Hepatic involvement of Langerhans cell histiocytosis in children—imaging findings of computed tomography,magnetic resonance imaging and magnetic resonance cholangiopancreatography

Background

Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor.

Objective

To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP).

Materials and methods

Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n?=?5) or MRI (n?=?4), or CT and MRI examinations (n?=?4) to evaluate the liver involvement.

Results

Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n?=?6), and cystic-like lesions on CT and MRI (n?=?3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses.

Conclusion

Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children.     

Clinical and genetic analyses of Joubert syndrome in children北大核心CSCD

目的 分析儿童Joubert综合征(Joubert syndrome,JS)的临床特征及遗传学特点。方法 回顾性分析2017年1月—2022年7月郑州大学第三附属医院儿童康复科诊断的20例JS患儿的临床资料、遗传学结果及随访资料。结果 20例JS患儿中,男11例,女9例。患儿临床表现以发育迟缓(20例,100%)、眼球运动异常(19例,95%)、肌张力低下(16例,80%)多见,5例(25%)出生时呼吸节律异常,3例(15%)面容异常(包括前额突出、耳位低、三角口),所有患儿均无肢体畸形。20例(100%)患儿头部影像学均有典型的“磨牙征”、“中线裂征”,6例(30%)患儿眼部检查异常。完成基因检测7例,发现6个致病基因(CPLANE1、RPGRIP1L、MKS1、CC2D2A、CEP120、AHI1基因)。结论 对于发育迟缓患儿,尤其是伴随眼球运动异常、肌张力低下症状,建议完善头部影像学检查明确有无“磨牙征”、“中线裂征”等特征,以排查JS,避免漏诊、误诊。JS有多种致病基因,全外显子组测序可协助诊断。     

The role of bronchoscopy in slide tracheoplasty in children北大核心CSCD

目的 总结支气管镜在儿童Slide气管成形术中的作用。方法 回顾性分析2017—2020年湖南省人民医院收治的4例气管狭窄患儿的诊疗经过,总结支气管镜在Slide气管成形术术前评估、术中定位与测量及术后创面评估和治疗中的作用。结果 Slide气管成形术术前支气管镜评估显示,4例患儿中3例存在完全气管环,2例合并肺动脉吊带,2例存在多处狭窄。3例在该院完成Slide气管成形术,术中支气管镜下判断狭窄段中点及测量狭窄段长度,协助离断气管狭窄段,术后灌洗明确病原;1例外院Slide气管成形术术后9个月出现瘢痕牵拉,经支气管镜下介入治疗好转。2例术后第4天支气管镜下发现气管黏膜改变,调整治疗方案;2例术后1个月出现肉芽增生,经支气管镜下冷冻治疗好转。1例因吻合口坏死放弃治疗死亡,存活的3例随访6个月以上,预后可,但均存在气管支气管软化。结论 支气管镜可用于气管狭窄患儿Slide气管成形术的管理,有助于术后康复及随访。     

Distribution of memory B cell subsets in peripheral blood of children with frequently relapsing nephrotic syndrome北大核心CSCD

目的观察记忆B细胞在频复发肾病综合征(frequently relapsing nephrotic syndrome,FRNS)患儿病程中分布变化。方法前瞻性选择2020年10月—2021年10月就诊于徐州医科大学附属医院儿科的原发肾病综合征(primary nephrotic syndrome,PNS)患儿35例,根据其糖皮质激素(glucocorticoid,GC)治疗后的反应及复发频次,分为FRNS组、非频复发肾病综合征(non-frequently relapsing nephrotic syndrome,NFRNS)组;选择同期15例体检儿童为健康对照组。比较各组GC治疗前后记忆B细胞变化,并与临床指标作相关性分析。结果治疗前,FRNS组、NFRNS组总B细胞、总记忆B细胞、IgD^(+)记忆B细胞、IgE^(+)记忆B细胞比例均较健康对照组增高,且FRNS组较NFRNS组增高明显(P<0.05);FRNS组类别转换记忆B细胞比例较NFRNS组及健康对照组降低(P<0.05);治疗后,FRNS组、NFRNS组总B细胞、总记忆B细胞、IgM^(+)IgD^(+)记忆B细胞、IgM^(+)记忆B细胞、IgE^(+)记忆B细胞、IgD^(+)记忆B细胞、IgG^(+)记忆B细胞比例较治疗前降低(P<0.05);类别转换记忆B细胞比例较治疗前增高(P<0.05)。FRNS组尿蛋白定量高于NFRNS组及健康对照组(P<0.05),FRNS组白蛋白水平低于健康对照组(P<0.05);FRNS组尿蛋白定量与类别转换记忆B细胞比例呈负相关,与IgE^(+)记忆B细胞比例呈正相关(P<0.05)。结论FRNS患儿存在记忆B细胞亚群的分布异常;而IgE^(+)记忆B细胞和类别转换记忆B细胞比例可作为FRNS患儿在GC治疗后复发的正相关和负相关因素。     

