2.7 (0.6, 5.1) years. Their age at radiofrequency ablation was (6.5±3.6) years, and the weight was (23.4±10.0) kg. Thirty-two children (76%) had sustained AT. The incidence of tachycardia cardiomyopathy was 43% (18/42). Compared to that of the normal cardiac function group, the ablation age and the heart rate at atrial tachycardia of the tachycardia cardiomyopathy group were higher ((8.1±3.8) <i>vs.</i> (5.3±3.1) years, <i>t</i>=-2.63, <i>P</i>=0.012; (173±41) <i>vs.</i> (150±30) beats per minute, <i>t</i>=-2.05, <i>P</i>=0.047. Thirty-eight children (90%) responded poorly to two or more antiarrhythmic drugs. The immediate success rate of radiofrequency ablation (RFCA) was 57% (24/42), and the AT recurrence rate was 17% (4/24). Twenty-two children underwent RAA resection, and their AT were all converted to sinus rhythm after the surgery. During the RAA resection, 10 cases of right atrial appendage aneurysm were found, 9/18 of which failed the RFCA. <b>Conclusions:</b> The AT originated from the RAA in children tend to present with sustained AT, respond poorly to antiarrhythmic drugs, and has a low success rate of RFCA as well as high recurrence rate. Resection of the RAA is a safe and effective complementary treatment.</p>","PeriodicalId":23998,"journal":{"name":"Zhonghua er ke za zhi = Chinese journal of pediatrics","volume":"61 8","pages":"714-718"},"PeriodicalIF":0.0,"publicationDate":"2023-08-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9922399","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
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Zhonghua er ke za zhi = Chinese journal of pediatrics
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