头和脖子|概述
话题：青少年鼻咽血管纤维瘤的遗传，荷尔蒙和分子决定因素：系统评价
首席研究员：迈克尔·坎宁安博士
Summary:We present a systematic review of the molecular and somatic genetic determinants of JNA, including a qualitative and, where appropriate, quantitative meta-analysis of pooled small sample sized studies. We highlight areas of particular clinical relevance, including potential points of therapeutic intervention with respect to tumorigenesis and vasculogenesis. Additionally, we explore areas of controversy in pathogenesis, including the histological origin of JNA and the relationship of JNA to familial adenomatous polyposis (FAP) as related to the APC/B-catenin pathway. Lastly, we examine the potential clinical implications of a deeper genomic understanding of JNA, including the role for hormonal therapy and the capability to include genetic or molecular markers in staging criteria.
Summary:We present a systematic review of the molecular and somatic genetic determinants of JNA, including a qualitative and, where appropriate, quantitative meta-analysis of pooled small sample sized studies. We highlight areas of particular clinical relevance, including potential points of therapeutic intervention with respect to tumorigenesis and vasculogenesis. Additionally, we explore areas of controversy in pathogenesis, including the histological origin of JNA and the relationship of JNA to familial adenomatous polyposis (FAP) as related to the APC/B-catenin pathway. Lastly, we examine the potential clinical implications of a deeper genomic understanding of JNA, including the role for hormonal therapy and the capability to include genetic or molecular markers in staging criteria.
话题：Endoscopic Radiofrequency Ablation Assisted Resection of Juvenile Nasopharyngeal Angiofibroma: Comparison with Traditional Endoscopic Technique
首席研究员：Michael Cunningham博士和Eelam Adil博士
Summary:鉴于青少年鼻咽血管纤维瘤（JNA）的血管性质，在肿瘤切除过程中，实现止血和组织消融的能力在理论上是有益的。这项研究的目的是评估JNA射频消融辅助（RFA）切除的可行性。我们还旨在将JNA的内窥镜RFA切除与年龄和阶段匹配的控制措施进行比较，他们使用传统的内窥镜（TE）仪器技术进行了内窥镜切除。
Summary:鉴于青少年鼻咽血管纤维瘤（JNA）的血管性质，在肿瘤切除过程中，实现止血和组织消融的能力在理论上是有益的。这项研究的目的是评估JNA射频消融辅助（RFA）切除的可行性。我们还旨在将JNA的内窥镜RFA切除与年龄和阶段匹配的控制措施进行比较，他们使用传统的内窥镜（TE）仪器技术进行了内窥镜切除。
没有使用射频的统计优势ablation device compared to traditional endoscopic instrumentation was identified with respect to operative time, intraoperative blood loss, transfusion rate, hospital length of stay, or recurrence rate. RFA instrumentation, however, does offer the surgeon an alternative option for tumor debulking during resection of these highly vascularized tumors.
话题：小儿囊性纤维化中的Sinus CT成像：附加值？
首席研究员：Michael Cunningham博士和Eelam Adil博士
Summary: In the pediatric CF population, the need for frequent imaging increases the potential risk for significant cumulative lifetime doses of ionizing radiation given the multisystem involvement of this disease. The aims of this study were to 1) review the overall prevalence of CT imaging in our pediatric CF population with a focus on paranasal sinus imaging indications and outcomes 2) evaluate changes in Lund-Mackay (LM) scores over time and 3) estimate radiation exposure secondary to sinus CT imaging.
话题：诊断鼻睫毛运动障碍的鼻和气管支气管活检的电子显微镜：系统评价和荟萃分析Summary: In the pediatric CF population, the need for frequent imaging increases the potential risk for significant cumulative lifetime doses of ionizing radiation given the multisystem involvement of this disease. The aims of this study were to 1) review the overall prevalence of CT imaging in our pediatric CF population with a focus on paranasal sinus imaging indications and outcomes 2) evaluate changes in Lund-Mackay (LM) scores over time and 3) estimate radiation exposure secondary to sinus CT imaging.
All patients with CF have relatively high LM scores regardless of clinical context. Routine imaging for disease evaluation is probably not necessary and represents an area where radiation exposure can be decreased in these patients. Clinicians should limit paranasal sinus imaging to the preoperative context or the evaluation of suspected sinusitis complications. In this context, otolaryngology consultation should be considered prior to imaging. In addition, LM scores for individual patients do not change significantly with time or intervention. If there has been no prior surgical anatomical disruption and the child is of an age in which significant developmental changes between imaging studies is unlikely, then repeat paranasal sinus imaging can also be avoided.