Long-term trajectories of lung function and risk factors in children with bronchial asthma北大核心CSCD

目的建立支气管哮喘(哮喘)患儿肺功能长期变化的发展轨迹, 确定哮喘患儿出现长期肺功能损伤的危险因素。方法采用回顾性队列研究, 纳入2019年1月至12月在首都儿科研究所附属儿童医院定期随诊, 并完成肺功能检测的14岁以上哮喘患儿, 收集其肺功能资料及临床信息。采用潜变量增长模型(LCGM)拟合哮喘患儿肺功能发展轨迹, 建立不同的轨迹组, 组间比较采用t检验、方差分析或χ2检验, 确定肺功能长期变化危险因素采用多分类Logistic回归分析。结果共纳入哮喘患儿173例, 年龄6~17岁, 获得肺功能测定1 160例次。拟合4条1秒率(FEV1/FVC)潜分类轨迹:持续高水平组、高于平均水平组、低于平均水平组、持续低水平组, 其病例数分别为27例(15.6%)、66例(38.1%)、66例(38.1%)、14例(8.1%)。不同轨迹组患儿的FEV1/FVC在每一年龄组间的差异均有统计学意义(均P<0.05)。持续高水平组各年龄段的FEV1/FVC均在90%以上, 其余各轨迹组FEV1/FVC随年龄变化整体呈下降趋势, 低于平均水平组的FEV1/FVC在青春期后下降至80%以下;持续低水平组的FEV1/FVC均值在学龄期后即下降至80%以下, 至青春期接近70%。最大用力呼气中段流量(MMEF)的轨迹和波动情况与FEV1/FVC相似。危险因素分析显示, 与持续高水平组相比, 典型哮喘患儿肺功能轨迹处于低于平均水平组的风险是咳嗽变异性哮喘患儿的11.940倍(P=0.008);多重致敏患儿的肺功能轨迹处于低于平均水平组的风险是单一致敏的7.462倍(P=0.015);未规律用药患儿肺功能处于持续低水平组的风险是规律用药者的6.337倍(P=0.035);男童肺功能轨迹处于低于平均水平组的风险是女童的6.186倍(P=0.002)。结论 6~17岁哮喘患儿的长期肺功能变化可确定4条不同轨迹:持续高水平、高于平均水平、低于平均水平、持续低水平;近半数患儿的长期肺功能轨迹处于低水平, 较多患儿在青春期, 少数患儿在学龄期出现持续性气流受限;典型哮喘、多重致敏、未规律用药、男性是哮喘患儿长期肺功能降低的危险因素。     

Clinical and laboratory results of 270 children with infectious mononucleosis北大核心CSCD

