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Correlation between TERT C228T and clinic-pathological features in pediatric papillary thyroid carcinoma

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Science China(Life Sciences) 2019年第12期62卷 1563-1571页

作者： Jiangqiao Geng Yuanhu Liu Yongli Guo Huanmin Wang Jun Tai Yaqiong Jin Jie Zhang Yongbo Yu Shengcai Wang yingluan song Xin Ni Otolaryngology Children's Hospital of Hebei ProvinceShijiazhuang 050031China Otolaryngology Head and Neck SurgeryBeijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck SurgeryBeijing Pediatric Research InstituteBeijing Children's HospitalCapital Medical UniversityNational Center for Childrens HealthBeijing 100045China Department of Surgical Oncology Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China

The aims of the present study were to reveal the prevalence of the TERT C228 T mutation in pediatric papillary thyroid carcinoma(PPTC) and to further investigate the role of the TERT C228 T mutation in PPTC. We also tested another TERT mutation, TERT C250 T, although this was not detected in PPTC patients. In this study, 48 patients with PPTC(41 with classic PPTC) were enrolled. DNA was extracted from PPTC tissues and TERT C228 T mutation analysis was performed. Chi-squared analysis,Fisher’s exact test, and a t-test were applied to test the significance of differences. The TERT C228 T mutation presented in 13(27.1%) of the 48 PPTC patients and 10(24.4%) of the 41 classical PPTC patients. There were significant differences between PPTC patients with the TERT C228 T mutation and those without in terms of modified radical neck dissection, multifocality,capsular invasion, extrathyroidal invasion, and American Joint Committee on Cancer(AJCC) tumor stage(P<0.05). In classical PPTC, there were additional significant differences in other clinic-pathological features, such as AJCC nodal stage(P=0.009)and American Thyroid Association(ATA) PPTC stage(P=0.021) between patients with and without the TERT C228 T *** findings indicate that the TERT C228 T mutation is significantly correlated with certain aggressive clinic-pathological features of PPTC.

关键词： pediatric papillary thyroid cancer TERT C228T mutation clinic-pathological characteristics

Correlation between BRAF^(V600E) mutation and clinicopathological features in pediatric papillary thyroid carcinoma

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Science China(Life Sciences) 2017年第7期60卷 729-738页

作者： Jiangqiao Geng Huanmin Wang Yuanhu Liu Jun Tai Yaqiong Jin Jie Zhang Lejian He Libing Fu Hong Qin yingluan song Jinzhu Su Aiying Zhang Xin Wen Yongli Guo Xin Ni Otolaryngology Head and Neck Surgery Beijing Children's Hospital Capital Medical University National Center for Children 's Health Beijing 100045 Chin Otolaryngology Children's Hospital of Hebei Province Shijiazhuang 050031 China Department of Surgical Oncology Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck Surgery Beo'ing Pediatric Research Institute Beijing Children 's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China Department of Pathology Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China

In adults, the presence of the BRAF^(V600E) mutation in papillary thyroid cancer(PTC) has been demonstrated to be strongly associated with aggressive cancer-cell characteristics and poor patient prognosis. In contrast, the frequency of this mutation in pediatric PTC has undergone limited study, and the few available estimates range from 0 to 63%. Furthermore, the role of the BRAF^(V600E) mutation in pediatric PTC is controversial; thus, the present study aimed to investigate the prevalence and role of the BRAF^(V600E) mutation in48 pediatric patients with PTC, aged 3–13 years. Of these patients, 41 were diagnosed with classic PTC, five were found to have a follicular variant of PTC, and two to exhibit a diffuse sclerosing PTC variant. The BRAF^(V600E) mutation was identified to be present in 35.4% of the 48 analyzed patients, and in 41.5% of the patients diagnosed with classical PTC. Furthermore, the presence of the BRAF^(V600E) mutation was found to be associated with a patient age at diagnosis of less than ten years(P=0.011), the performance of a thyroidectomy(P=0.03), exhibited tumor multifocality(P=0.02) and/or extra-thyroidal invasion(P=0.003), and both a low MACIS(Metastases, Age, Completeness of resection, Invasion, Size)(P=0.036) and AMES(Age, Metastasis, Extent of tumor,Size)(P=0.001)score. Together, these data suggest that the presence of the BRAF^(V600E) mutation may be negatively correlated with partial aggressive clinicopathological features of pediatric PTC.