主要研究人员：Michael Cunningham博士和Eelam Adil博士
Summary:在疑似PCD的患者中，确认诊断的最广泛接受的方法涉及纤毛呼吸粘膜的活检，然后进行超微结构电子显微镜（EM）评估，高速视频显微镜检查或基因检测已知突变。关于最有效的活检部位或方法，鼻腔和气管支原体活检部位均未达成共识。这项系统综述和荟萃分析的目的是分析有关替代活检部位和PCD诊断方法的功效的文献。
目前尚无针对PCD患者的单一确定诊断测试。
Ultrastructural EM evaluation of ciliary epithelium provides valuable information that is complementary to clinical history and alternative diagnostic methods in individuals suspected to have PCD. Nasal ciliary biopsy should be considered prior to tracheobronchial biopsy in patients who can tolerate an office-based procedure. Scrape biopsy may be superior to brush and forceps techniques, but a more definitive study taking into account the training and experience of the physician performing the biopsy is necessary to confirm this finding.
话题：急性小儿鼻窦炎的轨道并发症
首席研究员：Eelam Adil博士
Summary:While acute sinusitis is a relatively common condition in children, it has the potential to spread to the adjacent orbit and brain. The majority of complications involve the orbit, and Oxford and McClay report that a remarkable 91% of their pediatric patients with complicated acute sinusitis had orbital involvement (2005). Chandler's classification of orbital complications include I. periorbital cellulitis, II. orbital cellulitis, III. subperiosteal abscess, IV. orbital abscess, V. cavernous sinus thrombosis. With this study, we hope to take a deeper look at these complications and their course of treatment. We will review all pediatric patients with acute sinusitis seen at Boston Children's Hospital over the past 10 years, and we will categorize the patients into 4 groups: 1) patients admitted with acute sinusitis who have no orbital or intracranial complications; 2) patients admitted with acute sinusitis who have an orbital complication who are treated medically and eventually discharged; 3) patients admitted with acute sinusitis who are initially treated medically with IV antibiotics but fail to improve and eventually require surgery; 4) patients with an orbital complication who undergo immediate surgical intervention. We will compare groups based on age, gender, ethnicity, past medical and surgical history, prior sinusitis and treatment history, duration of symptoms prior to presentation, extent of sinusitis and infection, oral antibiotic use, type of orbital complication, presence of fever and fever duration, laboratory studies (e.g. white blood cell count), IV antibiotic therapy, ophthalmology findings, nasal endoscopic findings, imaging findings, microbiology and sensitivities, permanent complications, length of hospitalization, surgical intervention, readmission, and duration of antibiotic therapy. We are hopeful that this retrospective case control study will better inform treatment decisions and improve our understanding of how orbital complications impact the clinical outcome of acute sinusitis.
话题：儿童鼻中隔穿孔：表现，病因和管理
首席研究员：Eelam Adil博士
Summary:小儿鼻中隔穿孔是一种罕见的临床状况。隔膜穿孔的症状包括鼻腔吹口哨，复发性脑力，结皮和不适。虽然有大量有关成人隔膜穿孔的文献，但有关小儿间隔穿孔的文献仅限于几个小系列，每例患者有3个或更少的患者。我们建议对波士顿儿童医院的诊断和管理诊断和管理进行全面回顾性回顾。我们有兴趣研究患者人口统计学，症状，病因学，临床表现，治疗和手术修复该病情。我们认为，这将不仅可以提高我们对小儿鼻中隔穿孔的病因的理解，而且还可以提高我们对最有效的管理技术的理解。
话题：Endoscopic sinus surgery in pediatric cystic fibrosis patients
首席研究员：Eelam Adil博士, Dr. Michael Cunningham
Summary:这项研究的目的是描述和记录波士顿儿童医院在囊性纤维化的小儿患者中进行内窥镜鼻窦手术的经验。欧宝彩票平台具体而言，我们想研究内窥镜鼻窦手术是否影响这些患者的肺功能。我们正在分析手术前和手术后长达一年的囊性纤维化患者肺功能测试的数据。通过查看术前和术后数据，我们希望从肺部的角度来确定内窥镜鼻窦手术是否有益。