Objective To summarize the clinical and laboratory characteristics of infectious mononucleosis (I M) in children. Methods Clinical features and laboratory results of 270 cases with IM admitted to the Department of Pediatrics in Strategic Support Force Medical Center of People's Liberation Army from January 2012 to December 2020 were retrospectively analyzed. χ2 test was used for comparison between groups. Results IM mainly occurred in children aged 5 months to 18 years old in autumn and spring. The highest incidence rate (105 cases, 38.9 %) was 3 - <6 years old (preschoolers). There were 253 cases (93.7%) with fever, 266 cases (98.5 %) with adenopharyngitis, 196 cases (72.6%) with tonsil pseudomembrane or exudation, 248 cases (91.9%) with cervical lymphadenopathy, 92 cases (34.1%) with eyelid edema, 202 cases (74.8%) with nasal obstruction, 124 cases (45.9%) with nasal obstruction and snoring, 24 cases (8. 9 %) with rash, and 112 cases (41.5%) with splenomegaly. A total of 225 cases (83.3%) presented with typical triplets of IM (fever, adenopharyngitis and cervical lymphadenopathy). Sixty - two IM patients were complicated with pulmonary infections and 3 cases with diarrhea. The main co - infection pathogens in children with IM were Mycoplasma pneumonia (M P) (79 cases, 29.3%), influenza A or B virus (34 cases, 12.6%), Streptococcus pneumonia (S P) (18 cases, 6.7%), adenovirus (22 cases, 8.1%) and cytomegalovirus (3 cases, 1.11%). A total of 46 cases (17.0%) had multiple infections. Laboratory test results suggested that absolute lymphocyte count 5. 0 x 109/L was found in 199 cases (73.7%), and abnormal lymphocyte ratio > 0.10 was found in 225 cases (83.3%). Some children had elevated transaminase levels. Epstein - Barr virus capsid antigen - immunoglobulin M (EBV - VCA - IgM) was positive in 249 cases (92.2%), Epstein - Barr virus capsid antigen - immunoglobulin G (EBV - VCA - IgG) was positive in 238 cases (88.1%), and Epstein - Barr virus nuclear antigen - immunoglobulin G (EBV - NA - IgG) was negative in all cases. EBV - VCA - IgG showed low affinity in all cases (< 40%). EBV DNA tests of peripheral blood plasma were carried in 153 cases, of which 118 cases (77.1%) were positive. Conclusions EBV related IM mainly attacks preschoolers. Most patients are presented with typical triplets of IM. Eyelid edema, nasal obstruction, snoring, splenomegaly and elevated transaminase levels are prevalent in IM children. Most cases have a favorable prognosis. © 2022 ＣｈｉｎＪＡｐｐｌＣｌｉｎＰｅｄｉａｔ. All rights reserved.     

Diversity and functional prediction of gut microbiota in children with autism spectrum disorder北大核心CSCD

目的分析孤独症谱系障碍(autism spectrum disorder,ASD)儿童肠道菌群结构和多样性,并预测分析菌群的代谢功能。方法采集30例ASD儿童(ASD组)和20例正常发育(typically developing,TD)儿童(TD组)的粪便样本。提取基因组DNA,PCR扩增16S rDNA V4区,使用Illumina NovaSeq6000平台进行高通量测序。分析2组肠道菌群的构成和分布特征,并预测分析菌群的代谢功能。结果ASD组和TD组儿童肠道菌群的α多样性指数(Chao1、Shannon和Simpson)比较差异均无统计学意义(P>0.05)。在门和纲水平,2组儿童肠道菌群的结构差异无统计学意义(P>0.05)。在属水平,ASD组巨单胞菌属、巴恩斯氏菌属、小杆菌属、巨球菌属、瘤胃球菌属扭链群及梭杆菌属的丰度大于TD组(P<0.05)。功能预测分析显示,ASD组肠道菌群的色氨酸降解、谷氨酸降解及丁酸盐生成等代谢功能的丰度低于TD组(P<0.05),而γ-氨基丁酸降解功能的丰度高于TD组(P<0.05)。结论ASD儿童和TD儿童肠道菌群的α多样性无显著差异,但属水平物种构成不同,菌群的代谢功能有差别。     

Efficiency of Rituximab in treating children with refractory nephrotic syndrome北大核心CSCD

目的评价利妥昔单抗(RTX)在难治性肾病综合征患儿中维持缓解的有效性。方法回顾性研究。将2018年11月至2020年11月华中科技大学同济医学院附属武汉儿童医院肾内科诊断为难治性肾病综合征的22例患儿纳入研究, 予RTX治疗。外周血中CD19+B淋巴细胞≥1%总淋巴细胞者追加1剂RTX(375 mg/m2), 每例患儿使用3~4剂, 早期停用钙调神经磷酸酶抑制剂(CNI), 后续使用霉酚酸酯治疗。采用Kaplan-Meier法对RTX治疗后患儿的蛋白尿无复发率和无频复发肾病综合征或激素依赖肾病综合征发生率进行分析, 采用Wilcoxon秩和检验对使用RTX前后的复发次数进行分析。采用秩和检验对RTX治疗前后患儿的体质量指数(BMI)及身高进行比较。结果 22例患儿中, 20例患儿完成治疗方案, 1年和2年的蛋白尿无复发生存率分别为85%和40%, CNI停用后复发频率降低。所有患儿BMI及身高在使用RTX治疗前与使用RTX 1年后、2年后比较, 差异均有统计学意义(均P<0.05), 使用RTX后1、2年比较, 差异均无统计学意义(均P>0.05)。结论使用RTX在停用激素和其他免疫抑制剂情况下也可有效降低难治性肾病综合征的复发率, 同时可使患儿BMI及身高得到明显改善。RTX治疗难治性肾病综合征患儿安全有效。     