关键词： pediatric papillary thyroid cancer BRAF^(V600E) mutation clinicopathological characteristics

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儿童中耳胆脂瘤合并颅内外并发症1例

临床耳鼻咽喉头颈外科杂志 2022年第2期36卷 139-141页

作者：温鑫宋英鸾权珊黄爱萍周永青河北医科大学附属河北省儿童医院耳鼻咽喉科石家庄050031 联勤保障部队第980医院耳鼻咽喉头颈外科

1 病例报告患儿,女,9岁,主因双耳流脓20余天,发热伴呕吐14 d入院。患儿20 d前无明显诱因出现双侧外耳道流脓,伴耳痛,右侧为著,当地医院以"化脓性中耳炎"给予静脉点滴拉氧头孢抗感染及点耳治疗,仍有反复流脓,随后患儿出现发热,体温最高4... 详细信息

1 病例报告患儿,女,9岁,主因双耳流脓20余天,发热伴呕吐14 d入院。患儿20 d前无明显诱因出现双侧外耳道流脓,伴耳痛,右侧为著,当地医院以"化脓性中耳炎"给予静脉点滴拉氧头孢抗感染及点耳治疗,仍有反复流脓,随后患儿出现发热,体温最高41.5℃,伴寒战、头痛、呕吐、咳嗽、咳痰,于当地医院完善相关检查后考虑"颅内感染"给予阿奇霉素联合头孢吡肟(4 d)、万古霉素联合美罗培南(2 d)抗感染等治疗后.

关键词：中耳胆脂瘤儿童并发症

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42例儿童笔帽类气管支气管异物的诊治体会

临床耳鼻咽喉头颈外科杂志 2021年第9期35卷 836-838页

作者：王亚芳宋英鸾赫莉温鑫史雅楠赵海涛河北省儿童医院耳鼻咽喉科石家庄050031 河北省儿童医院麻醉科

气管、支气管异物是严重危及儿童生命的急症,异物性质以坚果等有机物最为多见,笔帽类特殊异物较为少见,此类异物体积较大,气道堵塞较为严重,且异物取出具有一定难度,其中又以笔帽底部无通气孔的病例病情更为危重,一旦处理不当,会出现危... 详细信息

气管、支气管异物是严重危及儿童生命的急症,异物性质以坚果等有机物最为多见,笔帽类特殊异物较为少见,此类异物体积较大,气道堵塞较为严重,且异物取出具有一定难度,其中又以笔帽底部无通气孔的病例病情更为危重,一旦处理不当,会出现危及生命的严重后果[1]。现回顾我院收治的42例经硬质支气管镜检查证实为笔帽类异物的患儿资料(图1),按照笔帽底部是否有通气孔进行分组(图2、3),并对两组患儿的临床特点及诊治情况进行比较,以提高对此类疾病的认识。

关键词：异物支气管镜检查儿童

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气管异物误诊食管异物一例

中华耳鼻咽喉头颈外科杂志 2020年第10期55卷 965-966页

作者：温鑫赫莉左路杰权珊史雅楠宋英鸾河北医科大学附属河北省儿童医院耳鼻咽喉科石家庄050031

本文报道1例气管别针异物误诊为食管异物的病例。患儿男,8个月25 d,主诉误咽别针12 h入院,结合病史、临床表现及胸部X线正位片诊断为食管异物。在急诊全身麻醉气管插管下行食管镜检查,未见异物。术中床旁胸部X线正侧位片显示异物位于气... 详细信息