Effectiveness of autologous hematopoietic stem cell transplantation in the treatment of high-risk neuroblastoma in children: a single-center clinical study北大核心CSCD

Objective To investigate the effectiveness of high-dose chemotherapy combined with autologous hematopoietic stem cell transplantation (ASCT) in the treatment of children with high-risk neuroblastoma (NB). Methods A retrospective analysis was performed on 29 children with high-risk NB who were admitted to Shanghai Children's Hospital and were treated with high-dose chemotherapy combined with ASCT from January 2013 to December 2021, and their clinical features and prognosis were analyzed. Results Among the 29 children treated by high-dose chemotherapy combined with ASCT, there were 18 boys (62%) and 11 girls (38%), with a median age of onset of 36 (27, 59) months. According to the International Neuroblastoma Staging System, 6 children (21%) had stage III NB and 23 children (79%) had stage IV NB, and the common metastatic sites at initial diagnosis were bone in 22 children (76%), bone marrow in 21 children (72%), and intracalvarium in 4 children (14%). All 29 children achieved reconstruction of hematopoietic function after ASCT. After being followed up for a median time of 25 (17, 45) months, 21 children (72%) had continuous complete remission and 8 (28%) experienced recurrence. The 3-year overall survival rate and event-free survival rate were 68.9% ±16.1% and 61.4%±14.4%, respectively. Presence of bone marrow metastasis, neuron-specific enolase ≥370 ng/mL and positive bone marrow immunophenotyping might reduce the 3-year event-free survival rate (P<0.05). Conclusions Children with high-risk NB who have bone marrow metastasis at initial diagnosis tend to have a poor prognosis. ASCT combined with high-dose chemotherapy can effectively improve the prognosis of children with NB with a favorable safety profile. © 2023 Xiangya Hospital of CSU. All rights reserved.     

Features of unintentional injury in children under the impact of coronavirus disease 2019北大核心CSCD

目的探究新型冠状病毒肺炎疫情下儿童意外伤害的特征,旨在为疫情背景下儿童意外伤害的防治提供参考。方法回顾性分析2019年7月—2022年6月上海交通大学附属医学院新华医院收治的意外伤害患儿2526例的病例资料,按时间段分为5个阶段:疫情前(2019年7—12月)、武汉疫情期(2020年1—4月)、全国疫情平稳期(2020年5月—2022年2月)、上海疫情期(2022年3—5月)、上海疫情平稳期(2022年6月)。比较不同时间阶段和年龄阶段儿童意外伤害发生情况,并进一步对其中107例患儿进行问卷调查,探讨儿童意外伤害的特征。结果5个时间阶段意外伤害患儿的性别构成、年龄、年龄分布及多种类型意外伤害构成比的比较,差异均具有统计学意义(P<0.05)。武汉疫情及上海疫情期因意外伤害就诊的人数明显减少。外伤相关意外伤害患儿在各个阶段占比均超过50%,其中武汉疫情期及上海疫情期外伤相关意外伤害人数占比分别为63.9%和82.0%。意外伤害多见于学龄期及学龄前期儿童(1823例,72.17%)。与2021年同期相比,上海疫情期发生意外伤害患儿年龄更小(中位数7岁vs 11岁),外伤相关意外伤害患儿占比增加(97%vs 69%),差异均有统计学意义(P<0.05)。结论新型冠状病毒肺炎疫情背景下儿童意外伤害病例数减少,而外伤相关意外伤害占比增加。学龄前期及学龄期儿童意外伤害更常见。     

Clinical efficacy of combined therapy in children with stage 4 neuroblastoma北大核心CSCD

目的 观察4期神经母细胞瘤联合治疗的早期疗效,探讨提高生存率及改善生活质量的治疗方案。 方法 对2016年1月至2021年6月在香港大学深圳医院确诊的14例4期神经母细胞瘤患儿的临床资料、治疗及随访情况进行回顾性分析。 结果 14例患儿中位发病年龄为3岁7.5个月。骨髓检查阳性9例,N-Myc基因扩增4例,神经元特异性烯醇化酶增高13例,尿香草扁桃酸增高7例。病理结果：分化型6例,未分化型1例,混合型1例,分化差型6例。N7方案化疗10例（含2例N7方案+三氧化二砷化疗患儿）,Rapid COJEC方案化疗4例。手术13例;自体造血干细胞移植14例;放疗10例。Ch14.18/CHO免疫治疗8例,其中1例因在治疗过程中出现过敏性休克而停止免疫治疗;其余7例完成治疗,期间未观察到严重不良反应。完成免疫治疗患儿中1例复发后进行3次Lu177 Dotatate治疗,目前仍在化疗中。14例患儿中位随访时间为45个月。2年内复发4例,2年内总生存率100%;3年内复发4例,3年内无病存活7例。 结论 儿童4期神经母细胞瘤建议选择多学科联合方案治疗,使4期神经母细胞瘤患儿获得更好的生存及预后。     

Main causes of aplastic anemia misdiagnosed as immune thrombocytopenia in children北大核心CSCD

目的归纳曾被误诊为免疫性血小板减少症(ITP)的50例儿童再生障碍性贫血(再障)的主要原因和鉴别诊断经验,以供临床参考。方法参照儿童再障和ITP诊断标准,回顾性分析2007年1月至2020年12月上海市同济医院儿科收治的外院误诊病例的病初资料和本院复诊检测结果,归纳误诊原因和鉴别诊断要点。结果在同期收治的165例儿童再障中,共有50例(30.3%)曾被误诊为ITP。分析归纳主要误诊原因为:1.临床表现不符合典型ITP,未按照国际指南标准行必要的骨髓检查以明确诊断,共22/50例。2.骨髓检测结果解读有误。在28例初诊行骨髓涂片检查者中,6例(21%)骨髓显示典型再障骨髓象,但仍被诊断为ITP。3.骨髓涂片结果不典型者,未行骨髓活检以助诊断(15/28例,54%)。4.初诊时符合ITP诊断标准,但经糖皮质激素等治疗无效后,未行必要复查以核实诊断(7/28例,25%)。结论临床应严格参照执行相关疾病诊断标准,以避免经验性错误。诊断ITP需要慎重,尤其是临床表现不典型,或一线药物无效者,必须进行骨髓检查(必要时行骨髓活检),并按诊断标准正确解读检测结果,以避免临床误诊或漏诊。     

Detection of RAS gene mutation and its clinical significance in children with acute lymphoblastic leukemia北大核心CSCD

Objective To investigate the mutation rate of the RAS gene and its clinical significance in children with acute lymphoblastic leukemia. Methods A retrospective analysis was performed on the medical data of 120 children with newly diagnosed acute lymphoblastic leukemia, who were admitted to the Third Affiliated Hospital of Zhengzhou University from January 2015 to January 2020 and underwent next-generation sequencing. The clinical and molecular features were analyzed. The impact of RAS gene mutation on the overall survival rate was evaluated in these children. Results Among the 120 children, 35 (29.2%) had RAS gene mutation, 30 (25.0%) had KRAS gene mutation, and 5 (4.2%) had both NRAS and KRAS gene mutations. All NRAS mutations and 71% (25/35) of KRAS mutations were located at the 12th and 13th codons. RAS gene mutation was detected in 35 (33.3%) out of 105 children with B-lineage acute lymphoblastic leukemia, but it was not detected in those with acute T lymphocyte leukemia. Of all the children, 11 (9.2%) were lost to follow-up, and among the 109 children followed up, 16 (14.7%) died. The children with RAS gene mutation had a significantly lower 2-year overall survival rate than those without RAS gene mutation (P<0.05). The prognosis of children with RAS gene mutation combined with WT1 overexpression and WBC>50×109/L at diagnosis was worse (P<0.05). Conclusions RAS gene mutation is commonly observed in children with B-lineage acute lymphoblastic leukemia and may have an adverse effect on prognosis. © 2022 Xiangya Hospital of CSU. All rights reserved.     