本文报道1例气管别针异物误诊为食管异物的病例。患儿男,8个月25 d,主诉误咽别针12 h入院,结合病史、临床表现及胸部X线正位片诊断为食管异物。在急诊全身麻醉气管插管下行食管镜检查,未见异物。术中床旁胸部X线正侧位片显示异物位于气管,已被气管插管推到隆突附近。后在硬性支气管镜下将异物别针取出。术前详细询问病史,对阳性异物常规行胸部X线正侧位片检查,可以减少误诊发生。

关键词：食管异物气管异物食管镜检查误咽隆突气管插管减少误诊别针

头孢泊肟酯颗粒剂治疗儿童急性化脓性中耳炎的临床研究

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中华实用儿科临床杂志 2019年第16期34卷 1264-1266页

作者：王登茂耿江桥赫莉宋英鸾河北省儿童医院耳鼻咽喉科石家庄050031

目的对头孢泊肟酯颗粒剂在儿童急性化脓性中耳炎的治疗效果和安全性方面进行评估。方法选取2017年6月至2018年12月河北省儿童医院耳鼻咽喉科门诊就诊的年龄1~12岁患儿120例,按随机抽签法将其分为对照组和治疗组各60例。治疗组男33例,女2... 详细信息

目的对头孢泊肟酯颗粒剂在儿童急性化脓性中耳炎的治疗效果和安全性方面进行评估。方法选取2017年6月至2018年12月河北省儿童医院耳鼻咽喉科门诊就诊的年龄1~12岁患儿120例,按随机抽签法将其分为对照组和治疗组各60例。治疗组男33例,女27例;年龄(4.0±1.8)岁。对照组男31例,女29例;年龄(4.5±1.6)岁。2组患儿同时给予5 g/L小儿盐酸麻黄碱滴鼻(连续使用时间<7 d),1~2滴/次,3次/d。治疗组口服头孢泊肟酯颗粒剂5 mg/kg,2次/d;对照组口服头孢羟氨苄颗粒10 mg/kg,3次/d,疗程均为10 d,对有耳漏耳流脓的患儿,加用30 g/L过氧化氢耳浴治疗。观察患儿的主要症状和体征的评分值变化,计算治疗前后症状、体征改善百分率、治愈率和总有效率。同时,对有耳流脓者的脓液进行细菌培养和药敏试验,计算细菌清除率。密切观察有无皮疹、恶心呕吐、腹泻休克等不良反应,并准确记录其反应程度、治疗情况及恢复情况。结果对照组治愈率和总有效率分别为80.0%(48/60例)和81.7%(49/60例),治疗组治愈率和总有效率分别为90.0%(54/60例)和96.7%(58/60例),2组临床疗效比较差异有统计学意义(χ^2=8.886,P<0.05)。治疗组2例出现恶心、腹泻的胃肠道反应,给予便常规检查未见明显异常,停药后症状消失。对照组用药后3例出现厌食、呕吐和稀便,便常规检查也未见明显异常,停药并适当对症治疗后好转。结论头孢泊肟酯颗粒剂治疗儿童急性化脓性中耳炎安全有效。

关键词：头孢泊肟酯急性化脓性中耳炎儿童

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气管支气管异物致突然窒息三例

中华耳鼻咽喉头颈外科杂志 2018年第9期53卷 692-693页

作者：温鑫宋英鸾王登茂史雅楠阎玉彦河北省儿童医院耳鼻咽喉科石家庄050031

呼吸道异物是儿童常见的意外伤害之一，异物通过喉部时容易出现喉痉挛，或者在取出异物的手术过程中出现“窒息性异物变位”，严重者可出现缺氧、窒息甚至死亡。近几年，河北省儿童医院耳鼻喉科收治入院后突然发生窒息的气管支气管异物... 详细信息

呼吸道异物是儿童常见的意外伤害之一，异物通过喉部时容易出现喉痉挛，或者在取出异物的手术过程中出现“窒息性异物变位”，严重者可出现缺氧、窒息甚至死亡。近几年，河北省儿童医院耳鼻喉科收治入院后突然发生窒息的气管支气管异物患儿3例，报道如下。