Recent research on the application of defoamers in children undergoing digestive endoscopy北大核心CSCD

内镜是诊断和治疗儿童消化道疾病的常见手段。胃肠道内存在气泡,是影响内镜视野清晰度的重要因素之一。祛泡剂的应用,可显著减少肠道内气泡,提高胃肠道准备质量,进一步提高疾病检出率。现关于儿童内镜检查前的胃肠道准备各有研究,但缺乏对应用祛泡剂的统一方案。故该文就儿童消化内镜检查前祛泡剂的使用情况及研究进展作一总结,并指出对祛泡剂的现有研究样本量少,用药选择和时机仍存在争议,为以后祛泡剂的深入研究提供借鉴。     

Demographic features and compliance of children with allergic diseases receiving sublingual immunotherapy北大核心CSCD

目的探讨标准化舌下免疫治疗(sublingual immunotherapy,SLIT)过敏性疾病患儿的人口学特征,并分析依从性的影响因素。方法纳入江西省儿童医院2018年1月1日—2020年12月31日接受SLIT的1789例过敏性疾病患儿为研究对象,回顾性分析患儿的人口学特征和随访资料,分析SLIT的依从性及其影响因素。结果共1789例SLIT患儿,其中男性1271例(71.05%),女性518例(28.95%),年龄4~14岁;完全依从777例(43.43%);脱落1012例(56.57%),因自觉使用不便脱落354例(34.98%),因疗效欠佳脱落346例(34.19%),因症状改善自行停用253例(25.00%),因不良反应停用的59例(5.83%)。脱落主要集中在开始治疗后1~4个月(74.31%,752/1012)。女性患儿依从率低于男性患儿,患单一疾病者依从率低于患多种疾病者(P<0.05)。多因素分析结果显示,与男性相比,女性发生脱落的风险增加(OR=0.811,95%CI:0.658~0.998,P<0.05);相对于患多种疾病者,患单一疾病者发生脱落的可能性增加(OR=1.313,95%CI:1.005~1.715,P<0.05)。结论过敏性疾病患儿SLIT依从性不理想,与患儿性别、所患疾病数量等特征有关;主要脱落原因为自觉使用不便和疗效欠佳。     

Analysis of the inhalant allergen spectrum in 4488 children with allergic rhinitis in Tianjin北大核心CSCD

目的探讨天津地区儿童过敏性鼻炎(AR)吸入性变应原种类及特点,为儿童AR预防与诊疗提供依据。方法回顾性收集2016年3月至2022年2月天津医科大学第二医院儿科哮喘和过敏专病门诊AR患儿4488例的临床资料,分析儿童AR的吸入性变应原分布特点,探讨吸入性变应原阳性率及变应原种类与性别、年龄以及共患其他过敏性疾病的关系。采用UniCAP100系统(荧光酶联免疫法)测定血清变应原特异性IgE(sIgE)。计数资料以例数及百分率(%)表示,组间比较采用χ^(2)检验。结果1.4488例AR患儿中吸入性变应原阳性3116例,阳性率69.43%。单一变应原阳性比例28.47%(887/3116)、双重变应原阳性比例25.22%(786/3116)、3种变应原阳性比例19.67%(613/3116)、4种及4种以上变应原阳性比例26.64%(830/3116)。最常见吸入性变应原为霉菌类(45.72%,2052/4488),其次依次为粉尘螨(34.71%,1558/4488)、艾蒿(33.95%,313/922)、屋尘螨(31.13%,1397/4488)、豚草(30.97%,227/733)等。2.学龄前组、学龄组、青少年组吸入性变应原阳性率依次为56.15%(1132/2016)、79.26%(1624/2049)、85.11%(360/423)(χ^(2)=309.72,P<0.001)。在学龄前组、学龄组首位吸入性变应原均为霉菌类(40.23%、50.85%),在青少年组首位吸入性变应原为粉尘螨(56.74%),其次为屋尘螨(53.66%)、霉菌类(47.04%)。3组吸入性变应原阳性种类比较差异有统计学意义(χ^(2)=466.99,P<0.001)。在学龄前期组以单一变应原阳性为主,学龄组、青少年组吸入性变应原阳性种类增多,以4种及4种以上变应原为主。3.男童组吸入性变应原阳性率达73.28%(2139/2919),明显高于女童组阳性检出率62.40%(979/1569)(χ^(2)=58.28,P<0.001),男童和女童组常见吸入性变应原前3位均为霉菌类、艾蒿、粉尘螨。吸入性变应原阳性种类在男童与女童组比较差异有统计学意义(χ^(2)=75.02,P<0.001),女童组以单一变应原为主(20.78%),男童组变应原阳性种类增多,以4种及4种以上变应原阳性为主(20.45%)。4.AR共患支气管哮喘和特应性皮炎组吸入性变应原阳性率最高,达79.21%,其次AR共患支气管哮喘阳性率73.67%,AR共患特应性皮炎组阳性率61.05%,单纯AR组阳性率57.05%,组间比较差异有统计学意义(χ^(2)=178.57,P<0.001)。结论天津地区儿童AR主要吸入性变应原依次为霉菌类、粉尘螨、艾蒿、屋尘螨、豚草,不同年龄、性别及共患不同过敏性疾病吸入性变应原分布特征存在差异。应尽早为AR患儿检测变应原,做到提前预防,减少药物使用量,也为开展变应原免疫治疗提供依据。     