关键词：气管支气管异物突然窒息河北省儿童医院呼吸道异物意外伤害手术过程取出异物耳鼻喉科

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婴儿毛息肉13例临床特征及诊治分析

中华耳鼻咽喉头颈外科杂志 2020年第9期55卷 850-854页

作者：黄爱萍张海中崔莉耿江桥温鑫王娟宋英鸾河北省儿童医院耳鼻咽喉科石家庄050030 河北医科大学第二医院耳鼻咽喉科石家庄050005

目的探讨分析婴儿毛息肉的临床特征及诊治体会。方法回顾性分析河北省儿童医院自2010年1月至2019年9月收治的经病理确诊的13例婴儿毛息肉患儿的临床资料,其中男4例,女9例,男女比例为1∶2.25;年龄为出生3 h至1岁,中位年龄1月龄。13例患儿... 详细信息

目的探讨分析婴儿毛息肉的临床特征及诊治体会。方法回顾性分析河北省儿童医院自2010年1月至2019年9月收治的经病理确诊的13例婴儿毛息肉患儿的临床资料,其中男4例,女9例,男女比例为1∶2.25;年龄为出生3 h至1岁,中位年龄1月龄。13例患儿中12例因呼吸困难或喂养困难而被发现。所有患儿在全身麻醉下行鼻内镜辅助下鼻咽部肿物等离子切除术,术中发现肿物根蒂位于鼻咽侧壁8例;软腭与扁桃体腭咽弓上极交界处1例;舌根部1例;食道入口1例;鼻中隔2例,其中1例合并口腔毛息肉,位于两门齿之间。术中根蒂处创面以低温等离子射频消融并彻底止血。结果13例患儿手术过程顺利,术后临床症状消失,术后随访3个月至2年,12例患儿无复发,1例患儿术后2年纤维喉镜检查见右侧咽鼓管咽口肿物,考虑毛息肉复发,之后患儿失访。结论婴儿毛息肉为罕见病,主要表现为上呼吸道梗阻,早期诊断,尽早彻底手术是治疗该疾病的关键。

关键词：毛息肉婴儿上呼吸道梗阻

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新生儿鼻咽部胶质瘤一例

中华小儿外科杂志 2021年第7期42卷 661-663页

作者：黄爱萍崔莉王娟赫莉刘晓峰宋英鸾河北省儿童医院耳鼻咽喉科石家庄050000

患儿男,23 d,主因"间断呼吸困难18 d"于2020年1月25日收治入院。患儿出生时一般情况良好,无窒息抢救史,出生5 d后出现鼻塞、呼吸不畅、吃奶费力等现象,于当地医院就诊发现鼻咽部肿物,家属未选择住院治疗。今晨起患儿反应差,自主呼吸弱,... 详细信息

患儿男,23 d,主因"间断呼吸困难18 d"于2020年1月25日收治入院。患儿出生时一般情况良好,无窒息抢救史,出生5 d后出现鼻塞、呼吸不畅、吃奶费力等现象,于当地医院就诊发现鼻咽部肿物,家属未选择住院治疗。今晨起患儿反应差,自主呼吸弱,口唇青紫,遂急就诊于当地医院,当地医院接诊时,患儿无呼吸,无心跳,紧急行心肺复苏及气管插管术,呼吸机辅助呼吸后转入我院。入院体格检查:体温37.8℃,脉搏130次/min,呼吸机辅助呼吸下的呼吸频率为36次/min,经皮血氧饱和度为93%,足月儿貌,反应差,活动少,咽部未窥及肿物影,颈软无抵抗,三凹征阳性,双肺呼吸音粗,可闻及痰鸣音,余未见明显异常。

关键词：肺呼吸音口唇青紫气管插管术鼻咽部痰鸣音颈软无抵抗心肺复苏呼吸困难