Progress of sensory abnormality and intervention in children with autism spectrum北大核心CSCD

Autism spectrum disorder (ASD) is a group of neurodevelopmental behavioral disorders, with the annually increased incidence in recent years. As one of the core symptoms of ASD, sensory abnormality not only affects the skill acquisition of affected children, but also brings great pain to caregivers and families. It is shown that early intervention of sensory abnormality can significantly improve the prognosis of ASD. However, early intervention depends on the early identification and diagnosis of sensory abnormality by clinicians. This study aims to review the clinical features, assessment tools, and intervention methods of sensory abnormalities in ASD children in recent years, thus contributing to the accurate and effective diagnosis, and timely intervention of ASD. © 2022 ＣｈｉｎＪＡｐｐｌＣｌｉｎＰｅｄｉａｔ. All rights reserved.     

The role of mother-child relationship in the association between maternal parenting stress and emotional and behavioral problems in preschool children北大核心CSCD

Objective To study the moderating effect of mother-child relationship in the association between maternal parenting stress and emotional and behavioral problems in preschool children, and to provide reference for the prevention and control of emotional and behavioral problems in preschool children. Methods Using a stratified cluster sampling method, 2 049 preschool children were surveyed from November to December 2021, who sampled from 12 kindergartens in Wuhu City, Anhui Province. The emotional and behavioral problems of preschool children were assessed with the Strength and Difficulties Questionnaire. Pearson correlation analysis was used to evaluate the relationship of maternal parenting stress and mother-child relationship with children's emotional and behavioral problems. The PROCESS Macro was used to analyze the moderating effect of conflicted and dependent mother-child relationships in the association between maternal parenting stress and emotional and behavioral problems in these preschool children. Results Among these preschool children, maternal parenting stress was positively correlated with the scores of emotional symptoms, conduct problems, hyperactivity, and peer problems subscales and total difficulty scores (P<0.001); intimate mother-child relationships were negatively correlated with the scores of conduct problems, hyperactivity, and peer problems subscales and total difficulty scores (P<0.001); conflicted and dependent mother-child relationships were positively correlated with the scores of emotional symptoms, conduct problems, hyperactivity, and peer problems subscales and total difficulty scores (P<0.001). After controlling for relevant confounding factors, conflicted mother-child relationship (β=0.05, P=0.001) and dependent mother-child relationship (β=0.04, P=0.012) were found to have a moderating effect on the association between maternal parenting stress and total difficulty scores in these preschool children. Conclusions Negative mother-child relationships play a moderating role in the association between maternal parenting stress and emotional and behavioral problems in preschool children. Prevention of emotional and behavioral problems in preschool children should focus on reducing maternal parenting stress and improving negative mother-child relationships. © 2023 Xiangya Hospital of CSU. All rights reserved.     

Establishment of the normal reference range of neurotransmitters in Han - nationality children aged 3-12 in Hubei province北大核心CSCD

Objective To establish the normal reference range of neurotransmitters in Han - nationality children aged 3-12 in Hubei province. Methods A prospective study was conducted on healthy Han - nationality children aged 3-12 who took physical examination in Wuhan Children's Hospital, Hubei province from January to August 2021. The children were asked for their medical histories, and those with neurological diseases, psychiatric diseases, infection, trauma, and a drug history in the past 2 weeks were excluded. The plasma of 324 children (262 males, 62 females;217 cases in the 3-7 years old, 107 cases in the 8-12 years old) and urine of 391 children (302 males, 89 females;266 cases in the 3-7 years old, 125 cases in the 8-12 years old) were collected. They ultra performance liquid chromatography - mass spectrometry multiple techniques (UPLC - MS/MS) were used to detect 10 kinds of neurotransmitters (e. g., dopamine, epinephrine, glutamic acid, etc.) in plasma and 8 kinds of neurotransmitters (e. g., dopamine, epinephrine, 5 - hydroxyindoleacetic acid, etc.) in random urine. The normal reference range of neurotransmitters in Han - nationality children aged 3-12 in Hubei province was established. The Kruskal - Wallis H test was made for statistical analysis of the differences in neurotransmitter levels among different age groups and gender groups. The neurotransmitter levels between different groups were compared by the Nemenyi test. Results There were no significant differences in the levels of various neurotransmitters in children of different genders(all P > 0. 05). There were significant differences in the levels of dopamine, methoxy - norepinephrine, tryptophan and γ - aminobutyric acid in the plasma of children aged 3-7 years and 8-12 years. There were significant differences in the levels of dopamine, epinephrine, norepinephrine, methoxy - norepinephrine, high vanillic acid and 5 - hydroxyindoleacetic acid in the random urine between the 3-7 years old group and the 8-12 years old group. Conclusions The normal reference range of neurotransmitters in Han - nationality children aged 3-12 in Hubei province is established. This study provides reference for clinical practice and lays a foundation for the study of neurotransmitter - related diseases in children. © 2022 Chinese Journal of Applied Clinical Pediatrics. All rights reserved.     

Clinical features,diagnosis and treatment of intussusception secondary to inverted Meckel's diverticulum in children北大核心CSCD

Objective To explore the clinical characteristics, diagnosis, treatment and prognosis of inverted Meckel's diverticulum in children, and to summarize the clinical experience. Methods Ten children who received surgical treatment in Beijing Children's Hospital, Capital Medical University from October 2018 to October 2021 were retrospectively analyzed. The children were diagnosed with Meeker's diverticulum. Their data were complete and their follow-up information was available. The clinical features, diagnosis, treatment and prognosis were summarized. There were 6 males and 4 females, whose age of onset ranged from 5 months to 12 years and 5 months. The clinical manifestations were abdominal pain in 8 cases, crying in 2 cases and bloody stool in 3 cases. B - ultrasound showed suspected diseases secondary to inverted Meckel's diverticulum, including small intestinal intussusception in 6 cases and back junction type intussusception in 4 cases. One case of small intestinal intussusception was misdiagnosed after laparoscopic exploration in other hospitals and suffered from recurrent intussusception. All patients underwent segmental bowel resection. Results Of the 10 children, 8 cases received laparoscopic exploration and 2 cases underwent open surgery for secondary intestinal necrosis. Postoperative pathology revealed Meckel's diverticulum. Ectopic tissue was accessible in 8 cases, including ectopic gastric tissue in 7 cases, and ectopic pancreas in 1 case. During the postoperative follow - up period (1 month - 3 years), the patients were generally in good condition with no complications such as intestinal obstruction. Conclusions Intussusception secondary to inverted Meckel's diverticulum is rare and difficult to diagnose before operation. The diagnosis should be confirmed by surgical exploration and postoperative pathology. However, careful surgical exploration is required since inverted Meckel's diverticulum is prone to be misdiagnosed during exploration. © 2022 ＣｈｉｎＪＡｐｐｌＣｌｉｎＰｅｄｉａｔ. All rights reserved.